Combined en bloc liver-double kidney transplantation in an infant with IVC thrombosis.

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.

H-type rectovaginal fistula in a patient with bilateral single ectopic ureters.

Congenital H-type rectovaginal fistulas and single ectopic bilateral ureters are each rare malformations. We describe a baby girl with a congenital rectovaginal fistula diagnosed 2 years after correction of single ectopic bilateral ureters. To our knowledge, this is the first association of these entities. Repair of fistula was complicated by recurrence, requiring a second procedure. The recommended operation for this anomaly requires separating the suture lines on the vagina and rectum. The practice of simply oversewing and then buttressing the suture lines is probably not sufficient.

Penoplasty for buried penis in infants and children: report of 100 cases.

PURPOSE: The aim of this study is to report single surgeon's experience in treatment of buried penis in children and describe the surgical technique which was developed by the senior author. METHODS: Described surgical technique avoids circumferential incision at the base of the penis and thus prevents formation of post-operative lymphedema. Repair is based on a vertical incision in median raphe, complete degloving of penis and tacking its base to prepubic fascia. Shaft skin is attached to base of penis with vertical mattress sutures. RESULTS: Patient age varied from 1 month to 11.4 years (mean 1.9 years). All patients had good to excellent outcome with uniformly improved visualization of penile shaft post-operatively. There was one case of wound infection successfully treated with oral antibiotics. Revisions were needed in 4% patients. CONCLUSION: Surgical correction of buried penis in infants and children is safe and effective. Described technique is applicable for essentially all cases of congenital buried penis as well as for iatrogenically entrapped penis after circumcision. In our experience there were no additional procedures required to assure skin coverage of penile shaft.

Minimally invasive closure of pediatric umbilical hernias.

BACKGROUND: Pediatric umbilical hernias may close spontaneously by concentric fibrosis and scar tissue formation. Some hernias do not close. This study was developed to assess this novel minimally invasive closure (MIC), using injectable material to close the umbilical defect. METHOD: Twenty-five children with umbilical hernias of 1.5 cm or less were included in the study. Deflux (Q Med, Uppsala, Sweden), a biodegradable compound of dextranomer microspheres in hyaluronic acid, was injected percutaneously in the border and preperitoneal space in 4 quadrants of the hernia defect, thereby occluding the lumen. Follow-up visits were obtained at approximately 1 week, 3 months, and 1 year. RESULTS: Two to twenty-four months after surgery, 21 of the 25 umbilical hernias were closed (84%). To date, there have been no complications from the injected compound substance. The average age at the time of the MIC was 6 years and 7 months, ranging from 4 months to 17 years. The average defect was more than 6.4 mm, ranging from 4 to 14 mm. CONCLUSION: Minimally invasive closure procedure with injection of dextranomer hyaluronic acid copolymer can safely be used to close umbilical hernias. The procedure closed or reduced the size of hernias in our patients immediately after surgery; and within months, 21 (84%) of 25 were closed. One defect has not closed in 1 year and will need repair. The remaining 3 defects are small and may go on to close by ongoing fibroblast ingrowth and collagen deposit. The MIC procedure may be an alternative to open repair of umbilical hernias. Increased experience and long-term follow-up will determine the true efficacy of this new technique.

Revision of circumcision in children: Report of 56 cases.

BACKGROUND/PURPOSE: Circumcision is the most commonly performed surgical procedure in the United States today. Despite the large number of specialists who perform this procedure, only occasionally are the results unsatisfactory. The purpose of this study is to review the indications for circumcision revision, attempt to identify the specialists who are performing unsatisfactory circumcisions, describe the authors' surgical technique for circumcision revision, and review current coding and billing issues related to this procedure. METHODS: The authors reviewed the charts of 56 consecutive children who underwent circumcision revision over a 4-year period (1995 to 1999). They also reviewed their current coding and billing practices for this procedure. RESULTS: Children undergoing revision of circumcision ranged in age from 6 weeks to 11 years with a mean of 26.7 months. Redundant foreskin was the most common indication for circumcision revision. In 38 patients (68%) the authors were able to identify the specialist who performed the procedure. Pediatricians were most commonly identified (n = 26), followed by residents in training (n = 10) family physician (1), and nurse midwife (1). The authors were unable to identify the type of neonatal circumcision originally performed. Their surgical procedure was the conventional sleeve technique without variation in 55 cases. A gomco clamp was utilized in one patient under local anesthesia and resulted in significant difficulty in the performance of the revision. All patients had a satisfactory cosmetic outcome. There was one complication in a child who required a return to the operating room for postoperative bleeding and hematoma. In review of the authors coding practices they found that there was an appropriate diagnosis code available for redundant foreskin but a specific procedure code was lacking before 2002. The authors also found that midwives perform circumcisions throughout the United States. CONCLUSIONS: Considering the number of neonatal circumcisions performed in the United States, revision of circumcision uncommonly is required. The most common indication for circumcision revision is redundant foreskin. Although pediatricians were most commonly implicated in this study as the source of unsatisfactory circumcisions, that finding probably is more a reflection of local practices and referral patterns. Our recommended surgical procedure, the conventional sleeve technique, is familiar to pediatric surgeons, produces a satisfactory cosmetic result, and is easy to teach to residents and fellows. The authors do not recommend the use of a gomco clamp for circumcision revision. The authors do not feel that a circumcision revision should be delayed expecting that the child will grow into the redundant foreskin. Appropriate diagnosis codes have been available, but a new and more specific procedure code has just been introduced in 2002.