Laparoscopic gastric bypass is becoming a frequently performed bariatric operation for patients with obesity. However, bariatric surgery has inherent risks and complications, which are further potentiated by the high risk nature of bariatric patients. These complications can be either site specific i.e. anastomotic leaks, or systemic i.e. venous thromboembolism, however they can intimately related. We present the case of one patient undergoing routine gastric bypass complicated postoperatively by recurrent intra-abdominal collections and sepsis, and aortic thrombosis with distal embolisation.

INTRODUCTION

Laparoscopic gastric bypass surgery is amongst the most frequently performed operations in bariatric surgery. We report the case of one patient undergoing routine gastric bypass who experienced major complications.

CASE REPORT

A 56 year old female was referred from a district general hospital after a protracted history of abdominal pain and vomiting. Past medical history included osteoarthritis and hypertension, and a past surgical history of laparoscopic Roux-en-Y gastric bypass in 2008 (pre-operative BMI of 48 Kg/m2).

A contrast swallow on admission showed evidence of multiple small bowel strictures.  A laparotomy and revision of Roux-en-Y gastric bypass was undertaken with post operative management on HDU, but the patient had ongoing problems with pain and wound discharge. A CT scan revealed a 7cm collection adjacent to the alimentary limb which was percutaneously drained under ultrasound guidance.

Due to clinical deterioration, a further CT was performed which demonstrated a large communicating collection filling the paracolic gutters and pelvis, with a multiloculated left lobe liver abscess, and a smaller abscess in the right lobe (figure 1). This was percutaneously drained and there was clinical improvement. The patient was discharged home.

Fig. 1: Multiloculated abscess within the left lobe of liver

The patient re-presented 2 weeks later with increasing epigastric pain and malaise. Imaging showed a persistent collection in the pelvis and liver, and a further two percutaneous procedures were performed.

During inpatient stay, she became haemodynamically unstable with spiking temperatures and an elevating white cell count. She also complained of bilateral discolouration of her toes and examination revealed increasing oedema with diminished pedal pulses. A contrast CT scan of the abdomen confirmed ongoing collections with evidence of an aortic thrombus extending into the left iliac artery, presumed to be the embolic source for her vascular findings (figure 2 & 3). She underwent a further laparotomy and washout, and was heparinised postoperatively. There was resolution of vascular symptoms and evidence of improvement in peripheral perfusion postoperatively, and the patient was eventually discharged.

Fig. 2: Thrombus within the distal aorta shown by a filling defect (red arrow)

Fig. 3: Extension into the left iliac artery shown by a filling defect (red arrow)

DISCUSSION

Surgery has inherent risks and complications, which may be further be potentiated by the high risk nature of bariatric patients.  A systematic review of the literature concluded that peri-operative complications were encountered more frequently in bypass patients compared to those undergoing laparoscopic gastric banding (9% and 5%), although long-term re-operation rates were lower (16% and 24% respectively) (1). The mortality of both bypass and band operations were low (0.17% and 0.06%). Further systematic reviews have reported major early complications rates of gastric bypass surgery as 6.3%, with major complications including death (0.2%), perforation (0.5%), venous thromboembolism (0.3%), significant postoperative bleed (0.9%), postoperative infection (2.9%) and anastomotic leaks (0.9%) (1).

Despite these risks gastric bypass is an effective operation for obesity; one study reported 76% excess body weight loss and a 78% resolution of diabetes at 1 year in patients undergoing bypass compared to 48% weight loss and 50% diabetes resolution in patients undergoing banding (2). Generally high levels of patient satisfaction are recorded, estimated to approach 80% (3).

Venous thrombosis is well documented in the literature as a complication of obesity and bariatric surgery. One study reported an incidence of deep vein thrombosis in the group of 500 consecutive patients to be 0.2% (4). However, there is little data regarding arterial thrombosis as a complication of bariatric surgery.

There are numerous predisposing factors to arterial thrombosis within this clinical picture. Bariatric patients have significant risk factors for atherosclerotic disease, both due to obesity, and to secondary diseases such as hypertension and diabetes, which may develop as part of the metabolic syndrome. However, with this patient there was no evidence of aortic thrombosis on previous imaging, and a new presentation of clinical signs and symptoms would be in keeping with a new onset thrombosis.

There are also factors relating to a hypercoagulable state, which in this patient could be numerous. This patient had recurrent episodes of sepsis, which is well known to precipitate hypercoagulability, which at the severe end of the spectrum, exists disseminated intravascular coagulation (DIC). Sepsis mediated hypercoagulability occurs as a result of the activation of inflammatory and coagulation cascades (5). Dehydration secondary to both poor intake and insensible losses through either infection or surgery may also cause an increased coagulable state. Inflammatory and hypercoagulation may be due to the direct effects of surgery through the stress response (6).

Roux-en-Y gastric bypass is deemed a safe operation in the management of obesity. It generally has low complications and high patient satisfaction (1-3). We present a case complicated postoperatively by recurrent intra-abdominal collections and sepsis, and aortic thrombosis with distal embolisation.

REFERENCES

1. Tice, J, Karliner L, Walsh J, Petersen AJ, Feldman MD. Gastric Banding or Bypass? A Systematic Review Comparing the Two Most Popular Bariatric Procedures. The American Journal of Medicine 2008; 121(10):885–893
2. Cottam DR, Atkinson J, Anderson A, Grace B, Fisher B. A case-controlled matched pair cohort study of laparoscopic Roux-en-Y gastric bypass and Lap-Band patients in a single US center with three-year follow-up. Journal of Obesity Surgery 2006;16:534–540
3. Bowne WB, Julliard K, Castro AE, Shah P, Morgenthal CB, Ferzli GS. Laparoscopic gastric bypass is superior to adjustable gastric band in super morbidly obese patients: a prospective, comparative analysis. Archives of Surgery 2006;141:683–689
4. Escalante-Tattersfield T, Tucker O, Fajnwaks P, Szomstein S, Rosenthal RJ. Incidence of deep vein thrombosis in morbidly obese patients undergoing laparoscopic Roux-en-Y gastric bypass. Journal of the American Society of Bariatric Surgery 2008; 4(2):126–30
5. Aird W. The role of the endothelium in severe sepsis and multiple organ dysfunction syndrome. Blood. 2003; 101(10):3765–3777
6. Hahnenkamp K, Theilmeier G, Van Aken HK, Hoenemann CW. The effects of local anesthetics on perioperative coagulation, inflammation, and microcirculation. Anaesthesia & Analogues. 2002;94(6):1441-1447

Malignant mesothelioma of the tunica vaginalis is a rare but potentially fatal disease. Lack of characteristic clinical features and tumour markers makes the pre-operative diagnosis very difficult. A 54 year-old man with no history of exposure to asbestos presented with a short history of scrotal swelling and pain. Ultrasound revealed a suspicious nodularity involving his tunica vaginalis, suggestive of mesothelioma. Excisional biopsy revealed a malignant mesothelioma; hence the patient was treated with radical inguinal orchidectomy and hemiscrotectomy. We present a case of this rare tumour, its management and a review of the literature.

INTRODUCTION

Malignant mesothelioma (MM) of the tunica vaginalis testis is a rare but often fatal malignancy. The lack of characteristic clinical features and tumour markers makes the pre-operative diagnosis of this fatal malignancy extremely difficult. We report a case of this tumour with a pre-operative sonographic diagnosis.

CASE REPORT

A 54 year-old male farmer was referred by his GP to our radiology department for a scrotal ultrasound to investigate a two week history of right testicular pain and swelling. He was an ex-smoker and denied exposure to asbestos in his occupation. Clinical examination of the scrotum was normal except for small bilateral hydroceles. Urine examination and blood tests including HCG, AFP and LDH were within normal limits.

Ultrasound demonstrated multifocal echogenic lesions with intrinsic vascularity confined to the tunica vaginalis. These findings were felt to be highly suspicious for mesothelioma (Figure 1).  Subsequently the patient had a staging CT scan of the thorax, abdomen and pelvis and this demonstrated no evidence of metastatic disease.

Fig. 1: (A) Ultrasound scan demonstrating the largest of multiple small nodules studding into the right tunica vaginalis in addition to a hydrocele. Doppler interrogation suggests that this nodule has parasitized blood flow from the testis. (B) CT scan demonstrating bilateral hydroceles with enhancing nodularity of the tunica vaginalis on the right (arrow)

Following this, the patient underwent an excisional biopsy for what appeared to be a primary mesothelioma of the tunica vaginalis. At surgical exploration, there were two subcentimetre lesions on the surface of both testes. The largest on the right measured 6 mm. The lesions on the left were tiny and measured 2 to 3 mm. These lesions were excised for histology. The tiny specimens from the left side demonstrated no malignancy. On histological examination, the biopsies taken from the right testis showed a biphasic exophytic nodule composed of a mixture of epithelioid and spindled cells with mild to moderate cytological atypia (Figure 2).

Fig. 2: Biopsy from the surface of the right testis (20X). It shows a biphasic exophytic nodule composed of a mixture of epitheloid (blue arrow) and spindled cells (black arrow) with mild to moderate cytological atypia. A shoulder of normal mesothelial lining was included (arrow head). Occasional apoptotic and mitotic figures are present.

Occasional apoptotic and mitotic figures were also present. The morphological and immunohistochemical profile is congruent with the diagnosis of a malignant mesothelioma (Figure 3). The patient then underwent a right inguinal orchidectomy and hemiscrotectomy. There was no residual malignancy in the final specimen. A medical oncology opinion was sought and it was decided the patient should kept under surveillance with clinical and sonographic follow-up.

Fig 3: Immunohistochemistry demonstrates: A: Both epitheloid and spindled components are strongly and diffusively positive for pancytokeratin AE1/3. B: To a lesser degree both components stain positively for CAM 5.2, another cytokeratin stain. C: To epithelioid component is positive for Calretinin. D: WT-1 nuclear positivity is seen in both epitheloid and spindled components.

DISCUSSION

Mesothelioma is a neoplasm of the serosal surfaces. It involves the pleura, less frequently the peritoneum and very rarely the pericardium or the tunica vaginalis of the testis.

More than two-thirds of the cases of malignant mesothelioma (MM) are patients older than 45 years of age with the median age being 60 years (1), however it has been reported in a 10 year-old child (2). The majority of patients present with a hydrocele and some patients present with a testicular tumour. One third of these patients reported exposure to asbestos (1).

Sonographically, the most common feature of MM is a hydrocele, which can be associated with multiple extratesticular soft tissue fronds of mixed echogenicity. The hydrocele may contain multiple heterogeneous extratesticular nodular masses, ranging in size from a few millimetres to several centimetres, attached to the tunica vaginalis; or a single heterogeneous extratesticular mass arising from the scrotal wall. Less frequently, a simple hydrocele may be the only sonographic finding (1),  however, appearance as a predominantly intratesticular mass has been reported (3).

Macroscopically, MM of the tunica vaginalis typically appears as multiple, firm, tan-to-white nodules or papillary excrescences on the surface of a hydrocele sac. The tunica vaginalis is usually thickened and the tumour mass may invade the spermatic cord or testis or both (3). Histologically there are 3 types of malignant mesothelioma: epithelial, mesenchymal or sarcomatous, and biphasic or mixed (4). The most frequent type found in malignant tunical mesothelioma is the epithelial type, followed by the mixed type, with the pure sarcomatous type being very rare (1,5).

Surgery is the only curative treatment for MM of the tunica vaginalis. Because most of the cases are diagnosed intra-operatively or at final histology, a second procedure is usually required to excise the remaining tissue. Radical inguinal orchidectomy and hemiscrotectomy is the recommended surgical treatment. The necessity for inguinal or iliac lymph node dissection as primary therapy remains controversial (1).

There is not enough data to support the role of chemotherapy and /or radiotherapy, however Plas et.al (1) believe that adjuvant radiotherapy should be considered for patients with locally advanced MM following tumour resection with safe  margins, and for patients with metastatic disease who are otherwise fit. In their review, the common sites of metastasis were lymph nodes (13.8%), lung (9.7%) and liver (4.2%). The mean disease-specific survival for patients with or without systemic treatment was reported to be 26 and 36 months, respectively (6).

Prognostic parameters demonstrated better survival in younger age group ( < 60 years) and worse survival with the presence of metastasis. Exposure to asbestos was significantly related to shorter interval before tumour recurrence (1).

In summary, we report a case of a rare Malignant Mesothelioma of the tunica vaginalis with no history of exposure to asbestos. Our case was a pre-operative sonographic diagnosis which is unusual in this potentially aggressive tumour that lacks tumour markers and characteristic clinical features.

REFERENCES

1. Plas E, Riedl CR, Pfluger H. Malignant mesothelioma of the tunica vaginalis testis: review of the literature and assessment of prognostic parameters, Cancer 1998; 83:2437
2. Antman K, Cohen S, Dimitrov NV, et al. Malignant mesothelioma of the tunica vaginalis testis. J Clin Oncol 1984;2:447–51
3. Wolanske K, Nino-Murcia M. Malignant mesothelioma of the tunica vaginalis testis: atypical sonographic appearance. J Ultrasound Med. 2001;20:69-72
4. Eimoto T, Inoue I. Malignant fibrous mesothelioma of the tunica vaginalis. Cancer 1977; 39:2059–2066
5. Jones MA, Young RH, Scully RE. Malignant mesothelioma of the tunica vaginalis. A clinicopathologic analysis of 11 cases with review of the literature. Am J Surg Pathol 1995; 19:815–825
6. Spiess PE, Tuziak T, Kassouf W, et al. Malignant mesothelioma of the tunica vaginalis. Urology 2005;66:397–401

Here, we present the first case of post-thymectomy Myasthenia Gravis with onset at Osserman stage III. An 81-year-old woman was admitted for an abnormal shadow seen in a chest radiograph. She had no symptoms of Myasthenia Gravis. Acetylcholine receptor antibody was within the normal range. Chest computed tomography (CT) showed a bulky anterior mediastinal tumor. She was diagnosed as having thymoma by tissue biopsy under CT guidance. The tumor was completely resected by performing thymothymectomy, left upper lobectomy, pericardial resection, and phrenicectomy. Pathological examination of the tumor identified it as a thymoma (type B2, Masaoka stage II). Two months after the surgery, she experienced the onset of post-thymectomy myasthenia gravis with Osserman stage III. The acetylcholine receptor antibody level was remarkably elevated (220 nmol/L); however, there was no evidence of tumor recurrence.

INTRODUCTION

Thymectomy is generally recommended for patients with thymoma. However, Myasthenia Gravis occasionally develops postoperatively in patients who have had thymoma despite no signs of Myasthenia Gravis before the surgery. Some studies (1-8) have reported post-thymectomy Myasthenia Gravis (PTMG); however, its mechanism and risk factors remain unclear. Moreover, no case of PTMG with onset at Osserman stage III has been reported. Here, we present the first case of PTMG at Osserman stage III.

CASE REPORT

An 81-year-old woman was admitted to our hospital for examination and treatment of an abnormal shadow seen on chest radiograph. She had no symptoms on admission. Tumor markers were within the normal range. Acetylcholine receptor antibody (ARA) was negative. Her lung function was normal. Chest computed tomography (CT) showed a bulky mediastinal tumor bulging outward into the left thoracic space (Figure 1).

Fig. 1: Preoperative chest computed tomography (CT) showing a bulky mediastinal tumor bulging outward into the left thoracic space and invading the pericardium and pleura.

The tumor was diagnosed as a thymoma by tissue biopsy under CT guidance. The operative findings of the tumor was completely resected by performing thymothymectomy, left upper lobectomy, pericardial resection, and phrenicectomy with median sternotomy and superior longitudinal anterior mediastinotomy. The most part of tumor had a place in the left pleural space and invaded the left superior lobe of the lung, pericardia and left phrenic nerve. Pathological examination of the tumor identified as a thymoma (Masaoka stage II). She was discharged 40 days after the surgery without postoperative complications. Two months after the surgery, the patient was admitted to our hospital for respiratory failure requiring mechanical ventilation. ARA level was remarkably elevated (220 nmol/L). Evoked electromyography showed waning. However, there was no evidence of tumor recurrence on imaging examination. We diagnosed the patient as having PTMG with Osserman stage III and started steroid pulse therapy (prednisolone 1g/day for 3 days). Additionally, we treated the patient with immunoadsorption to shorten the duration of the steroid therapy. At three months after the admission, she was removed from the ventilator with a drug regimen of 60 mg/day prednisolone. Moreover, the ARA level reduced to 43.5 nmol/L (Figure 2).

Fig. 2: A chart showing the clinical course.

DISCUSSION

Here, we present the first case of myasthenia gravis onset with Osserman stage III after thymectomy. Studies have reported that the incidence of PTMG is 0.9–20% (1-6). However, we could not find reports of PTMG occurring at Osserman stage III.

The current hypothesis of the pathogenic mechanism of PTMG includes: [1]thymoma recurrence (7,8); [2] surgical exposure to larval MG (2); and [3]activation of peripheral lymphocytes from thymoma after surgery (9,10). The risks of developing PTMG have been documented in various studies. However, these studies did not highlight risks such as operative method that may be statistically significant. In this regard, Nakajima et al reported that all patients with Myasthenia Gravis before or after thymectomy had high ARA levels at the onset of Myasthenia Gravis. Thus, high ARA level might be a predictive indicator of PTMG (6).

In our case, postoperative imaging examination and preoperative physical findings showed that PTMG did not develop because of the factors highlighted in hypotheses 1 and 2. PTMG onset was more likely to be caused by the factors described in hypothesis 3. The methods for evaluating the hypothesis 3 are currently available for clinical use. However, we regret that we evaluated ARA at an earlier date after the surgery. Moreover, after the surgery we might examine her about Myasthenia Gravis such as Harvey-Masland test.

We experienced a case of PTMG onset at Osserman stage III with a negative preoperative ARA. We conclude that surgery of thymoma needs close and detailed pre- and postoperative myasthenia gravis examinations to screen for PTMG onset. We believe that this case may offer some useful information for patients with thymoma, be considered surgery.

REFERENCES

1. Namba T, Grunner NG, Grob D. Myasthenia gravis in patients with thymoma, with particular reference to onset after thymectomy. Medicine 1978;57:411-433
2. Li J, Zhang DC, Wang LJ, Zhang DW, Zhang RG. Myasthenia gravis occurring after resection of thymoma. Zhonghua Wai Ke Za Zhi 2004;42:540-542
3. Ohta M, Itoh M, Hara H, Itoh N, Nishitani H, Hyashi K, et al. Anti-skeletal muscle and anti- acetylcholine receptor antibodies in patients with thymoma without myasthenia gravis: relation to the onset of myasthenia gravis. Clin Chim Acta 1991;201:201-205
4. Ito M, Fujimura S, MondenY, Watanabe Y, Shimizu N, Hirono T, et al. A retrospective group study on post-thymectomy myasthenia gravis. Nippon Kyobu Geka Gakkai Zasshi 1992;40:189-193
5. Kondo K, Monden Y. Myasthenia gravis appearing after thymectomy for thymoma. Eur J Cardiothorac Surg 2005;28:22-25
6. Nakajima J, Murakawa T, Fukami T, Sano A, Takemoto S, Ohtsu H. Postthymectomy myasthenia gravis: relationship with thymoma and anti acetylcholine receptor antibody. Ann Thorac Surg 2008;86:941-945
7. Freshtand JB, Shaw RR. Malignant tumor of the thymus gland, myasthenia gravis developing after removal. Ann Intern Med 1951;34:1025-1035
8. Green RA, Booth CB. The development of myasthenia gravis after removal of thymoma. Am J Med 1958;25:293-302
9. Hoffacker V, Schultz A, Tiesinga JJ, Gold R, Schalke B, Nix W, et al. Thymomas after the T-cell subset composition in the blood: a potential mechanism for thymoma associated autoimmune disease. Blood 2000;96:3872-3879
10. Buckley C, Douek D, Newsom-Davis J, Vincent A, Willcox N. Mature, long-lived CD4+ and CD8+ T cells are generated by the thymoma in myasthenia gravis. Ann Neurol 2000;50:64-72

Chordoma is a rare, malignant neoplasm thought to develop from the notochord.  It most commonly occurs in the base of the cranium or the sacro-coccygeal region but around 15-20% affect the vertebral body.  Extra-lesional resection with or without adjuvant radiotherapy is generally accepted as the mainstay of treatment for this slow-growing tumour.  We present a case whereby a patient with a solitary L3 vertebral body lesion causing caudal compression was treated with spinal decompression and posterior stabilisation.  Pre-operative biopsy was not performed as the lesion was presumed to be a metastatic deposit from a co-existing renal mass.  Intra-operative biopsy, however, identified the tumour to be chordoma of the L3 vertebral body which would have been more appropriately treated with ‘en-bloc’ excision.  This case highlights the importance of pre-operative tissue diagnosis, and that, although rare (0.8 per 100,000), chordoma should always be considered.

INTRODUCTION

We present a case of chordoma, causing caudal compression, which was initially presumed to be a metastatic deposit from a renal cell carcinoma on the basis of radiological investigations.  Consequently the patient received initial surgical intervention in the form of spinal decompression with posterior stabilisation, where extra-lesional resection would have been more appropriate.

CASE REPORT

A 72-year old man presented with an 8-month history of lower back pain and increasing left leg pain and weakness.  An abnormal appearance of the L3 vertebral body on plain radiographs prompted CT investigation which suggested pathological fracture of L3 with posterior convexing of the vertebral body and epidural mass.  Also noted was a mass within the right kidney which was felt to be in keeping with the appearances of renal cell carcinoma.  MRI confirmed a solitary vertebral lesion causing posterior convexity of the vertebral body with extension into the pedicle, causing significant compression of the thecal sac and clumping of nerve roots. The patient was commenced on Dexamethasone.  Based on the radiological presumption that this was a secondary deposit from a renal cell carcinoma within the right kidney, plans were made for posterior spinal decompression and stabilisation, and tissue biopsy was felt to be unnecessary.  Pre-operative embolization of the tumour was attempted, however, no tumour enhancement was demonstrated during the aortogram, and embolization was therefore not performed.  Decompression and posterior stabilisation from T12 to L5 was performed and biopsies of L3 sent for analysis.  The biopsy revealed trabecular bone and marrow fat replaced by malignant tumour, in-keeping with chordoma, not the presumed diagnosis of renal cell carcinoma.

Fig. 1: MRI of lumbar spine showing mass causing compression of the cauda

The patient developed an ileus post-operatively but soon settled and his lower back pain gradually improved.  Following discharge, a plan was made for further investigation of the renal lesion.  Unfortunately his lower back pain returned 6 months later, and subsequent imaging showed enlargement of the lumbar spine mass causing further compression of the cauda.  (Figure 1).  This subsequently required further surgery in the form of tumour debulking with anterior stabilisation of L3.  Investigation of his renal mass is on-going.

DISCUSSION

Thought to arise from cellular remnants of the notochord (the mesodermal precursor of the vertebral column), chordoma is a rare, slow-growing, locally-invasive malignant tumour. The vast majority affect the base of the cranium and sacro-coccygeal region with round 15-20% involving the vertebral body(1,2).  It is generally accepted that ‘en-bloc’ surgical excision with wide, tumour-free margins is the treatment of choice.  Adjuvant radiotherapy may also be used post-operatively, however Chordomas are relatively radio-resistant and the value of radiotherapy in their treatment is still a matter of debate.  Chordomas are well known for their high local recurrence rates following surgery and distant metastases following treatment is reported in greater than 40% of patients (3,4,5,6).

It is extremely rare for chordoma to present in a lumbar vertebral body and this case report highlights an unusual presentation of spinal pathology. Much more common is the presentation of metastatic deposits from a primary tumour elsewhere.  In 2005, Patchell et al (7) published their randomised trial which established the superiority of direct decompressive surgery plus post-operative radiotherapy over radiotherapy alone for patients with spinal cord compression from metastatic disease.  Since then we have noticed a dramatic increase in the volume of referrals of patients with this condition.  In the case of our patient, it was assumed that this L3 lesion was metastatic deposit from renal cell carcinoma based on radiological appearances.  Pre-operative biopsy would have established the true nature of the disease process and altered management significantly.

Pre-operative work-up including tissue biopsy and MDT discussion is vital to guide management.  Although rare, chordoma and other primary bone tumours should always be considered as a potential diagnosis of a vertebral lesion, despite its location. With our experience of the increasing presentation of spinal metastatic disease, it is important to be aware that primary tumours of the spine still do occur even when pointers to metastatic disease are present. Three dimensional imaging can occasionally be misleading, and diagnostic assumptions cannot always be reliably made based on radiological investigations alone.

REFERENCES

1. Mindell ER. Chordoma. J Bone Joint Surg Am 1981;63:501–5
2. McMaster ML, Goldstein AM, Bromley CM, Ishibe N, Parry DM. Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control. 2001 Jan;12(1):1-11
3. Fuchs B, Dickey ID, Yaszemski MJ, et al. Operative management of sacral chordoma. J Bone Joint Surg Am 2005;87:2211–16
4. Sundaresan N, Galicich JH, Chu FC, et al. Spinal chordomas. J Neurosurg 1979;50:312–19
5. Samson IR, Springfield DS, Suit HD, et al. Operative treatment of sacrococcygeal chordoma. A review of twenty-one cases. J Bone Joint Surg Am 1993;75:1476–84
6. Cheng EY, Ozerdemoglu RA, Transfeldt EE, et al. Lumbosacral chordoma. Prognostic factors and treatment. Spine (Phila Pa 1976) 1999;24:1639–45
7. Patchell RA, Tibbs PA, Regine WF, Payne R, Saris S, Kryscio RJ, Mohiuddin M, Young B. Direct decompressive surgical resection in the treatment of spinal cord compression caused by metastatic cancer: a randomised trial. Lancet. 2005 Aug 20-26;366(9486):643-8

Inguinoscrotal bladder hernias are rare, occurring in 1-4% of all inguinal hernias. Massive inguinoscrotal bladder hernias, where >50% of the bladder is found in the hernia sac are extremely rare. Patients can suffer significant morbidity from such a hernia. These include sepsis, unilateral or bilateral ureteric obstruction, renal failure and strangulation with secondary ischaemia of the bladder wall and bladder rupture. Inguinoscrotal bladder hernias are most commonly diagnosed at the time of surgery. This may lead to significant complications for the patient, particularly if undetected during surgery.

INTRODUCTION

The urinary bladder is involved in 1-4% of all inguinal hernias (1,2), with the incidence rising to 10% in obese males between 50 (3). There is a 70% male predominance, with most occurring on the right side and are more commonly direct herniations (5). The inguinal and femoral areas account for 75% and 23% respectively, with 2% occurring outside the groin. Massive (where >50% of the bladder is out of its pelvic position) are extremely rare, with less than 120 reported in the literature.

CASE REPORT

A 56 year old male presented with a 12 month history of an intermittent swelling in his right hemi-scrotum, moderate storage lower urinary tract symptoms, and of late, manual compression of his scrotum to complete bladder emptying.

On physical examination, the abdomen was soft, with no flank mass or tenderness. There was a non-tender irreducible inguinoscrotal swelling. The prostate was mildly enlarged with a normal PSA and negative urine microscopy and culture. An ultrasound examination was unhelpful in aiding diagnosis and an MRI was performed (Figure 1)

Fig. 1: T2 weighted image showing large fluid attenuated right scrotal mass, exerting mass effect on the right testicle

Intra-operative findings (Figure 2) showed a paraperitoneal subtotal prolapse of the bladder through the internal inguinal ring, into the scrotum. Following reduction of the bladder to its normal anatomical position, a tension free mesh repair was performed. He had an uneventful recovery, with significant improvement in his symptomology.

Fig. 2: Bladder herniation (BH) with hernia sac, accompanied with vas deferens (Large arrow) and spermatic cord (Small arrow)

DISCUSSION

Small bladder hernias are usually asymptomatic while patients with large inguinoscrotal bladder hernias, as in our case, commonly report 2 stage micturition with the second stage precipitated by external compression of the inguinoscrotal region (5,8).

The pathophysiology of inguinoscrotal bladder hernias is conditioned by weakness of the abdominal and bladder walls. The presence of bladder outlet obstruction, such as prostatic enlargement or urethral stricture disease, increases intra-abdominal pressure during micturition which in turn favours herniation(9). Weakness of the pelvic floor or space occupying pelvic masses can also lead to herniation. Finally obesity maybe responsible and herniation could also follow trauma or surgical complications.

Complications of an inguinoscrotal bladder herniation include cystolithiasis, vesico-ureteric reflux, sepsis, unilateral or bilateral ureteric obstruction, renal failure and strangulation with secondary ischaemia of the bladder wall and bladder rupture(7,9). Oruç et al (6) reported an 11% incidence of urothelial malignancies in a case series of 116 patients with bladder herniations, the majority being bladder urothelium carcinoma followed by hyperplastic polyps and prostate cancer.

The pre-operative diagnosis of a bladder herniation is preferable to avoid potential iatrogenic surgical complications. Injury to the bladder occurs in approximately 12% of patients(4,7). A voiding cystogram should be performed in patients whom a bladder hernia is suspected from the history. Characteristically, a dumbbell shaped bladder is seen on cystography. Investigative modalities for scrotal cystocoeles can include ultrasonography, computerised tomography and magnetic resonance imaging. Imaging aids diagnosis and surgical planning, as anatomical deformities and possible complications can be identified (9). Tissue with similar consistency to the bladder within the hernial sac, change in the dimensions in the inguinal canal pre- and post-micturition and continuity between bladder and the hernia contents may indicate a bladder herniation (10).

The majority of bladder hernias are discovered intra-operatively, with only 7% diagnosed pre-operatively and 16% post-operatively owing to complications(6). Inguinal bladder hernias are classified as intraperitoneal, paraperitoneal or extraperitoneal. Most cases are paraperitoneal (60%), with an extraperitoneal portion of herniated bladder only partly accompanied by peritoneum (7,9). Purely intraperitoneal cases are rare, whilst extraperitoneal cases account for 30% (7).

An inguinoscrotal bladder hernia encountered during hernia repair should be reduced and a standard hernia repair performed. Bladder resection should be reserved for a necrotic portion of bladder, or the presence of a bladder malignancy (4,7,9,10).

The rarity of the condition endows this with interest and should be a timely reminder to the surgeon lest he accidentally damages the bladder during surgery. The injury itself may not be noticed until the post-operative period and in cases of intraperitoneal trauma, a fatal result is a real possibility.

REFERENCES

1. Fisher PC, Hollenbeck BK, Montgomery JS et al. Inguinal bladder hernia masking bowel ischaemia. Urology. 2004;63:175-6
2. Huerta S, Fairbanks T, Cinat M. Incarcerated vesicoinguinal hernia presenting with gross haematuria. J Am Coll Surg. 2005;201:992-3
3. Izes BA, Larsen CR, Izes JK et al. Computerised tomographic appearance of hernias of the bladder. J Urol. 1993;149:1002-1005
4. Gurer A, Ozdogan M, Ozlen N et al. Uncommon contents in groin hernia sac. Hernia 2006;10:152-155
5. Wagner AA, Arcand P, Bamberger MH. Acute renal failure resulting from huge inguinal bladder hernia. Urology. 2004;64:156-7
6. Oruç MT, Akbulut Z, Ozozan O et al. Urological findings in inguinal hernias: a case report and review of the literature. Hernia 2004;8:76-9
7. Gomella LG, Spires SM, Burton M et al. The surgical implications of herniation of the urinary bladder. Arch Surg. 1985; 120:964-7
8. Shelef I, Farber B, Hertzanu Y. Massive bladder hernia: ultrasonographicimaging in two cases. Br J Urol. 1998;81:492-3
9. Bisharat M, O’Donnell ME, Thompson T et al. Complications of inguinoscrotal bladder hernias: a case series. Hernia. 2009;13:81-84
10. Casas JD, Mariscal A, Barluenga E. Scrotal cystocele: US and CT findings in two cases. Comput Med Imaging Graph.1998;22:53-6

A 66 year old woman presented to A&E with per vagina bleeding and a mass protruding from the vagina. The patient was examined under anaesthesia, which revealed vaginal prolapse with evisceration of approximately 20-30 cm of bowel. The patient had received an abdominal hysterectomy 30 years ago for menorrhagia. In the last decade, the patient had experienced other recurrent episodes of prolapse (cystocoele and retrocoele). Vaginal vault evisceration is a recognised rare complication of hysterectomy and is a gynaecological emergency. This patient’s condition was rapidly recognised and surgically managed. The repair was achieved in two surgeries. Initially, the small bowel was re-inserted into the peritoneal cavity through the vaginal wall defect and the vaginal defect repaired. After sufficient time for healing, a sacrocolpopexy was performed to repair the prolapse.

INRTRODUCTION

The first report of vaginal evisceration was described by Hyernaux in 1864, as disruption of the anterior wall of the proximal vagina, resulting in prolapse of the abdominal contents (1). Since then, there have been just over 120 reports in the literature, although in reality some cases may go unreported (1). Vaginal evisceration is a rare postoperative complication of hysterectomy (regardless of the surgical approach) in women of this age group that carries a high risk of morbidity and mortality and requires rapid surgical intervention. There are different aetiologies depending on the age group – pre-menopausal vs postmenopausal (2). In post-menopausal women vaginal evisceration tends to occur after a hysterectomy. Interestingly, in pre-menopausal women there have been reports of vaginal evisceration following transvaginal ultrasonography (3). The management of vaginal evisceration involves prevention and emergency surgery. There are various surgical approaches (abdominal, vaginal or combined laparoscopic abdominal). The use of each approach depends on the viability of the bowel, whether the defect in the vaginal wall is sufficiently large to replace the bowel back into the peritoneal cavity and on any evidence of foreign bodies in the abdominal cavity.

CASE REPORT

A 66 year old woman presented to A&E with per vagina bleeding, abdominal pain and a mass protruding from the vagina. Prior to this presentation, the patient had suffered for the past decade with cystocoele and retrocoeles. Three decades ago the patient had received an abdominal hysterectomy in order to relieve her symptoms of menorrhagia. Other than arthritis, the patient does not suffer from any other medical condition and was in good health.

On examination the patient seemed well, but her abdomen was distended and tender in the suprapubic, left and right iliac fossa regions. Bowel sounds were absent. On inspection of the genital region there was evidence of vaginal prolapse with a 20-30 cm evisceration of small bowel via a vaginal vault defect. The patient was immediately referred to the gynaecology team.

Fig.1 Vaginal evisceration through vaginal vault defect at presentation.

Prior to the emergency operation which aimed to salvage the small bowel, the patient was given antibiotics. The aim of the surgery was to reduce the prolapsed small bowel and re-suture the vaginal wall defect. On inspection by the consultant gynaecologist, the small bowel appeared slightly dusky and an opinion was sought from a consultant general surgeon (who deemed the bowel healthy). The small bowel was replaced through the vaginal wall defect and vaginal wall biopsies taken. The defect in the vaginal wall was closed using vinyl. The patient’s vagina was catheterised and packed. The patient recovered well in the first few days after the operation, though she did have some trouble maintaining the catheter in situ, due to the still uncorrected vaginal prolapse. The catheter was removed on day 5 post-operatively, but the patient had developed a urinary tract infection while catheterised and had to be on i.v. augmentin for this infection. The patient was discharged on day 8, but was to come back 6-8 weeks later for an elective sacrocolpopexy to correct the vaginal wall prolapse.

Fig. 2 After complete reduction of small bowel evisceration and correction of vaginal vault defect.

Initially exploratory laparoscopy was performed, the pelvis appeared normal and the consultant decided to perform an abdominal sacrocolpopexy. A Pfannenstial incision was made, small bowel adhesions to right pelvic side wall were noted and divided. The peritoneum between sacrum and posterior vaginal wall was exposed, allowing prolene mesh to be attached to the vaginal wall with prolene and vinyl sutures. The mesh was also sutured to the anterior sacrum. The entire mesh was covered with peritoneum. The rectus sheath was sutured using vinyl and the skin closed with staples (with a drain in situ). Cystoscopy was used to verify the function of the ureters, which revealed both were working well. The patient recovered quickly, there were no post-operative complications and was discharged on day 2 post-operatively.

DISCUSSION

Vaginal evisceration can be a rare complication of hysterectomy which requires urgent surgical attention. This patient had had a hysterctomy many years ago and it is unlikely her vaginal evisceration was a consequence of this surgery directly, but hysterectomy can lead, for example to changes in the vaginal axis. The vagina may come to lie in a more descended position, as it has lost part of its more proximal attachments. This new position combined with factors such as cystocoeles and retrocoeles and their management (for example the insertion of pessaries) may predispose the vagina walls to more “wear and tear” damage. These are risk factors that are likely to have contributed to this lady’s transvaginal evisceration, together with her increasing age susceptibility.

Vaginal evisceration if not recognised quickly can lead to peritonitis and bowel ischaemia. In this lady it quickly recognised and managed well surgically. However, it is important to make emergency doctors aware of these complications and encourage their proper management. When the patient is received by an emergency department and the problem is determined, the emergency doctors must not delay the administration of broad spectrum antibiotics in preparation for taking the patient to theatre and ensure the prolapsed bowel is covered in saline-soaked gauze, as this improves the bowel chances of viability (4).

This case illustrates the need for rapid recognition of vaginal evisceration and its appropriate management in order to avoid morbidity and mortality to the patient.

REFERENCES

1. Gandhi P, Jha S. Vaginal vault evisceration. The Obstetrician & Gynaecologist 2011 13:231–237
2. Rajesh S, Kalu E, Bong J, Wales N. Evisceration 5 years post abdominal hysterectomy. J. Obstet. and Gynaecol. Res. 2008 34:425-427
3. Lundvall L, Flemming J, Roed H, Ottosen C, Ewertsen, Henriksen BM. Vaginal rupture caused by transvaginal ultrasonography in follow-up for ovarian cancer. BMJ Case Rep. 2009
4. Lawson L, Patterson L, Carter K. Transvaginal evisceration progressing to peritonitis in the emergency department: a case report. International Journal of emergency medicine 2011 4:66

ABSTRACT

Subdural hematoma (SDH) is a common neurosurgical pathology, characteristically recognised on plain CT and can be treated with simple and effective surgical intervention. In contrast, dural metastatic adenocarcinoma of the prostate with SDH and malignant extension into the subdural membranes is extremely rare. We describe the case of a 62-year old Caucasian male, provide a brief review of the literature, and explore the potential role of neoangiogenesis and disseminated intravascular coagulopathy in SDH development.

INTRODUCTION

Poorly differentiated metastatic prostate carcinoma to the axial skeleton and visceral organs is common (1), while metastasis to cerebral parenchyma is uncommon (0.63% of cases) and is associated with poor prognosis (2). A recent study found that dural prostate metastasis were more frequent (19.5%) than other cancers (3). Despite this finding however, there is a scarcity of literature describing the presence of chronic subdural haematoma (cSDH) associated with dural metastasis, malignant invasion of the subdural membranes and concurrent DIC.

CASE REPORT

A 62-year old male presented to the emergency department of our institution with epistaxis, anaemia (Hb: 48g/L) and thrombocytopenia (platelets: 52×10⁹/L). Past history included a diagnosis of metastatic prostate adenocarcinoma 7 years earlier. He denied headaches and there were no neurological deficits other than mild cognitive impairment. He was noted to be coagulopathic with an INR of 1.5, despite platelet, fresh frozen plasma and red blood cell transfusion, his coagulopathy ultimately worsened. His level of consciousness deteriorated and a CT Brain was performed identifying acute on chronic SDH (Figure 1 & 2).

Fig. 1: Right acute on chronic SDH

Fig. 2: Right acute on chronic SDH

He received further platelet and plasma transfusion to correct his INR of 3.5, and was taken to theatre where a right parietal craniotomy and left parietal burrhole were performed to evacuate the SDH. Intraoperatively, the dura was noted to be thickened and subdural membrane tissue was obtained for histopathology. This was consistent with metastatic adenocarcinoma of the prostate (Figure 3 & 4). The patient recovered well day one post-operatively with no neurological deficits. His level of consciousness rapidly deteriorated on day two post-operatively, with no evidence of haematoma recurrence or neurosurgical complication on repeat CT Brain. The patient subsequently died on post-operative day four.

Fig. 3: Histopathology Right subdural membranes: extensive infiltration by poorly differentiated adenocarcinoma cells with glandular infiltration (x10).

Fig. 4: Histopathology Right subdural membranes: Immunoperoxidase staining for PSA (x4)

DISCUSSION

From our review, there have been five reported cases of SDH secondary to metastatic prostate carcinoma to the dura (4-8). Only two of these (including the current case) have shown extension of malignant cells into the subdural membranes.

In the setting of metastatic cancer refractory to therapeutic intervention, it is important to ensure optimal quality of life. Evacuation of mass lesions in the subdural space is a procedure associated with relatively low morbidity and mortality and will improve cognitive ability. Concurrent DIC in our patient may have compromised his prognosis significantly.

A characteristic feature of malignant prostate carcinoma is its ability to promote tissue invasion (through MMP9), cancer cell survival (Caveolin-1 upregulation and promotion of cell survival through Akt-mediated activit and angiogenesis. Endothelial cell dysfunction in tumor microvasculature results in increased vascular permeability (9). In unison, these features enable invasion of the dura, underlying membranes and promote leakiness that is compounded by coagulopathy.

DIC represents the result of a widespread activation of coagulation pathways and is the most frequent coagulation complication in prostate cancer (10). In acute DIC there is a massive generation of thromboplastic material, as well as a consumption of haemostatic elements. Compensatory mechanisms are not sufficient to restore coagulation proteins and platelets. Consequently, transfusion of blood products is often indicated.

It has been postulated that SDH development, in the presence of dural prostate metastasis, might be secondary to dural venous obstruction, hemorrhagic effusion (due to dural metastasis) or an angiodesmoplastic response of the dura to the invasion by carcinomatous cells. We hypothesize that tumor cell invasion of SDH membranes (with permeable microvasculature) in association with coagulopathy (secondary to DIC due to disease progression) may also be potential mechanisms.

In patients with refractory metastatic prostate adenocarcinoma, concurrent DIC and a deteriorating level of consciousness, a differential diagnosis of SDH would enable expedient diagnosis and review by a neurosurgical team.

REFERENCES

1. Rubens RD, Coleman RE. Bone Metastases. In Abeloff MD, Armitage JO, Lichter AS, Niederhuber JE. Clinical Oncology. New York: Churchill Lingston, 1995: 643-665
2. Tremont-Lukats IW, Bobustuc G, Lagos GK, Lolas K, Kyritsis AP, Puduvalli VK. Brain Metastasis from Prostate Carcinoma: The M. D. Anderson Cancer Center Experience. Cancer 2003; 98:363-368
3. Laigle-Donadey F, Taillibert S, Mokhtary K, Hilebrand J, Delattre JY. Dural Metastases. Journal of Neuro-Oncology 2005; 75:57-61
4. Tomlin JM, Alleyne C. Transdural Metastasis from Adenocarcinoma of the Prostate mimicking SDH: Case Report. Surg Neurol 2002;58(5):329-31
5. Patil S, Vernon A, Hosseini P, Bates R, Guthikonda B, Desouza R. Metastatic prostate cancer mimicking chronic subdural haematoma: a case report and review of the literature. J La State Med Soc 2010; 164:203-205
6. Dorsi MJ, Zenonos G, Hsu W, Huang J. Dural prostate adenocarcinoma metastasis with subdural haematoma mimicking the appearance of an epidural hematoma. Clinical Neurology and Neurosurgery 2010; 112:501-504
7. Bucci MN, Farhat SM. Metastatic adenocarcinoma of the prostate as a cause of subdural hematoma. Journal of Urology 1986; 135(4):803-804
8. Tasaki K, Shima T, Matsumura S, Okada Y, Nishida M, Yamada T, Okita S, Kagawa R. A case of Subdural effusion secondary to dural Metastasis of prostatic cancer: case report. No Skinkei Geka 1990; 18(6):539-542
9. Hashizume H, Baluk P, Morikawa S, McLean JW, Thurston G, Roberge S, Jain RK, McDonald DM. Openings between defective endothelial cells explain tumor vessel leakiness. American Journal of Pathology 2000; 156:1363-1380
10. De la Fouchardiere C, Flechon A. Droz JP. Coagulopathy in prostate cancer. Netherlands. The Journal of Medicine. 2003; 61(11):347-354

Laparoscopic surgery in a patient with Partial Situs Inversus may pose interesting challenges to the surgeon. Here we report a case of a morbidly obese young female with partial situs inversus who  underwent Laparoscopic Vertical Sleeve Gastrectomy (LSG). The peri-operative challenges very many and these have been enumerated. The mirror image approach is recommended in such cases for a successful surgery which was not employed in this case. Postoperative barium swallow was normal and the patient has been on regular follow up.

INTRODUCTION

Prevalence of massive obesity continues to increase and only bariatric surgery has succeeded in providing sustained weight loss (1).  Laparoscopic Sleeve Gastrectomy (LSG) accounts for approximately 30% of bariatric procedures performed worldwide; and its coexistence with partial situs inversus is one in a million. Most of the global documented procedures in situs inversus involve gastric bands, gastric bypass and cholecystectomies (2). Very limited/solitary data on LSG in partial situs inversus with this being the first of its kind in India.

Situs Inversus partial is a congenital developmental anomaly wherein the abdominal organs are reversed or mirrored to the opposite side of the body through the sagittal plane. In contrast, situs inversus totalis is a similar condition in which both the thoracic and abdominal contents are reversed (2). Transmitted through an autosomal recessive inheritance, these anomalies have been in vogue since the 17^th century.

CASE REPORT

A 32 yr old morbidly obese female weighing 111 Kg and BMI of 41.41 was electively admitted to the hospital. She had tried a number of diet and exercise programmes but failed to sustain reduced weight loss. She was a known case of partial situs inversus as diagnosed earlier during her pregnancy few years ago. She underwent standard pre-operative workup including physician, psychological and anaesthetic assessment satisfying our selection criteria for surgery.  This Grade 3 obese patient suffered from cough, dyspnoea and chest pain. She had no history of diabetes or Ischemic heart disease though blood pressure was 130/80 mmHg. Her general examination and preoperative investigations were within normal limits. Heart sounds were normal and on the left side in the chest. Abdominal girth was increased. Preoperative barium follow through under fluoroscopic control showed situs inversus with malrotation of gut. Stomach was on the right side, normal in shape, increased size, normal mucosal pattern, no ulcer. Duodenal  C Loop was opposite. Small  bowel on right side and normal, no narrowing. Ileocecal  junction, caecum and appendix was in left iliac fossa. Descending and sigmoid colon located on the right side close to ascending colon. CT scan of chest/abdomen and pelvis showed enlarged mediastinal  lymph nodes and situs inversus. Patient was admitted one day prior to surgery and LSG was performed in January 2011. Following general anaesthesia, patient was placed in reverse Trendelenberg position. Primary operating surgeon stood on the left side of the patient as contrast to usual right side. Trocar positions were also mirror imaged. Operative feel was uncomfortable; as in a right handed person writing by a left hand. Entire upper quadrant was occupied by the liver. Enlarged spleen was in the right side. Orogastric tube negotiation was difficult because of the position of the stomach. Primary surgeon had to come to right side to reach to the right crus. Most of the time single hand technique had to be used instead of both hands. Surgical time was 90 minutes.

Fig. 1: Upper GI contrast studies

Postoperative upper GI contrast study was performed and showed no evidence of leak and a well designed vertical sleeve of the stomach. The post operative period was uneventful and patient was discharged after 4  days.  She was given standard advise to remain on liquid diet for 2 weeks, followed by 2 weeks of soft diet, before reintroducing solids. Her present weight is 75Kg and BMI is 28 Kg/m² .

DISCUSSION

Situs Inversus is a rare congenital developmental anomaly with autosomal recessive inheritance wherein the position of organs in chest and abdomen are reversed along sagittal plane. The incidence of partial situs inversus in less than 1 in 22000. If associated with primary ciliary dyskinesia, sinusitis and bronchiectasis and infertility in males, it is called Kartagener Syndrome found in 25% of patients (3). Incomplete or partial situs inversus is invariably associated with cardiac abnormalities like septal defects, pulmonary arterial stenosis, tetralogy of Fallot, transposition of great vessels, and alimentary tract problems like atresia and stenosis of duodenum (4).  Most of the individuals are unaware of their usual anatomy until they seek medical attention for an unrelated condition, when it is discovered incidentally. In our patient, though there was cough, dyspnoea and chest pain, routine Chest X-ray was normal as the defect involved only abdominal organs. Fortunately, the patient was aware of her condition. Our current practice involves routine chest X-ray and Sonography of abdomen.

Laparoscopic bariatric surgery in situs inversus has been reported in very few cases, wherein one patient underwent laparoscopic gastric banding (6), laparoscopic gastric bypass (5) and LSG each (3). In India, so far this case stands as the first of its kind in partial situs inversus (2,4).

Certain aspects of this type of surgery are challenging and it is recommended that an experienced laparoscopic surgeon carry out the procedure. Using a mirror image approach to all parts of the operation enabled us to successfully complete the procedure. Using this technique, our operating time was only slightly longer than normal (120 mins v/s 60 mins). LSG is thus a safe and feasible surgical procedure in situs inversus patients with very good results.

REFERENCES

1. Buchwald H, Williams SE. Bariatric Surgery Worldwide 2003. Obes Surg 2004; 14:1157-64
2. Kobus C, Targarona EM, Bandahan GE et al. Laparoscopic surgery in situs inversus: a literature review and a report of laparoscopic sigmoidectomy for diverticulitis in situs inversus. Langenbeck’s Arch Surg 2004; 389:396-9
3. Varano NR, Merkin RJ. Situs Inversus: review of literature. Report of 4 cases and analysis of clinical implications. J Int Coll Surg 1991; 33:131-5
4. Ersoy E, Koksal H, Ege B. Laparoscopic gastric banding for morbid obesity in a patient with situs inversus totalis. Obes Surg 2005; 15:1344-6
5. Gibbs KE, Forrester GJ, Vemulapalli P et al. Intestinal malrotation in a patient undergoing laparoscopic gastric bypass. Obes Surg 2005; 15:703-6
6. Catheline JM, Rosales C, Cohen R et al. Laparoscopic sleeve gastrectomy for a super super obese patient with situs inversus totalis. Obes Surg 2006; 16:1092-5

Persistent Mullerian duct syndrome (PMDS) with transverse testicular ectopia (TTE) is a rare syndrome. We report a case of PMDS associated with TTE in a 38 year old male discovered during surgery for a left inguinal hernia. Hernioplasty with excision of the persistent mullerian duct structures was done and the excised structures sent for histopathologic examination (HPE) which revealed PMDS.

INTRODUCTION

The paramesonephric duct or Mullerian duct first appears in the human embryo at the 10mm stage and begins to regress in the male due to Mullerian inhibiting substance (MIS) (1).  Apart from various disorders of regression, there is also a well documented syndrome known as the PMDS, caused by the failure of regression of the Mullerian duct structures. This is a rare form of male pseudohermaphroditism, characterized by the presence of a uterus and fallopian tubes, in phenotypically and genotypically normal males. The association between PMDS and TTE is even more uncommon. Von Cenhossek in 1886 described the first case of TTE and in 1895 Jordan described the syndrome of TTE with PMDS (2).

CASE REPORT

A 38 year old married man with two children presented with a left inguinal hernia (LIH) and absence of right testis since birth. Examination of the groins showed normal phallus, LIH, empty right hemiscrotum, impalpable right testis and normal left testis. The hernia was indirect with cough impulse and reducibility. The contents felt like thickened omentum. He was diagnosed as having LIH with undescended right testis (UDT). Patient was advised LIH repair and imaging for undescended right testis. Since patient was not willing for the imaging evaluation of the right testis and insisted more on the hernia repair to be done first, he was booked for a Lichtenstein’s repair.

Fig. 1: Resected Mullerian duct derivatives of PMDS patient

At exploration through the left inguinal incision, an irregular mass was identified within the indirect hernia sac. The sac contained two spermatic cords, separated by a rudimentary uterus, with one cornua leading to an ovary like structure (Fig.1). We thought it was uterus, fallopian tube and ovary. The entire mass was removed carefully without damaging the left spermatic cord and sent for HPE. The associated hernia was treated by Lichtenstein’s repair. We were surprised about these findings and initially had no clues, until we searched the literature and found about PMDS. The HPE report was confirmative, showing the presence of normal rudimentary uterus, testis (not ovary, as we thought) showing germ cell atrophy (Sertoli cell only), normal epididymis and spermatic cord (vas deferens).

DISCUSSION

During normal embryogenesis, differentiation of an indifferent gonad into a testis or ovary depends on the sex chromosomes. MIS and testosterone are the two principal hormones produced by the testis, the former causes the regression of Mullerian ducts, and the latter induces the development of internal male genital structures. Contrastingly, female development is an autonomous process, and does not require the presence of either gonad or hormone. Male pseudohermaphroditism results from the defective formation or action of androgens or MIS.

The male pseudohermaphrodite has male gonads and karyotype, but varying degrees of virilisation of the internal and external genital tracts. PMDS represents a small fraction of this broad spectrum of male pseudohermaphroditism, characterized by the presence of well developed or rudimentary uterus, cervix, vagina and fallopian tube in normal 46xy male.

Normally the testis is located in the scrotum at birth. Ectopic testes have been reported at various sites including superficial inguinal pouch, suprapubic, femoral, perineal region and the base of penis (3).

Migration of the testis to the opposite side where both testes pass through the same inguinal canal is known as TTE, 100 cases of which have been reported (4).

In most cases the PMDS is discovered during surgery for inguinal hernia. The pre-operative diagnosis of this condition is practically impossible. Routine pelvic ultrasound was recommended by Adamsbaum et al, in bilaterally cryptorchid patients and in patients with inguinal hernia of unusually hard consistency (5). Recently, with the advent of laparoscopic exploration of UDT, the diagnosis of TTE was possible.

In a case of PMDS with TTE, the optimal surgical approach includes tissue biopsies, hernoitomy or hernioplasty, orchidopexy and excision of Mullerian duct remnants. Some authors believe that excision of Mullerian duct structures and scrotal orchidopexy are not possible without sacrificing the vas deferens. In our patient it was possible to dissect the vas deferens from the rudimentary uterus and remove the Mullerian duct structures without risking the vas deferens. This approach is reported by many authors as it preserves the fertility of the individual (2,5,6).

There are  three anatomic variants:

A) in the most common male type, one testis is usually foundwithin the scrotum; the uterus and ipsilateral fallopian tube are either in the inguinal canal or can be brought into it by gentle traction in the presenting testis.

B) in some cases, the contralateral testis and the tube are also in the hernia sac; transverse testicular ectopia can also occur.

C) the least common form, or female type, is characterized by bilateral cryptorchidism with testis embedded in the broad ligaments in an ovarian position with respect to the uterus, which is fixed in the pelvis (7).

In conclusion, etiopathogenesis, differential diagnosis and operative strategies in PMDS have not been completely defined. Orchidopexy is recommended, if there is no evidence of malignancy. Removal of uterus and its derivatives can be performed provided, care is taken not to damage the vas deferens, if there is some spermatogenesis. Hence, surgeons dealing with inguinal hernia should suspect PMDS in the case of adult bilateral cryptorchidism.

REFERENCES

1. Josso N, Picard JY, Tran D. The Anti-Mullerian hormone. Recent Prog Horm Res 1977, 33:117-67
2. Martin EL, Bennett AH, Cromie WJ. Persistent Mullerian duct syndrome with transverse testicular ectopia and spermatogenesis. J.urology, 1992, 147:1615-1617
3. Gallady ES, Redman JF. Transvere testicular ectopia. Urology, 1982, 19:181-186
4. Mouli K, McCathy P, Ray V, Rosanthat IM. Persistent mullerian duct syndrome in a man with transverse testicular ectopia. J Urology, 1988, 139:373-375
5. Adamsbaum C, Rolland Y, Josso N, Kalifa G. Radiological findings in three cases of persistent mullerian duct syndrome. Pediatr Radiology, 1993, 23:55-56
6. Loeff DS, Imbeaud S, Reyes HM, Meller JL, Rosenthal IM, surgical and genetic aspects of persistent mullerian duct syndrome. J.Pediatric Surg, 1994, 29:66-65
7. Mohammadi MS, Heidarpour M, Dadkhah A, Rezvani M. Persistent mullerian duct syndrome with an irreducible inguinal hernia.Urol J. 2009;6:298-300

Isolated colonic tuberculosis (TB) is rare, and the symptoms are nonspecific making early diagnosis and management difficult. Although colonoscopy and biopsy is an important diagnostic modality, the features are variable and the distinction from other conditions of the colon, especially Crohn’s disease and cancer, may be impossible without surgical resection. We report a case of ascending colon TB which was mistaken for colonic cancer during colonoscopy, with non-specific results on biopsy. The diagnosis was finally made when the histopathology report was received. The diagnostic dilemma of colonic TB is discussed.

INTRODUCTION

Abdominal tuberculosis (TB) is not uncommon in the developing world, but isolated colonic TB is rare; it has been estimated that 2-3% of patients with abdominal TB have isolated colonic involvement (1). It also accounts for 10.8% of all gastrointestinal TB with the commonest affected site being the transverse colon, followed by rectum and ascending colon (2). It commonly presents with vague abdominal pain, fever, weight loss, diarrhea and abdominal mass. In more than two thirds of affected patients, there is no evidence of pulmonary TB (3). We report a case of ascending colon tuberculosis masquerading as carcinoma.

CASE REPORT

A 40-year-old male presented with 6-month history of vague right-sided abdominal pain, diarrhoea, and weight loss. Clinical examination revealed cachexia, pallor but no lymphadenopathy or jaundice. He was apyrexial with a palpable mobile mass in the right hypochondrium which was non-tender and measured 7×5cm. Blood tests revealed hypochromic microcytic anaemia. Tumor markers, renal and hepatic panels were normal. His chest x-ray was normal, but computed tomography (CT) scan of the abdomen revealed a large polypoid mass lesion in the right colon extending from the ileo-caecal valve with lumen-narrowing and mildly enlarged pericolic lymph nodes. The liver was grossly normal. Colonoscopy revealed a polypoidal mass in the proximal part of the ascending colon. Biopsies revealed inflammatory changes but no evidence of malignancy. Despite negative colonoscopic biopsies, a provisional diagnosis of right colonic cancer was made and he was prepared for right hemicolectomy.

Fig. 1: Cut section shows polypoidal growth arising from the ascending colon

On laparotomy, the mass in the subhepatic area was recognized with minimal free straw-colour peritoneal fluid. A provisional diagnosis of carcinoma colon was made. Hence, a right hemicolectomy was performed with an ileo-transverse anastomosis, and the specimen was sent for gross examination and histopathology (Figure 1 and Figure 2).  Histopathology revealed caseating granulomas diagnostic of TB, with no evidence of malignant cells. He was started on anti-tuberculous therapy consisting of daily doses of isoniazid 300mg, rifampicin 600mg and ethambutol 900mg for two months, followed by four months of isoniazid and rifampicin therapy; the whole treatment lasted six months. The patient made a complete recovery without complications and is currently on regular follow-up.

DISCUSSION

Abdominal TB continues to be a major health problem in the developing world. The incidence of colon TB is increasing with the rise in numbers of high risk patients such as HIV-infected individuals, patients with chronic renal disease, and immunosuppressed patients on immunosuppressive or prolonged steroid therapy. Most reported colonic TB cases in non-immunosuppressed individuals come from the Indian subcontinent (1-3). The most common presenting symptom and abnormal physical sign are abdominal pain and abdominal mass (90% and 58% of patients, respectively) (4). Varied and nonspecific presenting symptoms make early diagnosis difficult and elusive with subsequent delays in management. To avoid such a dangerous delay in management, some authors recommend a trial of anti-tuberculous chemotherapy in patients with high clinical suspicion on the basis of colonoscopic appearance alone, even in the absence of the classic features of TB, after ruling out other causes, especially cancer and Crohn’s disease by histopathological examination (1,3).

TB and colon cancer may, however, coexist (4,5). This coexistence may be of high frequency necessitating the need for epidemiological and histopathological investigations into the aetiological relationship between the two diseases (6). Colonoscopic examination with combined histopathologic and bacteriologic study of biopsy specimens is a powerful tool for the diagnosis of intestinal TB in 60% of patients without the need for resectional surgery (1). However, the colonoscopic features are quite variable, but typically are those of circumferential ulceration or a single ulcer with an elevated or nodular margin surrounded by hyperemic mucosa (3). Other colonoscopic features include nodules, deformed segment of the colon, polypoid lesions or pseudopolyps, strictures, and less commonly, fibrous bands forming mucosal bridges (3). Like in this case, tuberculous lesions may mimic carcinoma in 20% of cases and acid-fast bacilli (AFB) may not be isolated at all (4). Nevertheless, polymerase chain reaction (PCR) analysis of a colonic biopsy may reveal AFB of the mycobacterium tuberculosis species. It is a rapid, sensitive, and specific method in the diagnosis of intestinal TB. It is also valuable in the differentiation between intestinal TB and Crohn’s disease (7).

Imaging plays a diagnostic role with features that suggest the correct diagnosis. Such features are cecal amputation, ileocecal thickening and inflammation, shortening of the ascending colon, gaping of the ileocecal valve, mesenteric adenopathy, a misty mesentery, diffuse omental infiltration, loculated high-density ascites, an enhancing peritoneum with or without an omental line, nodularity of the surface of the mesenteric leaves, and transperitoneal permeation (8). Other commonly observed radiological findings include strictures (54%), colitis (39%) and less frequently polypoid lesions (7%) (8). Complications noted were in the form of perforations and fistulae in less than 20% of cases (2).

This case highlights the ease with which colonic TB may be mistaken for colonic cancer and the dilemma of diagnosing it radiologically and endoscopically. The clinical features of both these conditions are identical and, occasionally, only histopathology may provide the final diagnosis.

REFERENCES

1. Bhargava DK, Kushwaha AK, Dasarathy S, Shriniwas, Chopra P: Endoscopic diagnosis of segmental colonic tuberculosis. Gastrointest Endosc; 1992; 38: 571–4
2. Nagi B, Kochhar R, Bhasin DK, Singh K: Colorectal tuberculosis. Eur Radiol; 2003; 13(8): 1907-12
3. Alvares JF, Devarbhavi H, Makhija P, Rao S, Kottoor R: Clinical, colonoscopic, and histological profile of colonic tuberculosis in a tertiary hospital. Endoscopy; 2005; 37(4): 351-6
4. Kaushik R, Sharma R, Attri AK: Coexisting tuberculosis and carcinoma of the colon: a report of two cases and a review of the literature. Trop Gastroenterol; 2003; 24(3): 137-9
5. Leong AF, Seow-Choen F, Goh HS: Colorectal cancer and intestinal tuberculosis. Ann Acad Med Singapore; 1993; 22(6): 934-6
6. Jain BK, Chandra SS, Narasimhan R, Ananthakrishnan N, Mehta RB: Coexisting tuberculosis and carcinoma of the colon. Aust N Z J Surg; 1991; 61(11): 828-3
7. Gan H, Ouyang Q, Bu H, Li S, Chen D, Li G, et al.: Value of polymerase chain reaction assay in diagnosis of intestinal tuberculosis and differentiation from Crohn’s disease. Chin Med J (Engl): 1995; 108(3): 215-20
8. Jadvar H, Mindelzun RE, Olcott EW, Levitt DB: Still the great mimicker: abdominal tuberculosis. AJR Am J Roentgenol; 1997; 168(6): 1455-60

Metal on metal hip (MoM) resurfacing / replacement has recently been one of the most discussed topics in orthopaedics. Significant revision rates and complications are associated with MoM. We report a patient presenting with infection of a total hip prosthesis in conjunction with a psoas abscess in which the presentation is complicated by radiological evidence of suspected metallic debris in the lower limb tissues and elevated chromium and cobalt levels, suggestive of metallosis secondary to MoM.

INTRODUCTION

Metal on metal hip (MoM) resurfacing / replacement has recently been one of the most discussed topics in orthopaedics. Revision rates are high according to the British NJR database (1). Complications related to MoM include metallosis, pseudotumour and ALVAL (2).

We present a case of psoas abscess and infected hip prosthesis in a patient with suspected metallosis secondary to metal on metal total hip replacement. To our knowledge there has been no such cases in the literature.

CASE REPORT

A 49 year old male patient presented to the medical admissions unit with a one week history of left hip and thigh painful swelling. The only past medical history was bilateral metal on metal total hip replacements four years prior, the indication osteoarthritis (figure 1). The patient had no problems regarding the hips since surgery.

Fig 1. AP Pelvis radiograph demonstrating bilateral metal on metal total hip replacements

The patient was found to have gross swelling of the entire left leg, with overlying erythema. Observations revealed a low grade pyrexia. Blood investigations showed a neutrophilic leukocytosis with deranged renal and liver function, and prolonged INR. Blood analysis had been normal the year before. The clinical impression at time of admission was DVT or sepsis. The key differential diagnoses included deep vein thrombosis, cellulitis, necrotising fasciitis, septic arthritis, metallosis, and psoas abscess.

The patient underwent a Doppler scan which ruled out a DVT, then a CT scan of the left hip and thigh (figure 2). This demonstrated generalised superficial oedema with a small collection over the lateral thigh, and an irregular low density appearance of the gluteus and psoas muscles. With progressive deterioration in clinical state, the patient underwent ultrasound scanning of the thigh and hip. This demonstrated a small hip effusion with a large gas containing echogenic collection in the left psoas muscle (figure 3).

Fig 2. Axial CT demonstrating an irregular low density appearance within the left psoas (red arrow) and gluteal muscles (blue arrow)

Fig 3. USS of the hip demonstrating a large echogenic collection in the left psoas containing gas.

The patient underwent ultrasound guided drainage of 300ml of pus. Due to ongoing clinical concerns regarding the possibility of metallosis, the patient underwent blood sampling for cobalt and chromium levels, and was imaged with MRI. Chromium and cobalt levels returned elevated (117nmol/L and 169nmol/L), and MRI demonstrated the resolving psoas collection but low signal foci around the left hip which could represent metallic debris (figure 4).

Fig 4. coronal MRI demonstrating ongoing collections around the left hip with low signal foci which may represent metallic debris (red arrow)

The patient underwent exploration and drainage of the thigh collection which demonstrated an infective tract coming from the hip joint. After a 3 week course of intravenous antibiotics, the patient improved clinically. The patient was discharged home, but has been referred for revision arthroplasty surgery of the hip at which time histological analysis will take place to aid a formal diagnosis of metallosis.

DISCUSSION

There are several factors that were contributing to this patients hip pain; psoas abscess formation, infected total hip prosthesis, and suspected metallosis.

The true incidence of psoas abscess is unknown, but thought to be under reported (3,4). The relationship between psoas abscess and joint infection is understood, but it is difficult to ascertain which is the initiating event. Reports of psoas abscess associated with prosthetic hip infections has only rarely been reported in the literature, but a study concluded that 12% of patients with hip prostheses infections were complicated by psoas abscess formation (5). They hypothesised that the association was due to either direct spread through the bursa, or acetabular fissures arising at the time of surgery which allowed the initial infection to extend. Two case reports have also been published in the literature reporting the spread of infection to be the reverse direction of this, from an initial psoas abscess to a hip prosthesis (6,7).

The findings of elevated cobalt and chromium on a background of MRI suggestive metallic debris, further complicates the clinical picture. Metallosis secondary to metal on metal arthroplasties have been reported in the literature. A study described 4 patients presenting with groin pain after metal-on-metal hip resurfacing, with histological analysis in keeping with an immunologic response due to metal sensitivity (8). Another study postulated that there was an increased risk of infection in patients with metallosis since the combination of metal debris, ALVAL and tissue necrosis provided an optimum environment for peri-prosthetic bacterial growth and rapid spread of infection (9). A case report described a patient with an infected hemiarthroplasty which was associated with a retroperitoneal abscess in the presence of metallosis which was diagnosed by low signal areas on MRI scanning (10).

In summary, this complex case reports the occurrence of a prosthetic hip joint infection in association with a psoas abscess formation, on a background of suspected metallosis.

REFERENCES

1. Cohen D. Revision rates for metal on metal hip joints are double that of other materials. BMJ 2011; 343:5977
2. Campbell P, Ebramzadeh E, Nelson S, Takamura J, De Smet K. Histological Features of Pseudotumor-like Tissues From Metal-on-Metal Hips. Clin Orthop Relat Res. 2010; 468; (9): 2321–2327
3. Zhou Z, Song Y, Cai Q, Zeng J. Primary psoas abscess extending to thigh adductors: case report. BMC Musculoskeletal Disorders 2010; 6 (11): 176
4. Garner JP, Meiring PD, Ravi K, Gupta R. Psoas abscess – not as rare as we think? Colorectal Dis. 2007; 9(3):269-74
5. Dauchy F, Dupon M, Dutroc H, Barbeyrac B, Lawson-Ayayi S. Association between psoas abscess and prosthetic hip infection: a case-control study. Acta Orthop 2009; 80(2):198–200
6. Auerbach F, Kolbow B, Walz M. Infection of the hip joint following psoas abscess. Case report and literature review. Unfallchirurg. 2005; 108(8):672-8
7. Dala-Ali B, Lloyd M, Janipireddy S, Atkinson H. Journal of Orthopaedic Surgery and Research 2010; 16(5):70
8. Campbell P, Shimmin A, Walter L, Solomon M. Metal Sensitivity as a Cause of Groin Pain in Metal-on-Metal Hip Resurfacing. The Journal of Arthroplasty 2008; 23(7):1080-1085
9. Donaldson JR, Miles J, Sri-Ram K, Poullis C, Muirhead-Allwood S. The relationship between the presence of metallosis and massive infection in metal-on-metal hip replacements. Hip Int. 2010; 20(2):242-7
10. Fujishiro T, Hayashi S, Kanzaki N, Oka S, Kurosaka M. Retroperitoneal abscess following infected bipolar hemiarthroplasty diagnosed by metallosis: a case report. Hip Int. 2010; 20(3):338-9

Gastrointestinal metastasis of the breast cancer is rare and its management varies significantly from that of a primary bowel cancer. We report a case of invasive ductal cancer metastasizing to the rectum and masquerading as a rectal primary.

A 60 year old caucasian woman presented with fresh rectal bleeding nine years after treatment of her primary breast cancer. The investigations revealed features suggestive of primary rectal malignancy and was managed accordingly. However, the surgical histopathology revealed poorly differentiated metastatic adenocarcinoma and the immunohistochemical evaluation confirmed origin from a breast primary. She had an uneventful post-operative recovery and remains disease free thus far.

The continuing advancement in the management of breast cancer patients with resulting increase in the overall survival will lead to such unusual metastatic presentations. Hence, the awareness, identification and differentiation of such rare metastatic presentation are important in order to manage the patients appropriately in the future.

INTRODUCTION

Over the past few decades, the advancement in breast screening and management has increased the overall survival in women with breast cancer. But this in turn, exposes them to the risk of developing new primary malignancies or metastases. The metastatic involvement of gastrointestinal (GI) tract is rare and that of the rectum even rarer. Even though  lobular carcinoma constitutes only 10% of breast cancers, it is the commonest breast cancer metastasizing to the colon and rectum (1). These metastatic lesions can lead to diagnostic challenge for clinicians as they can mimic primary colo-rectal cancer due to the lack of diagnostic signs. We report a rather unusual case of metastatic invasive ductal carcinoma masquerading as primary rectal cancer.

CASE REPORT

A 60 year old woman presented with a suspicious lump in her left breast in early 1996 and underwent open diagnostic surgical excision. She was found to have a moderately differentiated invasive ductal carcinoma measuring up to 20 mm in its greatest diameter extending less than 1 mm from the diagnostic excision margin. Subsequently, she underwent left mastectomy and axillary node clearance which failed to show any residual invasive cancer. However, there was residual high grade cribriform DCIS present adjacent to the diagnostic surgical excision cavity in the mastectomy specimen.  There was no evidence of vascular invasion and none of the seven lymph nodes excised contained any metastatic carcinoma [G2T1N0Mx]. Since her cancer was oestrogen receptor (ER) positive, she was started on Tamoxifen post-operatively.

She presented 2 years later with localised pain in her right knee and bone scan revealed an isolated bony metastasis in the right proximal tibia. This was managed using palliative radiotherapy to control the symptoms and her adjuvant hormonal therapy was switched to Anastrazole (AstraZeneca plc, London, United Kingdom). She neither developed any loco-regional recurrence nor had any progression of her metastatic disease for the next 9 years. During this period, she underwent annual mammographic and clinical follow-up with her adjuvant hormonal treatment being stopped in 2005 after clinical consultation. In early 2007, she presented with ongoing history of fresh rectal bleeding, alternating bowel habit and mucus discharge per rectum. Her examination failed to demonstrate any anaemia, jaundice or generalised lymphadenopathy. Abdomen was soft, non-tender without any organomegaly and digital rectal examination revealed a palpable tumour at 6 o’clock position. Examination of her left mastectomy scar, right breast and axillae were unremarkable.

Fig.1: Fungating rectal tumour on colonoscopy

She underwent colonoscopy, which revealed a fungating tumour at 10 cm from the anal verge in the rectum and rest of the colon was normal (Figure 1). The histopathological examination of the biopsy revealed a poorly differentiated invasive adenocarcinoma (Figure 2).

Fig. 2: Haematoxylin and Eosin staining of the biopsy specimen. Tumour overlying the large bowel mucosa with the line demarcating the tumour edge with arrow pointing at the body of the tumour (magnification×4).

Considering her previous history of invasive breast cancer, pathologist performed immunohistochemistry of the colonic biopsies. The tumour cells were strongly positive for Cytokeratin (CK) 7 with focal positivity for ER but were negative for both Carcino Embryonic Antigen (CEA) and CK 20. The above immunohistochemistry profile with previous history of breast cancer favoured a metastatic breast origin over a primary colonic cancer even though the clinical and colonoscopic findings were not typical. The staging CT scan didn’t show any evidence of metastasis and the MRI scan revealed a locally advanced tumour with involvement of mesorectal facial margins with possible involvement of the peritoneum locally (Figure 3). The bone scan failed to reveal any evidence of active metastatic disease.

Fig.3: MRI scan. Arrow pointing to the localized tumour in the rectum with no evidence of widespread metastasis

After discussing in the multi-disciplinary team meeting (MDT), it was decided to treat the tumour with a short course of pre-operative radiotherapy. After radiotherapy, she underwent a low anterior resection with end colostomy formation. The histopathological examination of the resected tumour showed poorly differentiated metastatic adenocarcinoma with predominantly solid, but with focal glandular growth pattern and large areas of necrosis. The tumour involved full thickness of the bowel but the serosal surface and the resection margins were free of cancer. There was no evidence of metastatic cancer in any of the twenty two lymph nodes excised.

Immunohistochemistry showed tumour cells to be negative for CEA and CK 20, but was strongly positive for CK7 (Figure 4 & 5). The tumour also showed focal positivity for ER and was negative for both progesterone receptor (PR) and Human Epidermal Growth Factor Receptor 2 (Her2). After comparing the morphological and immunohistochemistry charachteristics of the primary breast cancer and rectal tumour, the pathologist concluded that the features were consistent with a primary breast cancer metastasising to the rectum. She underwent an uneventful post-operative recovery and remains disease free during her follow-up until now.

Fig. 4: Immunohistochemistry staining of the resected tumour. CK 20 immunohistochemistry positive normal large bowel mucosa with the adenocarcinoma being negative (magnification × 4).

Fig. 5: Immunohistochemistry staining of the resected tumour. Arrows identifying positive CK7 staining within tumour (magnification × 4).

DISCUSSION

In the largest clinical series till date, only <1% (17 out of 2604 patients followed over 18 years) of the patients with breast cancer were found to have Gastro-intestinal [GI] metastasis (2). Information gathered from autopsy series shows small intestine being the commonest site of metastasis (28%) followed by Oesophagus (25%), Stomach (25%), Colon (19%) and Rectum (4%). The commonest route of metastasis to the GI tract is through the  haematogenous route followed by lymphatic and peritoneal spread. Even though there are a few case reports of lobular carcinoma metastasizing to the rectum, there are only 3 case reports in the literature of invasive ductal carcinoma metastasizing to the rectum till date (3-5).

As a clinician the main challenge lies in the differentiation of such metastatic lesions from primary colo-rectal cancer as the management varies significantly. The clinical presentation of metastatic lesion can be non-specific and mimic a primary cancer as in the present case. The radiological investigations like, CT scan and double contrast barium enema are helpful in localizing the lesion but cannot differentiate between them.

On endoscopy, the colo-rectal metastasis appear as diffuse thickening of the colonic wall mimicking linitis plastica or like in crohn’s disease with ulcerated or nodular areas rather than as a solitary, discrete mass seen in primary colonic lesion (1). But the differentiation was made more challenging in our case, as colonoscopy revealed a solitary fungating mass arising from the rectal mucosa mimicking a primary rectal cancer. Histopathological examination by itself may not be conclusive as the tumour invasion is primarily in the sub-serosa, so unless deep endoscopic biopsies are available the diagnosis will be made only after resection of the tumour. But immunohistochemical markers such as ER, PR, gross cystic disease fluid protein (GCDFP-15), and differential expression of CK7 and CK20 can facilitate an accurate diagnosis as seen in the present report.

Systemic treatment with chemotherapy and/or hormonal therapy is usually employed in patients with confirmed diagnosis of gastrointestinal metastasis (1). The role of surgery is limited to palliation or in patients presenting acutely with obstruction or perforation of the hollow viscus. The lack of concordance between the clinical, radiological and colonoscopic findings with that of the histological findings resulted in our patient being managed with pre-operative radiotherapy followed with low anterior resection. The prognosis of GI metastasis from primary breast cancer is poor with few patients surviving beyond two years, although survival up to nine years has been reported. Our patient remains well and disease free four years after treatment for her rectal metastasis.

Given the increased survival of breast cancer patients due to the early diagnosis through breast screening and better management with current therapeutic regimens, more unusual presentations of metastatic disease, including involvement of the gastrointestinal tract should be anticipated. So the recognition of this rare entity (rectal metastasis from primary breast cancer) is important, as presentation resembles that of primary rectal carcinoma and differentiation is vital as different therapeutic modalities may be appropriate.

REFERENCES

1. Schwarz RE, Klimstra DS, Turnbull AD. Metastatic breast cancer masquerading as gastrointestinal primary. Am J Gastroenterol. 1998;93(1):111-4
2. Borst MJ, Ingold JA. Metastatic patterns of invasive lobular versus invasive ductal carcinoma of the breast. Surgery. 1993;114(4):637-42
3. Haubrich WS. Adenocarcinoma of the breast metastatic to the rectum. Gastrointest Endosc. 1985;31(6):403-4
4. McLemore EC, Pockaj BA, Reynolds C, Gray RJ, Hernandez JL, Grant CS, et al. Breast cancer: presentation and intervention in women with gastrointestinal metastasis and carcinomatosis. Ann Surg Oncol. 2005;12(11):886-94
5. Ikeda Y, Morita N, Ikeda T. Metachronous rectal metastasis from invasive ductal carcinoma of the male breast. Endoscopy. 2008;40 Suppl 2:E108-9

The UK National Bowel Cancer Screening Programme invites men and women aged between 60 – 74 years old to be routinely screened every 2 years. A 90% caecal intubation rate or intubation of the terminal ileum is considered to be the best practice means of identifying completeness. This case report describes how terminal ileal intubation carried out during a routine screening colonoscopy led to the identification and treatment of a carcinoid tumour. Despite evidence for improving colonic diagnoses, completion of colonoscopy by passing through the ileocaecal valve is not performed routinely due to the perceived difficulty of the manoeuvre. With practice, ileoscopy has been shown to be achievable in at least 85% of routine colonoscopies and contributes significantly to quality assurance and to the diagnostic yield.

INTRODUCTION

The UK National Bowel Cancer Screening Program invites men and women aged between 60 – 74 years old to be routinely screened every 2 years (1). This is based on the finding that population screening using biennial faecal occult blood testing has demonstrated a 16% reduction in mortality from colorectal cancer (2). A UK pilot study has successfully demonstrated this as a feasible and reliable method of screening (3). Screening endoscopists are required to pass an endoscopy ‘driving test’ before being allowed to screen and have their screening data rigorously evaluated. A 90% caecal intubation rate, verified by visualisation of the ileocaecal valve and appendix orifice, or intubation of the terminal ileum is considered to be the gold standard means of identifying completeness. Of these, terminal ileal intubation with ileoscopy has the additional benefit of determining whether the source of bleeding is the distal ileum. We present a case where terminal ileal intubation carried out during a screening colonoscopy led to the identification and treatment of a pathology that if missed, could have a significantly worsened prognosis.

CASE REPORT

A 74 year old man was referred to our endoscopy unit after a positive faecal occult blood test. He had no preceeding symptoms or history that would place him at increased risk of colorectal cancer. At colonoscopy, excellent views were obtained to the ileocaecal valve (ICV) and appendix orifice, confirming identification of the caecum. With no colonic pathology seen, the endoscopist proceeded to intubate the ICV, as part of his routine practice. A 3cm ileal polyp was identified 5cm proximal to the valve (Figure 1) and biopsies were taken.

Fig. 1: Endoscopic view of the carcinoid tumour in the distal ileum

Histology demonstrated features of a classical neuroendocrine carcinoid tumour infiltrating the muscularis mucosa.

Fig. 2: Abdominal CT scan, black arrow highlighting the carcinoid tumour in the distal ileum

CT scanning demonstrated the lesion intususscepting into the terminal ileum. There was no evidence of lymphadenopathy and no metastatic deposits were visualised. (Figure 2)

Fig. 3: Resected specimen, black arrow demonstrating the carcinoid tumour in the opened distal ileum

The patient underwent a laparoscopic right hemicolectomy one month after diagnosis. The resected specimen demonstrated a carcinoid tumour measuring 32mm in diameter. Histological examination showed the tumour had breached the serosa into the pericolic fat. Twelve out of fifteen lymph nodes resected were involved with further evidence of extranodal spread. It was staged as PT3 N1 M0.

DISCUSSION

Carcinoids are neuroendocrine tumours derived from enterochromaffin cells most commonly found in the gastrointestinal tract (65% of cases) with an annual incidence of approximately two per 100,000 cases. Around 22% of cases present with distant metastases and in half these cases no primary tumour can be found (4).

Despite evidence for improving colonic diagnoses, completion of colonoscopy by passing through the ICV is not performed routinely. This is due to the perceived difficulty of intubating the valve as well as the anticipated increase in procedure time. With practice, ileoscopy has been shown to be achievable in at least 85% of routine colonoscopies. In skilled hands, it adds on average just additional 3 minutes to the procedure and contributes significantly to quality assurance and diagnostic yield (5).

REFERENCES

1. Quality Assurance Guidelines for Colonoscopy. ‘NHS Bowel Cancer Screening Programme’. 2006; Publication Number 6, pg 7
2. Hewitson P, Glasziou P, Watson E et al. ‘Cochrane systematic review of colorectal cancer screening using the fecal occult blood test (hemoccult): an update’, American Journal of Gastroenterology 2008; 103:1541–1549
3. UK Colorectal Cancer Screening Pilot Group (2004), ‘Results of the first round of a demonstration pilot of screening for colorectal cancer in the United Kingdom’, BMJ, 329:133–135
4. Zuetenhorst JM, Taal BG. ‘Metastatic Carcinoid Tumours: A Clinical Review’, The Oncologist 2005; 10(2):123-131
5. Cherian S, Singh P. ‘Is routine ileoscopy useful? An observational study of procedure times, diagnostic yield, and learning curve’, American Journal of Gastroenterology 2004; 99(12):2324-9

Chest wall chondrosarcomas are a rare cause for an anterior mediastinal mass. Commonly patients present with a painless palpable mass with a third of patients describing diffuse chest pain. We present a patient with an anterior mediastinal chest wall chondrosarcoma presenting with neurological symptoms and flushing. As more of these cases are seen and reported this rare entity is more likely to be considered in the differential diagnosis under appropriate circumstances.

INTRODUCTION

Chest wall chondrosarcomas are a rare cause for an anterior mediastinal mass. We present the interesting case of a young male patient who presented with neurological symptoms from the mass. Although chondrosarcoma is considered the most common primary malignancy of the chest wall and sternum, it is a rare lesion with an incidence of 0.5 chest wall chondrosarcomas per million people per year. Approximately 75% of chest wall chondrosarcomas arise from the costochondral junction of the ribs and sternum.

CASE REPORT

A 26 year old male presented with sudden onset of severe shortness of breath and headache, more intense on the right side while swimming. This was associated with tunnel vision in his left eye, facial flushing and numbness on the left side of his face and down his left arm. On clinical examination he was found to have a murmur and investigation with a chest X-ray (CXR) (Fig 1A) revealed a large mediastinal mass which was causing significant displacement of the trachea to the right. A subsequent computed tomography (CT) (Fig 1B) scan demonstrated a large superior mediastinal mass, which was extending down into the anterior mediastinum and compressing the right ventricular outflow tract (RVOT) of the heart. A Doppler study with transthoracic echocardiogram showed the RVOT to have a systolic gradient of 60 mmHg and was the most probable source of the murmur. After discussion at the multidisciplinary meeting it was decided to proceed with surgery.

Fig. 1: Patient CXR (Figure 1A) with a large shadow (black arrow) of the mass in the superior mediastinum. Due to its size the trachea was displaced far to the right (black arrow head). Sagital views of the CT scan (Figure 1B) showed the mass (black star) in the superior mediastium extending inferiorly into the anterior mediastinum causing obstruction of the right ventricular outflow (black arrow head). The aortic arch was also pushed posteriorly.

In theatre before the skin incision was made the patient desaturated probably due to the complete obstruction of the RVOT by the mass.  An urgent sternotomy was performed with immediate relief of the RVOT compression, achieving complete stability in the patient’s clinical status. Initial inspection of the mass showed that it was stuck to the posterior of the sternum and the left sternoclavicular joint. Vessels stalks originating from the subclavian artery and directly from the aorta were supplying the mass. These were ligated and the entire mass was removed. There were no evidence of cervical or mediastinal lymphadenopathy. Following the successful debulking of the mass the patient made good postoperative recovery with no neurological deficit.

Histological examination (Fig 2) showed the mass to be a chondrosarcoma (grade 1). It appeared to be arising from the posterior aspect of the sternum and the left sterno-clavicular joint. However the tumour was seen to be extending to the excision margins. Hence despite the tumour having been successfully debulked the positive excision margins made it necessary to keep the patient under careful vigilance with initial six monthly imaging. At the 3 monthly follow up the patient was doing well and was devoid of any symptoms he had described before his operation.

Fig. 2: A 170x120x70 mm mass weighing 675 grams was removed. (Figure 2A) The mass was lobulated (Figure 2B) with the lobules separated by thick collagenous bundles (black arrow head). The tumour cells comprised of mature chondrocytes (Figure 2C) which showed minimal atypia. No necrosis or mitoses was seen. However metaplastic bone formation (Figure 2D) was observed (black star). These findings were consistent with those of a well differentiated chondrosarcoma.

DISCUSSION

Although chondrosarcomas are considered the most common primary malignancy of the chest wall and sternum, it is nevertheless a rare lesion as reported in the literature. They are more common in men within there fifth and sixth decade of life (1,2,3)

Patients with chest chondrosarcoma can present with a variety of symptoms depending on tumour size and location. 75% of chest wall chondrosarcomas arise from the costochondral junction between the ribs and sternum as was observed in our case. Sternal chondrosarcoma can present as a slowly growing mass, as in the presented case, producing dyspnoea. Lesions can invade within the medullary bone resulting in the destruction of the anterior and posterior cortical margins. These features were not observed in our case with only a mild abnormality observed along its posterior cortical surface of the sternum.  A review of 106 patients with chest wall chondrosarcoma by Windhe et al (3) found that the most common presentation was with a painless palpable mass. Thirty two per cent of patients reported diffuse chest pain while in thirteen per cent of patients the chondrosarcoma was an incidental finding. None of the patients reported periods of fever, pain at night, weight loss or general illness. The case presented here first came to medical attention due to neurological symptoms. Though it is not possible to explain all the patient’s symptoms with the presence of the sternal chondrosarcoma the patient remains symptom free at his 3 month follow-up, suggesting the mass was the direct or indirect cause of his symptoms.

Given the patient’s young age the other possible differential diagnosis considered by the authors was that of a germ cell tumour which produce 10% to 15% of anterior mediastinal tumours in young adults. Sixty to seventy percent of these tumours comprise of mature teratomas.  Other differential diagnosis of an anterior mediastinal mass, which one could consider include a thymoma, thymic carcinoma, lymphoma especially the nodular sclerosing subtype of Hodgkin’s disease which has a predilection for the anterior mediastinum and Castleman disease which is an uncommon entity in which giant lymph node hyperplasia involves the thorax in 70% of patients.

Our case highlights the importance of considering a chondrosarcoma in the differential diagnosis of an anterior mediastinal mass despite a nonconforming clinical presentation.

The keys to successful treatment are early recognition and radical excision with adequate margins, as chondrosarcoma is relatively resistant to radiotherapy and conventional cytotoxic chemotherapy (4). Complete resection of the tumour has shown 100% survival after 5 years compared to 75 % with incomplete resection. Wide surgical resection was attempted as local recurrence is common with incomplete excision usually after 3-years (3,5). The patient has now been referred to a specialist sarcoma centre for further surveillance and treatment.

Our case highlights the unusual presentation of a rare tumour. The case emphasises the importance of considering it in the differential diagnosis of chest wall tumours. The greater awareness of this possibility will hopefully shorten the time to diagnosis and allow for more planned treatment.

REFERENCES

1. Aoki J, Moser RP, Jr., Kransdorf MJ. Chondrosarcoma of the sternum: CT features. J Comput Assist Tomogr 1989 Sep;13(5):806-10
2. Ashford RU, Stanton J, Khan F, et al. Surgical treatment of chondrosarcoma of the sternum. Sarcoma 2001;5(4):209-13
3. Widhe B, Bauer HC. Surgical treatment is decisive for outcome in chondrosarcoma of the chest wall: a population-based Scandinavian Sarcoma Group study of 106 patients. J Thorac Cardiovasc Surg 2009 Mar;137(3):610-4
4. Philip A. Rascoe, Scott I. Reznik, W. Roy Smythe. Chondrosarcoma of the thorax. Sarcoma. 2011
5. Fong YC, Pairolero PC, Sim FH, et al. Chondrosarcoma of the chest wall: a retrospective clinical analysis. Clin Orthop Relat Res 2004 Oct;(427):184-9

We report successful treatment of colocutanteous fistulae at the site of a benign colonic anastomotic stricture using Self-Expandable Metal Stents (SEMS) and Percutaneous Ethanol Injection (PEI). The stricture and the secondary fistulae developed after Hartmann’s procedure for sigmoid diverticular abscess followed by reversal of the Hartmann’s procedure with a de-functioning loop ileostomy. Although there have been previously reported cases of SEMS for closure of malignant colonic fistula, there are limited reports of SEMS for closure of benign colonic fistulae and none of SEMS combined with PEI for benign colonic fistula. We conclude that this patient’s case exemplifies the potential applications of SEMS combined with PEI as a feasible option of therapy for benign colorectal disease.

INTRODUCTION

Self-Expandable Metal Stents (SEMS) are uncommonly used for benign colonic disease because of their lack of removability and their high rate of migration. A retrievable stent may offer effective therapy for benign colorectal strictures (1).

CASE REPORT

A 54-year-old lady underwent emergency Hartmann’s procedure for diverticular sigmoid abscess. Histology had confirmed the presence of diverticular disease in the sigmoid colon along with inflammatory changes in the tubes, ovaries and part of the uterus, which was resected en-bloc. The patient subsequently underwent reversal of her Hartmann’s procedure with a de-functioning loop ileostomy.

At a clinic review two months post Hartmann’s reversal, the patient complained of intermittent discharge from the lower end of her laparotomy wound associated with per-rectal intermittent discharge of mucous and blood. Contrast Studies had revealed the presence of an anastomotic stricture complicated with a colo-cutaneous fistula (Figure 1).

Fig. 1: Contrast study showing stricture and fistula tract

After a discussion with the patient, taking in account her obesity, the agreed plan was to adopt a non-operative approach rather than surgery that may result in a permanent end-colostomy.  Three months post Hartmann’s reversal a balloon dilatation was carried out successfully via flexible sigmoidoscopy as a joint procedure with a specialist radiologist.

Four months post Hartmann’s reversal it was decided to repeat the dilatation then to insert a colonic stent along with Percutaneous Ethanol Injection (PEI) of the fistula. Covered SEMS was deployed successfully across the anastomotic stricture. This was followed by injection of 20mls of absolute Ethanol through the abdominal fistulous tract whilst, at the same time, normal saline was injected into the stent via a Foley’s Catheter to dilute the alcohol within the lumen of the colon. No complications were encountered.

A month later, contrast studies showed complete resolution of the anastomotic stricture and no evidence of colo-cutaneous fistula (Figure 2). The SEMS was hence removed. At a clinic review one year post Hartmann’s reversal, the patient remained asymptomatic and the fistula had resolved both clinically and radiologically.

Fig. 2: Follow-up contrast study

A year post Hartmann’s reversal, patient was admitted for reversal of her loop ileostomy, which was performed without complications. Follow up at 6 months later confirmed the absence of symptoms.

DISCUSSION

The use of self-expanding metallic stents for benign colorectal diseases has been regarded previously as controversial (1,2). This partly relates to a lack of published data. Only 3% of stents placed in 567 patients considered in a systematic review in (2002) were for benign disease, predominantly diverticular disease (3). There has been only a few reports of its use for closure of benign fistulae in the colon (4).

In our case the presence of a stricture with fistulous tracts was an indication for the use of a covered stent for immediate fistula closure. Covered SEMS have a high rate of migration as compared to uncovered SEMS (2,5). In addition, spontaneous migration of SEMS for benign strictures occurs frequently, usually within the first month after insertion (6).

Ethanol injection for successful ablation of digestive tract fistula has been mentioned in few case reports in the literature (7).

To our knowledge, this is the first case demonstrating SEMS combined with PEI for benign postoperative fistulae. Although longer follow up and future prospective studies are needed, this approach may offer an alternative therapy for patients unfit for surgery.

REFERENCES

1. Seo TS, Song HY, Sung KB, Ko GY, Yu CS. A benign colorectal stricture: Treatment with a retrievable expandable nitinol stent. Cardiovasc. Intervent. Radiol. 2003; 26:181–3
2. Meisner S, Hensler M, Knop FK, West F, Wille-Jorgensen P. Self-expanding metal stents for colonic obstruction: experiences from 104 procedures in a single center. Dis Colon Rectum 2004; 47:444–50
3. Forshaw MJ, Sankararajah D, Stewart M, Parker MC. Self-expanding metallic stents in the treatment of benign colorectal disease: indications and outcomes. Colorectal Disease. 2005; 8:102–111
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