Wunderlich's Syndrome in a Tuberous Sclerosis Patient

Spontaneous non-traumatic renal hemorrhage known as Wunderlich's syndrome is known to occur in renal angiomyolipoma and may be the first manifestation of the disease. Angiomyolipomas in tuberous sclerosis are usually bilateral and multicentric. A 25yr old female, a case of Tuberous sclerosis with bilateral renal angiomyolipomas presented with right loin pain of increasing severity. On evaluation she had acute abdomen with increasing abdominal distension. Investigations revealed large right perinephric hematoma with bilateral renal angiomyolipomas. Exploratory laparotomy and right nephrectomy was done. Spontaneous hemorrhage in renal angiomyolipoma in a case of tuberous sclerosis and management are discussed.     

Dual Infection with Bluetongue Virus Serotypes and First‐Time Isolation of Serotype 5 in India

Bluetongue is endemic in India and has been reported from most Indian states. Of late, the clinical disease is most frequently seen in the states of Andhra Pradesh, Telangana (erstwhile Andhra Pradesh state), Tamil Nadu and Karnataka. Our analysis of diagnostic samples from bluetongue outbreaks during 2010–2011 from the state of Karnataka identified bluetongue virus (BTV) serotype 5 (BTV‐5) for the first time in India. One of the diagnostic samples (CH1) and subsequent virus isolate (IND2010/02) contained both BTV‐2 and BTV‐5. Segment 2 (seg‐2) sequence data (400 bp: nucleotides 2538–2921) for IND2010/02‐BTV5, showed 94.3% nucleotide identity to BTV‐5 from South Africa (Accession no. AJ585126), confirming the virus serotype and also indicating that Seg‐2 was derived from a Western topotype, which is in contrast to serotype 2, that belongs to an Eastern topotype. BTV‐5 has been recently reported from Africa, China, French islands and the Americas. Although the exact source of the Indian BTV‐5 isolate is still to be confirmed, recent identification of additional exotic serotypes in India is of real concern and might add to the severity of the disease seen in these outbreaks.     

Imaging evaluation of suspected pulmonary embolism.

Venous thromboembolism (VTE) is a common disorder that is difficult to diagnose clinically but carries significant morbidity and mortality if untreated. Additionally, although demonstrated to be of benefit in cases of proven deep vein thrombosis (DVT) and pulmonary embolism (PE), anticoagulation therapy is not without risk. Because the clinical exam is known to be unreliable for the detection of both DVT and PE, many imaging modalities have been used in the diagnostic imaging algorithm for the detection of VTE, including chest radiography, ventilation/perfusion (V/Q) scintigraphy, pulmonary angiography, and recently, spiral computed tomography (CT) and magnetic resonance imaging (MRI). Chest radiographic findings in acute PE include focal oligemia, vascular enlargement, atelectasis, pleural effusions, and air space opacities representing pulmonary hemorrhage or infarction. The chest radiograph can occasionally be suggestive of PE but is more often nonspecifically abnormal. The main use of the chest radiograph in the evaluation of suspected PE is to exclude entities that may simulate PE and to assist in the interpretation of V/Q scintigraphy. Lower extremity venous compression ultrasonography (CU) is both sensitive and specific for the diagnosis of femoropopliteal DVT, and the value of negative CU results has been established in outcomes studies. However, the reliability of CU for the detection of isolated calf vein thrombosis is not well established, and the clinical significance of such thrombi is debatable. Additional methods such as color and spectral Doppler analysis are also useful in the diagnostic evaluation of DVT but are best considered as adjuncts to the conventional CU examination rather than as primary diagnostic modalities themselves. Compression ultrasonography and Doppler techniques are useful in the evaluation of suspected upper extremity DVT; spectral Doppler waveform analysis is particularly useful to assess for the patency of veins that cannot be directly visualized and compressed with conventional gray-scale sonography. V/Q scintigraphy has been the initial modality obtained in patients suspected of PE for a number of years. Although many studies have investigated the role of V/Q scintigraphy in the evaluation of VTE, the Prospective Investigation of Pulmonary Embolism Diagnosis (PIOPED) study has provided the most useful information regarding the utility of V/Q scintigraphy in this setting. A high probability scan interpretation is sufficient justification to institute anticoagulation, and a normal perfusion scan effectively excludes the diagnosis of PE. A normal/near normal scan interpretation also carries a sufficiently low prevalence of angiographically proven PE to withhold anticoagulation. Although the prevalence of PE in the setting of low probability scan interpretations is low and several outcomes studies have demonstrated a benign course in untreated patients with low probability scan results, patients with inadequate cardiopulmonary reserve do not necessarily have good outcomes. Such patients deserve more aggressive evaluation. Patients with intermediate probability scan results have a 20% to 40% prevalence of angiographically proven PE and thus require further investigation. The radionuclide investigation of DVT includes such techniques as radionuclide venography and thrombus-avid scintigraphy. Although these methods have not been as thoroughly evaluated as CU, studies thus far have indicated encouraging results, and further investigations are warranted. Pulmonary angiography has been the gold standard for the diagnosis of PE for decades. Studies have indicated that angiography has probably been underutilized by referring physicians for the evaluation of suspected PE, likely because of the perception of significant morbidity and mortality associated with the procedure. (ABSTRACT TRUNCATED)     

Long-term follow-up on use of pericardial graft in the surgical management of Peyronie's disease

We have previously reported on the use of Tutoplast cadaveric pericardium as an alternative material for grafting the tunica albugineal defect after Peyronie's plaque excision with satisfactory results in 11 patients. We now review long-term outcomes in this cohort of men. Eleven patients with significant penile curvature interfering with sexual intercourse were evaluated after at least 12 months of conservative therapy. All patients underwent pre-operative evaluation, including penile duplex Doppler ultrasound studies. Chemically processed and gamma-irradiated pericardium (Biodynamics International, Parsippany, NJ) was used to graft the cavernosal defect after surgical excision of the penile plaque. Three patients simultaneously underwent placement of penile prostheses secondary to documented erection problems identified at duplex Doppler ultrasound evaluation. The long-term postoperative complications and erectile function were evaluated with a mean follow-up of 30 months (range 25-35 months). All patients reported resolution of penile curvature allowing for normal sexual function after a mean follow-up of the first 14 months. Thirty months after placement of cadaveric pericardium, the three prosthetic patients still reported excellent sexual function. For the eight patients who did not undergo placement of a prosthesis, three with small to medium plaque size (<2 x 5 cm) continued to do well. The remaining five patients with a large plaque size (>2 x 5 cm) did well initially, but later reported difficulty maintaining erection due to venous leakage, thus they are currently using either a vacuum constriction device or an Actis ring. Three out of these five venous leakage patients had ventral plaques; two had dorsal plaques, one of significant size (4 x 5 cm). We conclude that for those patients who do not undergo placement of a prosthesis, a better long-term outcome is observed when the plaque is small to medium in size (<2 x 5 cm) and dorsally located. Patients with ventral plaque, extreme curvature, or plaque size >4 x 5 cm were more likely to have venoocclusive dysfunction, necessitating further intervention.     

Fibrin sheath removal from central venous catheters: an internal snare manoeuvre.

BACKGROUND: Dysfunction of haemodialysis catheters is most commonly due to a narrowing of the catheter lumen and/or formation of a fibrin sheath around the catheter tip. Reported methods for restoring patency of the catheter lumen include passage of a J-tipped guide wire, passage of a biopsy brush through the catheter, or infusion of a thrombolytic agent into the catheter. While these methods are often effective, they suffer from several limitations. We present a minimally invasive technique to remove thrombi and debris from within the lumen of a partially thrombosed haemodialysis catheter while simultaneously stripping the fibrous sheath. METHODS: A 0.089 cm nitinol wire is bent to create a loop, which is then inserted via the catheters. Upon exiting the lumen of the catheters, the nitinol wire forces a snare open, which disrupts the fibrin sheath and catches intraluminal thrombi and debris. The technique requires no anaesthesia or recovery time. RESULTS: Initial clinical success in our series was achieved in all patients (7/7) as evidenced by restoration of target flow rates on subsequent haemodialysis. None of the patients experienced any complications as a result of the procedure. The catheter 2-, 4-, and 6-week primary success rates were 100% (8/8), 100% (8/8), and 100% (8/8) respectively with a mean duration of 17.1 weeks (range 8-40 weeks). CONCLUSIONS: The internal snare technique is an effective, inexpensive and minimally invasive approach to restoring patency to failed central venous access catheters.     

An unusual presentation of a recurrent neurofibroma in the hand

Neurofibromas in the hand are uncommon and constitute less than 5% of all hand tumours. Accurate diagnosis and appropriate management are important because malignant transformation has been reported, especially in Von Recklinghausen’s neurofibromatosis. We report a case of a large neurofibroma in the right hand of an 18-year-old girl of Afro-Caribbean origin. She was symptomatic with complaints of pain and weakness of grip. General examination revealed multiple café-au-lait spots on the trunk and one nodular swelling in the forearm. The swelling was excised through a palmar approach and histology proved it to be a neurofibroma. The case is being reported for its rarity and it emphasizes the importance of general examination. Histopathological examination is vital in excluding malignant transformation.     

Diffuse Parenchymal Urine Leak After Kidney Transplantation Following Degloving Injury During Donor Nephrectomy

Laparoscopic donor nephrectomy can result in trauma to the kidney which may affect recipient graft function. In this case, the kidney sustained a complete degloving of the capsule during extraction. The kidney was transplanted and had immediate, good renal function, but postoperative course was complicated by a large urinoma that drained through the wound. Exploration was negative for a defined urine leak, but the surface of the denuded kidney was leaking a significant amount of unconcentrated urine. The patient was successfully treated with tissue glue treatment to the kidney surface and peritoneal window.     

APACHE-II scoring system in perforative peritonitis

BACKGROUND: Prospective assessment of the Acute Physiology and Chronic Health Evaluation-II (APACHE-II) scoring system of stratification of disease severity has been shown to provide objective discrimination between low-risk and high-risk groups of patients with intra-abdominal sepsis. The current study was undertaken to evaluate the performance of APACHE-II score in prediction of mortality risk in patients with peritonitis due to hollow viscus perforation. STUDY DESIGN: Fifty patients admitted to a teaching hospital with peritonitis due to hollow viscus perforation were prospectively studied over a 2-year period. APACHE-II points were assigned to all patients in order to calculate their individual risk of mortality before undergoing emergency surgery. The accuracy in outcome prediction of the APACHE-II system was assessed by means of receiver operating characteristic (ROC) curve and the Pearson correlation coefficient and its significance test. RESULTS: Of the 50 patients admitted during the study period, there were 42 (84%) survivors and 8 (16%) nonsurvivors. Mean APACHE-II score of the study population was 11.38 with a range of 1 to 23. The predicted death rate was 23% and the observed death rate was 16%. Mean APACHE-II score in survivors was 9.88, whereas in nonsurvivors it was 19.25. Using ROC analysis, the area under the curve was found to be .984. Correlation of APACHE-II score and predicted death rate showed perfect correlation, with r = .99 and P <.001 [R2 = .9993]. APACHE-II score between 11 and 15 showed a sensitivity and specificity of 100% and 73.8%, respectively, and APACHE-II score of 16 to 20 had a sensitivity and specificity of 87.5% and 100%, respectively. CONCLUSION: APACHE-II score between 11 and 20 was shown to be a better predictor of risk of mortality in patients with peritonitis due to hollow viscus perforation. Predicted mortality did not correlate with observed mortality in patients with APACHE-II scores of 1 to 10 and greater than 20. The APACHE-II scoring system can be used to assess group outcomes in patients with peritonitis due to hollow viscus perforation. However, it does not provide sufficient confidence for outcome prediction in individual patients.     

Pancreas Graft Salvage Using Pancreatico-Duodenectomy With Enteric Drainage

As demand for donor pancreases increases, attempts are being made to utilize even marginal grafts for transplantation. Injury during pancreas recovery can predispose to posttransplant complications and graft loss. Early recognition and correction can salvage these grafts. The authors report an instance of poor segmental perfusion of the pancreas graft that was salvaged by pancreas head resection and enteric drainage through a Roux-en-Y pancreatico-jejunostomy.     

Laparoscopic incisional hernia repair after liver transplantation.

Incisional hernias occur in up to 17% of patients after liver transplantation. Laparoscopic ventral hernia repair is associated with fewer wound complications and a decreased incidence of recurrence when compared to open hernia repair in nontransplant patients. This is a retrospective review of 13 patients who underwent laparoscopic incisional hernia repair (LAP group) after liver transplantation compared to 14 patients who had open repairs (OP group; all but one with mesh). Primary immunosuppression in both groups at the time of transplantation was tacrolimus, but more patients in the LAP group were on sirolimus at the time of hernia, while more patients in the OP group were on prednisone at the time of hernia repair. All operations were completed with a laparoscopic approach; there were no conversions to open. Length of stay differed significantly between the 2 groups, with a mean of 5.4 days for the LAP group compared to 2.7 days in the OP group (0.0059). Complications occurred in 2 (15%) of the patients in the LAP group and 5 (36%) in the OP group. One patient in the LAP group required mesh removal to exclude causes of recurrent ascites, and 1 in the OP group for mesh infection. One (7.6%) of the patients in the LAP group developed a recurrence, compared to 29% (4) of the OP group (P =0.3259). In conclusion, laparoscopic incisional hernia repair is safe in patients after liver transplantation, with a low risk of infection or recurrence.