Neonatal Intestinal Obstruction Due to Kaposiform Hemangioendothelioma of the Jejunum.

Kaposiform hemangioendothelioma is a borderline vascular tumor usually seen in infants and children as cutaneous lesions classically associated with the Kasabach-Merritt phenomenon. Intestinal involvement is uncommon and can cause acute presentations, such as obstruction or gastrointestinal bleeding. A 5-day-old neonate presented with bilious vomiting for 2 days. The tumor was in the jejunum. Histopathological examination with immunohistochemistry of the resected jejunum showed CD34 positive endothelial lined vascular spaces infiltrating from submucosa to serosa, which is classical of kaposiform hemangioendothelioma. There was no deranged coagulation profile. This case forms an interesting cause for neonatal intestinal obstruction.

Subacute Arsenic Neuropathy: Clinical and Electrophysiological Observations.

We report a patient who developed subacute peripheral neuropathy following ingestion of a traditional medicine for obesity. A 9-year-old girl who had a residual equinus varus deformity and sphincter disturbance due to pelvic ganglioneuroma presented with subacute sensorimotor peripheral neuropathy of 2 weeks duration. Her symptoms started 3 weeks after she started taking a locally made traditional medicine for obesity. She had no other systemic features of arsenic toxicity. She had Mee's lines on her nails and high serum arsenic levels and 24-hour urine levels confirmed the diagnosis of arsenic neuropathy. Nerve conduction study on admission demonstrated axonal sensorimotor neuropathy with slowed conduction velocity. She was not given any specific treatment and recovery was slow. At 18 months, she showed complete recovery and electrodiagnostic parameters returned to normal values. Arsenic is a known ingredient of many Indian ethnic remedies and possibility of arsenic neuropathy should be thought of in patients presenting with acute or sub-acute peripheral neuropathy of unknown etiology.

Neonatal Teratoma: Craniofacial Treatment.

Teratomas are rare congenital neoplasms. Head and neck locations of the tumor are uncommon with combined intracranial and extracranial extensions being even more rare. The authors present a case of teratoma involving the temporal, buccal, maxillary, orbital and extending to the intracranial regions, which was successfully managed by surgical resection.

Pathogenesis of bladder exstrophy: A new hypothesis.

UNLABELLED: Classical bladder exstrophy affects 1 in 30 000 live births. Results of surgical treatment from different institutions employing various surgical techniques are not uniform, thus there is a need for a consensus on the best technique for bladder exstrophy repair. Surgical correction in bladder exstrophy would be more effective if the exact pathogenetic mechanism was deduced and the procedure was directed to correct the cause, which is responsible for the defect. The anatomy of exstrophy shows that the infraumbilical abdominal wall, the anterior wall of the bladder, and the urethra are split, with splayed out genitalia and musculature along with pubic diastasis. There is no tissue loss and hence embryological defect is unlikely to be the cause of bladder exstrophy. Thus there is a need to examine pathogenesis of bladder exstrophy. METHODS: A literature search was made of the various hypotheses for cause of bladder exstrophy, and attempts were made to propose a new hypothesis. The present hypothesis is also the basis for a technique of mobilization of pelvic musculature, done in two stages. RESULTS: The functional outcomes of 38 children with bladder exstrophy managed over a period of 10 years were reviewed. At a mean follow-up of 4.5 years (range 2.5-8 years), 82% of patients were functionally continent. CONCLUSIONS: The exact embryopathogenesis of bladder exstrophy is unknown. In this study a new hypothesis is proposed, with the aim of tailoring the surgical procedure to correct this defect. Bladder exstrophy epispadias complex (BEEC) is a deformative disruption occurring after embryogenic phase and pubic diastasis, and is central to exstrophy development. A working hypothesis can be formulated in line with our observation so that future experiments based this new hypothesis can aim to elucidate the exact pathogenesis.

Mobilization of pelvic musculature and its effect on continence in classical bladder exstrophy: a single-center experience of 38 exstrophy repairs.

UNLABELLED: Soft tissue mobilization of pelvic musculature in bladder exstrophy repair and its effect on continence. A single-center experience of 38 exstrophy repairs in children. INTRODUCTION/BACKGROUND: Bladder exstrophy is characterized by infra-umbilical abdominal wall defects, evaginated bladder plate of varying size, epispadias, abnormality of genitalia and bony pelvis. The goal of repair is to provide satisfactory continence, which should preferably be done in specialized centers dedicated to exstrophy management. The concept of functional reconstruction rather than urinary diversion is the gold standard worldwide, which can be accomplished by staged or one-stage procedures. Our technique of mobilization of pelvic musculature is based on the concept that continence in bladder exstrophy can be achieved by repairing the disorganized/splayed tissues involved in normal continence (as first advocated by J.H. Kelly) without osteotomy. OBJECTIVES: A systematic review of outcomes of neonatal bladder closure followed by mobilization of pelvic musculature in bladder exstrophy repair in children. STUDY DESIGN: A retrospective chart review of all exstrophy repairs conducted over a 10-year period (between 2001 and 2011). Repairs were done in two stages: bladder closure in the neonatal period (stage 1); and mobilization of pelvic musculature and epispadias repair (stage 2), preferably done between 4 and 6 months of age. The data on complications and continence were evaluated. RESULTS: Thirty-eight patients had completed all the stages of repair. Mean follow-up was 4.5 years (range 2.5-8 years). The following complications were noted: bladder dehiscence in eight patients after stage 1 repair, penopubic fistula occurred in four patients following stage 2 repair. Vulval scarring and vulval dehiscence (2 patients) were complications seen in girls. Twenty-four of the 38 patients (63.5%) achieved complete continence. Functional continence was attained by 31 of the 38 patients (82%). Older age at bladder closure affected continence, while the number of attempts at closure did not affect outcome. The age at pelvic mobilization was not a significant factor in outcome. DISCUSSION: The pelvic floor musculature and urethral sphincters are essential for voluntary control of micturition. In bladder exstrophy, these components are splayed out and our technique is based on reorganizing these components in the second stage. The voluntary urethral sphincter is a delicate complex of musculature located dorsal to the opened urethral plate and spread over the corpora. These are identified using a muscle stimulator and repaired around the tubularized urethra. Normally the levator ani muscle, by its attachment to the pubic bone, forms a loop, by which it compresses the urethra, providing additional aid in continence. In bladder exstrophy with wide pubic diastasis, this loop configuration is lost and becomes a hammock configuration and in fact becomes a pushing force. By mobilizing the pelvic musculature and repairing it in front of the bladder neck, this loop configuration is re-established and further aids in continence. After a successful second stage, patients may have increased frequency and dribbling initially, which improves with age as bladder capacity increases. Perineal exercises aimed at strengthening the pelvic floor musculature are an integral part of our bladder exstrophy management, which begins once the child can understand the technique. The results of our technique are shown in comparison with other series employing the original Kelly's technique (see Table). None of our patients have undergone additional bladder neck repair or permanent augmentation. CONCLUSIONS: Neonatal bladder closure followed by mobilization and repair of the pelvic musculature, produce satisfactory continence in exstrophic children. Proper identification and repair of the external sphincter and levatorplasty correct the altered anatomy by relocating the bladder neck and posterior urethra deep in the pelvis, simulating normal micturition. Early neonatal bladder closure improves outcome. The results are reproducible if basic principles governing continence are followed and when done in a specialized centers.

Multiple failed closure of bladder in children with vesical exstrophy: Safety and efficacy of temporary ileal patch augmentation in assisting bladder closure.

OBJECTIVE: The surgical approach to small bladder template in exstrophy bladder is difficult. Previously, many of these children underwent ureterosigmoidostomy and in recent times, the trend is to do a delayed primary closure. We have used ileal patch as a temporary cover for these small bladders with a view to encourage bladder growth and early results are encouraging. MATERIALS AND METHODS: In five of the 45 children with bladder exstrophy managed by radical soft-tissue mobilization over 10 years, primary bladder closure was not possible due to repeated failed closures. A detubularized ileum was used to patch the bladder initially and after 4 months the patch was excised and bladder closure with sphincter repair was done in second stage. RESULTS: In five children (three girls and two boys) the mean age at initial bladder closure was 14 months and mean age at ileal patch was 22 months. In four patients, the bladder grew facilitating closure and in one patient it failed. There were no complications with the use of gut in patch. CONCLUSION: A temporary ileal patch seems promising in managing failed bladder closure in exstrophy patients. Long-term studies are needed before such a technique can be used in all patients with failed primary bladder closures.

Posterior urethral valves and Cowper's syringocele: A rare association causing voiding dysfunction.

Urethral syringoceles are cystic dilatations of paired bulbourethral glands of Cowper. They can cause voiding dysfunction in male children and usually occur in isolation. We report a rare association of Cowper's syringocele with posterior urethral valves in a 4-year-old child, which was successfully managed endoscopically.

A rare complication of appendicitis: appendiculorenal sinus with renal cellulitis.

This report describes a rare complication of appendicitis, renal cellulitis, which led to a diagnostic dilemma in a 2-year-old girl who presented with fever of long duration.