Right heterotaxy with Hirschsprung's disease - a new association.

A baby girl with prenatal diagnosis of complex cardiac anomalies and diaphragmatic hernia was born at 36 weeks of gestation. At 4 hr of life, the baby developed respiratory distress and was intubated. She was found to have right hetetrotaxy with total anomalous pulmonary venous drainage into the portal vein, five hepatic veins draining the liver and intrathoracic herniation of the stomach. The child also developed abdominal distension on the second day of life with passage of scanty meconium. The diagnosis of Hirschsprung's disease (HD) was confirmed by histology. HD in association with right heterotaxy has not been reported earlier. The association of heterotaxy with HD in our patient raises a possible genetic link between the two anomalies that needs further research.

Solitary vascular malformation of the clitoris.

Isolated involvement of the clitoris by vascular malformation (VM) is very rare. Clinically, the lesion simulates female pseudohermaphroditism. A five-year-old girl presented with clitoromegaly and a clinical diagnosis of solitary VM of the clitoris was made. Magnetic resonance imaging showed characteristic features and confirmed the diagnosis and the extent of the VM. This is the first reported case of isolated involvement of the clitoris by VM to be diagnosed preoperatively.

A case of supercarbia following pneumoperitoneum in an infant.

A 4-month-old healthy male infant underwent left herniotomy under general anesthesia with caudal block. Carbon dioxide (CO2) pneumoperitoneum was created through the left hernial sac for inspection of the right processus vaginalis. Episodes of desaturation associated with significant reduction in chest compliance were noted intraoperatively. This was overcome by increasing the inspired oxygen concentration (FiO2). The infant failed to regain consciousness and spontaneous respiration at the end of surgery. The chest compliance deteriorated further and clinically a CO2 pneumothorax (capnothorax) was suspected. The endtidal carbon dioxide (P(E)CO2) was initially low in the immediate postoperative period. Subsequent to the readministration of sevoflurane and manual ventilation with a Jackson Rees circuit, a sudden surge in P(E)CO2 with improvement of chest compliance was observed. At that time arterial blood gas (ABG) analysis revealed a PCO2 of 17.5 kPa (134 mmHg) and pH of 6.9. The causes of severe hypercarbia and the physiological changes observed in this infant are discussed.

Spermatic cord metastasis from a medulloblastoma.

A 4-year-old boy presented with metastases in the spermatic cord and ribs 2 years after treatment for a primary medulloblastoma (MB). The testis was free of tumor. A MB presenting with a metastasis to the spermatic cord has not been reported to date. The role of a ventriculo-peritoneal shunt in promoting such spread should be considered in planning management.

Umbilical evagination of the bladder with omphalocele minor.

A neonate with umbilical evagination of the bladder (UEB) and omphalocele minor (OM) is reported. The findings in this case support the origin of the urachus from the upper end of the cloaca rather than the allantois. The frequent occurrence of OM with urachal anomalies, including UEB, suggests an embryological association between the two conditions.

Mediastinal teratoma in a neonate.

A neonate with severe respiratory distress due to a benign mediastinal teratoma (MT) is reported. Despite early and easy surgical excision of the tumor, the child died due to poor cardiac function. Only ten cases of MT in neonates have been reported in the literature so far. While the tumor has been known to interfere with lung development in utero, postnatal myocardial dysfunction due to poor heart development has not been previously documented.

Urolithiasis in Australian aboriginal children.

Thirty-six Australian Aboriginal children with urolithiasis were reviewed. Males dominated the series. The age distribution ranged from 8 months to 12 years and nearly 70% were 2 years or younger. Thirty-five patients had upper tract stones. Ultrasound was diagnostic in 35 patients and was falsely negative in one. Dietary factors, dehydration and recurrent diarrhoea are incriminated in the aetiology, because ammonium urate and oxalate were the main constituents of the stones. Malformations of the urinary tract were rare and known metabolic disorders were not seen. Chemical dissolution of the stones was found to be a safe and effective adjuvant in the management of urate stones.

Urethral injuries in girls with pelvic trauma.

Three girls with major urethral injuries following motor vehicle accidents are presented. Early diagnosis of this rare condition, which is often associated with vaginal injuries, is imperative and a cystourethrogram, examination under anesthesia and a cystovaginoscopy are usually indicated. Primary repair of these injuries is recommended and long-term follow-up is essential.

Type IVa choledochal cysts: surgical management and literature review.

Type IVa choledochal cysts with cylindrical dilatation of the intrahepatic ducts constitute a relatively less recognized variety of choledochal cysts, and differ from cystic dilatation of intrahepatic ducts in their clinical manifestations and response to treatment. Five patients with type IVa choledochal cysts and cylindrical dilatation of major intrahepatic ducts who underwent cyst excision and Roux-en-Y hepaticojejunostomy are reported. The duration of symptoms was less than 1 year in all patients. Palpable abdominal mass and abdominal pain were present in 3 patients. The traid of jaundice, abdominal pain and mass was present in only 1 patient. The intrahepatic dilatation regressed after excision of the extrahepatic cyst just below the hilum of the liver. The surgical technique is described and the need for excision of the cyst is emphasized.

Fournier's gangrene: its aetiology and management.

The clinical features and management of 12 patients with Fournier's gangrene are described. The patients differed from the usual description of Fournier's gangrene in that they were older, the disease had a less abrupt onset and a definite predisposing factor was identified in 10 of the 12 patients. The importance of early diagnosis and excision of necrotic tissue is emphasized.