Scrotal pop off in a congenital anterior urethral diverticulum.

INTRODUCTION AND OBJECTIVE: Congenital anterior urethral diverticulum is a rare entity. This teaching video shows the scrotal pop-off mechanism for an anterior urethral diverticulum and the unique voiding pattern of a boy who empties his bladder by compression of his scrotum. The findings during urethroscopy and open reconstruction are also demonstrated. PATIENT AND METHODS: A four-year-old boy presented to the clinic with a poor urinary stream and scrotal swelling during voiding. Physical examination during voiding revealed a dumbbell-shaped anterior urethral diverticulum with scrotal pop off and preserved renal function. RESULTS: Open excision of the scrotal part of diverticulum was performed. Urethroplasty was conducted using a de-epithelialised diverticular wall flap from the penobulbar urethra. On follow up the boy voided with a good flow and resolution of symptoms. CONCLUSION: Scrotal pop off with completion of voiding by manual compression of the diverticulum may preserve bladder and renal function. The preferred treatment of anterior urethral diverticulum is open excision of the diverticulum and reconstruction. The wall of the diverticulum may be used to reinforce the repair ventrally, where the corpus spongiosum is deficient.

Setting the speed limit: a pilot study of the rate of serum creatinine decrease after endoscopic valve ablation in neonates.

PURPOSE: In neonates with a posterior urethral valve serum creatinine is increased. It decreases after successful relief of obstruction. Clinicians consider urinary diversion if serum creatinine remains increased. However, the optimal rate of decrease of serum creatinine is not defined. We generated useful data on the rate of serum creatinine decrease in neonates with a posterior urethral valve by introducing the idea of a prediction curve. MATERIALS AND METHODS: We reviewed the medical charts of 15 consecutive children treated for a posterior urethral valve in the neonatal period at our institute between 2002 and 2007. The 11 children with a delayed diagnosis of a posterior urethral valve were excluded from analysis. Serial serum creatinine levels in the cohort of 15 patients were analyzed to estimate 1) the rate of decrease after valve ablation and 2) the time needed to achieve a nadir. RESULTS: One child died of renal insufficiency on day 10 of life and was excluded from study. Serum creatinine attained a nadir at about age 6 months but 73% of the total decrease occurred within the first 2 months of life, which was also equal to a 45% decrease from the peak values recorded soon after birth. The rate of decrease did not appear to be influenced by the peak values. Children with normal peak creatinine or intercurrent problems did not follow the trend. CONCLUSIONS: The rate of decrease in serum creatinine in the first few months of life may provide useful information on the adequacy of valve ablation.

Clitoroplasty: past, present and future.

This paper reviews the history of clitoral surgery throughout the twentieth century. The changing operations, from amputation through to attempts to produce a normal clitoris mirror the changing attitudes and available knowledge. It also examines the current issues that arise out of available data on long-term outcomes of clitoroplasty.

Lethal comorbidity with genital anomaly in the infant.

OBJECTIVE: Genital anomaly (GA) carries a risk of life-threatening comorbidity which is not well defined and is under reported. We aimed to determine the associated anomalies in children with GA who died. MATERIALS AND METHODS: We retrospectively reviewed the deaths among all GA patients presenting to our institution over 32 years (1970-2001). Seventy children with exstrophy/epispadias were excluded, leaving 200 patients, of whom 26 had died. Hospital and postmortem records were examined for biographical data, karyotype, morphological anomalies and causes of death. RESULTS: Sixty-eight out of 200 patients had congenital adrenal hyperplasia (CAH), with two deaths, from cardiomyopathy and encephalitis. Of the 132 non-CAH patients, 24 (17%) died. Sixteen deaths were in the non-CAH intersex group of patients with GA. There were eight deaths in the non-intersex genital anomaly group, which included five with a cloacal anomaly (+/- VATER association). Fifteen had major cardiac anomalies and 10 had facial dysmorphism suggesting a syndrome. Two infants died of renal failure with Denys-Drash syndrome. CONCLUSIONS: Infants with a non-CAH intersex GA have a high risk of mortality, particularly from an associated cardiac anomaly. This mortality may decrease in the future with cardiac surgical advances. There is a significant mortality in infants with non-intersex GA (anorectal malformations and cloacal anomalies) also due to complex cardiac and/or renal anomalies. Major chromosomal aberrations and facial dysmorphism were noted in some patients in both study groups. GA associated with testicular dysgenesis and/or imperforate anus requires screening for cardiac defects, chromosomal anomalies and dysmorphology syndromes.

Severe colonic diverticulitis in an adolescent with Williams syndrome.

Williams Syndrome (WS) is a condition with multisystemic involvement caused by a genetic deletion in chromosome 7. Colonic diverticulosis has been described in adults with WS; however, it has not previously been reported in adolescents with WS. We report an adolescent boy with WS who developed complicated colonic diverticulitis and briefly review the possible aetiology of diverticular disease.

Extrarenal Wilm's tumour - a rare entity.

A rare case of extrarenal Wilms' tumour (ERWT) that presented as an asymptomatic subcutaneous lumbar mass is reported. The diagnosis could only be established postoperatively.

Glanular diphallus with urethral stricture.

An unusual case of a 5-year-old boy with duplication of the glans, a rare variety of diphallia, is reported. The two glandes were arranged one above the other, the dorsal glans had only a blind pit while the ventral glans had a patent urethra with glanular hypospadias. The child also had right renal agenesis and a posterior urethral stricture.

Rapunzel syndrome with a long tail.

A rare form of gastric trichobezoar extending into small bowel with varying gastrointestinal symptoms is known as Rapunzel syndrome. A case history of 6 years old boy is described. This is the twelfth patient with Rapunzel syndrome in the literature.

Gastric teratoma: a rare cause of gastrointestinal bleeding.

Gastric teratoma is a rare tumor of infancy, almost exclusively found in males. We report a 6-month-old male child with gastric teratoma who presented with gastrointestinal bleeding; he was treated successfully with local excision of the tumor.

Hydatid cyst of the orbit with papilloedema.

A rare case of Hydatid cyst of the orbit causing, unilateral axial proptosis is reported. The presence of gross unilateral papilloedema misled us to the character of the cystic swelling causing axial proptosis.

Evaluation of sorghum starch as a tablet disintegrant and binder.

The starch prepared from the seeds of Sorghum bicolor, Moench has been evaluated as a disintegrant and binder in tablets of magnesium sulphate, calcium carbonate, sulphadimidine, and chloroquine phosphate to represent soluble and insoluble inorganic and organic substances. The starch performed as well as maize starch in binding and disintegrating properties and better than acacia as binder.

Investigation into raw potato bits as a basic material for the formulation of prolonged action coated beads.

Raw potato bits were prepared and were successfully coated with different coating materials to achieve prolonged action. Likewise three sets of different coated beads were prepared. The in vitro release of the drug was studied in different pH media. The release of the drug was found to be quite uniform and consistent throughout the period of dissolution.