Should rituximab be the rescue therapy for refractory mixed cryoglobulinemia associated with hepatitis C?

BACKGROUND: Mixed cryoglobulinemia (CG) is a systemic immune complex-mediated disease that involves small-to-medium vessel vasculitis, provoked by the CG containing immune complexes that precipitate in cold. It is associated with hepatitis C virus (HCV) infection in 80% of patients. Mixed CG-mediated vasculitis can affect vital organs such as kidney, liver and heart. Laboratory parameters show presence of cryoglobulin, and in most cases of mixed CG, rheumatoid factor IgM kappa. The current treatment strategy of HCV-associated CG includes targeting the viral trigger HCV with a combination of antiviral medication, interferon-alpha (IFN-alpha) and ribavirin, or the downstream pathogenic events by means of plasmapheresis, steroids or immunosuppression. With multiorgan involvement, the antiviral therapy may be limited due to severity of renal disease, treatment failure, side effects or contraindications. On the other hand, immunosuppressive therapy may be poorly tolerated or ineffective. Therefore, new treatment options such as rituximab (RIT), a chimeric monoclonal anti-CD20 antibody, have been proposed as a rescue therapy. METHODS: We reviewed the literature to evaluate the current evidence in treating HCV-related refractory mixed CG. RESULTS: The use of RIT in treatment of HCV-related CG was first described by Zaja et al. Since then there have been numerous published case series and case reports. So far there has been no randomized controlled trial. In the literature, there have been 60 patients with CG treated with RIT. The male to female ratio was 14:46. Fifty-three patients were HCV-positive. Forty-six patients had mixed type II CG, 7 had type III CG and for 7 the type was not specified. Twenty-five patients had renal involvement ranging from proteinuria, to nephrotic syndrome, to nephritic syndrome to chronic kidney disease. Eight patients had had a renal transplant and were on immunosuppression. Most patients responded to RIT, with only 17 of 60 patients relapsing, and 8 of 17 of those were rechallenged with RIT with a good response. Total follow-up period varied between 3 and 31 months. CONCLUSION: RIT is a suitable rescue therapy in recalcitrant CG associated with HCV. There is evidence supporting the use of RIT as first-line therapy, as opposed to the proposals of others who would strongly recommend antiviral therapy. However, a prospective randomized controlled trial is required to evaluate the efficacy and safety of RIT compared with current standard therapy, which includes antiviral therapy, immunosuppression and plasmapheresis.     

Rapid onset intratubular calcification following renal transplantation requiring urgent parathyroidectomy

BACKGROUND: Secondary hyperparathyroidism is a common complication of end-stage renal disease often requiring parathyroidectomy. Renal transplant with the restoration of normal renal function often allows resolution of hyperparathyroidism, avoiding the need for parathyroid surgery. However, a proportion of patients with hyperparathyroidism become overtly hypercalcemic after renal transplantation which poses management dilemmas between medical and surgical treatment. CASE: We present the case of a 48-yearold man with end-stage renal failure known to have secondary hyperparathyroidism who received a living related renal transplant. Postoperatively he developed prompt hypercalcemia, polyuria, polydipsia and rapid onset intratubular calcification, leading to acute tubular necrosis diagnosed on renal biopsy on Day 7 post transplantation. He underwent surgical parathyroidectomy with resolution of his hypercalcemia and improved renal transplant function. DISCUSSION: This case emphasizes the need for good management of secondary hyperparathyroidism together with close surveillance of PTH in patients awaiting renal transplantation. With good renal transplant function hyperparathyroidism usually resolves. Posttransplant surgical parathyroidectomy should be reserved for severe progressive end organ damage.     

Modified subpectoral biceps tenodesis

Purpose

The proximal part of the long head of the biceps muscle has become a recognized cause of significant shoulder pain. Tenodesis of the long head of the biceps has been advocated as a treatment for pain resulting from biceps tendonopathy, biceps instability, and biceps tendon tears. All of these pathologies may be encountered during rotator cuff, SLAP or Bankart surgery, or in isolation. Several techniques have been described for this tenodesis, including various arthroscopic and subpectoral methods.

Methods

We present a modified bone bridge technique of Mazzocca et al., for subpectoral biceps tenodesis. In this technique we tenodese the tendon through two bone tunnels back over the muscle itself without implants.

Results

Application of this technique on 30 patients (ages 25–48 years) with short-term follow-up of 12–18 months showed statistically significant improvement (P value < 0.05) of the mean Constant and Oxford shoulder scores (pre-operative mean scores were 39.03 and 21.3, respectively, while postoperative mean scores were 76.43 and 44.8, respectively).

Conclusion

This technique has potential advantages as it allows the possibility of adjusting the tension of the biceps tendon before final suturing, in addition to quicker soft tissue healing.     

Testicular atrophy after attempted pediatric orchidopexy for true undescended testis

Background/Purpose

A normal testis in the scrotum is the most important outcome of the attempted pediatric orchidopexy for a true undescended testis. The reports of post-orchidopexy testicular atrophy in the literature have ranged from non-existent to unclear. Our purpose in this study was to estimate the incidence of and associated risk factors for post-orchidopexy testicular atrophy.

Methods

We performed a retrospective review of data from children who had an attempted orchidopexy for a true undescended testis from 1969 to 2003 inclusive. REB approval 1000011987.

Results

There were 1400 attempted orchidopexies involving common (low) type (n = 1135), ectopic type (n = 174), and high type testes (n = 91). There were a total of 111/1400 (8%) atrophic testes, mostly right-sided. 66/111 (59%) were MADE atrophic, and 45 (41%) were FOUND atrophic. Of the 1135 common type, 56 (5%) were MADE atrophic. In the ectopic and high types, the incidence of post-operative testicular atrophy was 1% and 9%, respectively.The most significant risk factors associated with testes MADE atrophic were high testicle, vas problems, and pre-operative torsion.

Conclusions

In this series, the incidence of post-operative testicular atrophy that was MADE was 5% in the common (low) type and 9% in the high type. These numbers and the above risk factors should be quoted to the caregiver during pre-operative informed consent.     

Transanal pullthrough for Hirschsprung disease: Matched case–control comparison of Soave and Swenson techniques

Background

Both the Swenson and the Soave procedures have been adapted to a transanal approach. The purpose of this study was to compare outcomes following the transanal Swenson and Soave procedures using a matched case control analysis.

Methods

A retrospective chart review was performed to identify all transanal Soave and Swenson pullthroughs done at 2 tertiary care children’s hospitals between 2000 and 2010. Patients were matched for gestational age, mean weight at time of the operation, level of aganglionosis, and presence of co-morbidities. Student’s t-test and chi-squared analysis were performed.

Results

Fifty-four patients (Soave 27, Swenson 27) had adequate data for matching and analysis. Mean follow-up was 4 ± 1.6 years and 3.2 ± 2.7 years for the Soave and Swenson groups, respectively. No significant differences in mean operating time (Soave:191 ± 55, Swenson:167 ± 61 min, p = 0.6), overall hospital stay (6 ± 4vs7.8 ± 5 days, p = 0.7), and number with intra-operative complications (3 vs 4, p = 1.0), post-operative obstructive symptoms (6 vs 9, p = 0.5), enterocolitis episodes (4 vs 4, p = 1.0), or fecal incontinence (0 vs 2, p = 0.4) were noted.

Conclusion

After controlling for potential confounders, there were no significant differences in the short and intermediate term outcome between transanal Soave and transanal Swenson pullthrough procedures.     

Hepatobiliary rhabdomyosarcoma mimicking choledochal cyst: Lessons learned

INTRODUCTION

The differential diagnosis of hepatic cystic lesions is a challenging process especially in case of hepatic rhabdomyosarcoma (HRMS) presenting as hepatic cyst.

PRESENTATION OF CASE

We introduce our experience with a case of HRMS in a 3-year-old female patient who was misdiagnosed to have type IV-A choledochal cyst and definitive correct diagnosis was reached after the pathological and immunohistochemical examination of the surgically resected lesion. This case presentation is followed by important practical messages to hepatobiliary surgeons regarding HRMS.

DISCUSSION

HRMS is a rare pediatric tumor. Jaundice is the most common presentation of HRMS followed by abdominal pain and vomiting. Great effort is needed to differentiate the tumor from choledochal cyst and infectious hepatitis. Through evaluation using available imaging studies together with clinical anticipation is mandatory for establishing the correct diagnosis.

CONCLUSION

Differentiation of HRMs from choledochal cyst mandates through evaluation and clinical anticipation. HRMS should be suspected in any child with obstructive jaundice. Once diagnosis is established, multidisciplinary treatment is the best management strategy and it has proved better surgical outcome and long term survival.     

An ovary as unusual contents of an incarcerated umbilical hernia

We present the unusual case of a woman presenting with an incarcerated umbilical hernia. Intraoperatively, the contents of the hernia were found to be an ovary. We outline the clinical presentation of our patient, investigations and management as well as a discussion on unusual contents of umbilical hernias. To our knowledge, this is the first case of a non-malignant ovary incarcerated in an umbilical hernia.     

Fertility preservation for boys and adolescents facing sterilizing medical therapy

Improvements in childhood cancer survival have allowed boys and their families to increasingly focus on quality of life after therapy, particularly their future ability to father children. Treatments should maintain comprehensive cancer care goals and consider the long-term quality of life of these children. While semen cryopreservation is a well-established method of fertility preservation for post-pubertal children, the use of cryopreserved pre-treatment testicular tissue represents a promising, yet experimental method of fertility preservation for prepubertal males facing sterilizing therapy. Healthcare providers should counsel families about the fertility risks of therapy, discuss or refer patients for standard fertility preservation options, and consider experimental approaches to fertility preservation while being mindful of the ethical questions these treatments raise.