Feminizing genital reconstruction: experience with 52 cases of ambiguous genitalia.

AIM: The last decade witnessed the introduction of different modifications and even new techniques for feminizing genitoplasty. The claim was that the classical Hendren's procedure was associated with unsatisfactory results. At the King Khalid University Hospital, the Hendren's operation was the procedure of choice for feminizing genitoplasty. In this report, we describe our experience with a total of fifty-two cases of ambiguous genitalia. We understand that this comes in the midst of growing criticism of the Hendren's procedure. MATERIALS AND METHODS: The medical records of 52 consecutive patients with ambiguous genitalia who underwent feminizing genital reconstruction were reviewed. Forty-five patients (86.5%) had congenital adrenal hyperplasia (CAH), 3 patients had testicular feminization syndrome, 2 patients had mixed gonadal dysgenesis, one patient had vaginal atresia with Kaufman's syndrome and one patient had isolated congenital clitoromegaly. Age at first presentation ranged between 1 day to 15 years. Feminizing genital reconstruction using Hendren's technique was carried out as a single stage procedure in 29 (55.81%) patients and as staged surgery in 23 (44.2%) patients. A total of 50 clitoral operations and 43 vaginoplasties were performed. Follow-up ranged from 6 months to 12 years (mean 3 and 1/2 years). The surgical results were analyzed and complications documented. RESULTS: Reconstruction was completed in 47 (90.2%) patients. Thirty-six (78.2%) patients had a satisfactory genital appearance with normal looking clitoris. Seven patients required revision clitoroplasty. Thirty-six (83.7%) patients had a good size vagina appropriate for age. Seven patients (16.2%) had vaginal stenosis and two had revision vaginoplasty with good results. Postoperative complications included urethral fistula (one patient), meatal stenosis (one patient), and transient neurogenic bladder (one patient). The final cosmetic results were considered excellent in 40 (85.1%), fair in 4 patients, and poor in 3 patients. CONCLUSION: The Hendren's techniques of flap vaginoplasty for low confluence and vaginal pull-through for high confluence offer satisfactory cosmetic and functional results. Long-term follow-up is necessary to ensure social and psychosexual adjustment.

Circumumbilical pyloromyotomy: larger pyloric tumours need an extended incision.

Circumumbilical pyloromyotomy has been used widely to offer an optimum cosmetic approach. On occasion, there are difficulties in delivering the large pyloric tumour through a relatively small incision. The authors prospectively collected a series of 39 consecutive pyloromyotomies performed over a period of 3 years in three teaching hospitals. Patients were aged 13 to 90 days (average 35) and the male-female ratio was 5:1. The initial operative approach was a right upper quadrant (RUQ) incision (n = 6) and later a circumumbilical incision (CUI), with or without lateral extension, combined with transverse division of the linea alba and part of both rectus muscles (n = 33). Two infants (1 RUQ and 1 CUI) developed wound infections, which were treated with antibiotics. There were no other peri- or postoperative complications. It was necessary to extend the incision in one-third of CUI cases (n = 12) in order to facilitate the delivery of a large pyloric tumour through the incision. Both omega-shaped (n = 3) and a new modified extension (n = 9) have been used with good cosmetic results.

Fine needle aspiration cytology of mesenchymal hamartoma of the liver. A case report.

BACKGROUND: Mesenchymal hamartoma (MH) of the liver constitutes the third or fourth most common tumor of the liver in childhood and occurs most commonly in the first two years of life. MHs of the liver are seldom aspirated, and reports on the role of fine needle aspiration (FNA) in the diagnosis of MH are scarce. Clinically, cytologically and even histologically, MH can be mistaken for a number of reactive and neoplastic hepatic lesions that may occur in children under 2 years of age. CASE: A 10-month-old Pakistani female presented with a history of a right-sided, nonpainful abdominal swelling. Abdominal computed tomography showed a large, partly solid and partly cystic, heterogeneous hepatic mass. FNA cytology showed clusters of both epithelial and mesenchymal/spindle-shaped cells with pieces of loose connective tissue. A cytologic differential diagnosis of mesenchymal hepatic hamartoma and hepatoblastoma of the possible mixed mesenchymal/epithelial subtype was rendered. The histopathologic diagnosis of the resected tumor mass was benign mesenchymal hamartoma of the liver. CONCLUSION: In children under 2 years of age who present with partly solid and partly cystic hepatic masses, the possibility of MH of the liver should be considered. FNA has a role in the diagnosis of MH. The cytopathologist should be aware of the patient's age, radiologic features and cytologic appearances of this rare, benign neoplasm. Histologic examination of tru-cut biopsies and immunohistochemical stains can help to exclude other pediatric neoplasms that may show cytologic features similar to or mimicking those of MH.

Congenital cystic disease of the lung in infants and children (experience with 57 cases).

A retrospective analysis of 57 consecutive cases with congenital cystic disease of the lung admitted to King Faisal Specialist Hospital and Research Center and King Khalid University Hospital, Riyadh, between 1985-1995 is presented. There were 37 congenital lobar emphysema (CLE), 7 cystic adenomatoid malformation (CAM), 8 bronchogenic cyst (BC) and 5 pulmonary sequestrations (PS). There were 39 males and 18 females with ages ranging from 1 day to 5 years. All patients were symptomatic except three. Respiratory distress, repeated chest infections, and cystic changes noted in chest x-ray were the commonest presentation. Five of eight patients with BC presented with symptoms related to pressure effect of the cyst on the surrounding structures, these included bronchiectasis in two patients, bronchopleural fistula in one, pulmonary artery stenosis and bronchomalacia in one, airway obstruction mimicking bronchial asthma in one. Seven patients (12.2%) were treated conservatively, the remaining underwent surgery. Surgery included excision of the bronchogenic cyst and lobectomy for CLE, CAM, and intralobar sequestration. The post-operative course in most cases was uneventful. There were no deaths in this series, and the majority of patients had a satisfactory outcome with follow-up ranging from 1-72 months (mean 24 months). It appears that lobectomy for symptomatic CLE, CAM, and intralobar sequestration and excision for bronchogenic cyst offer the best treatment modality and is well tolerated by pediatric patients. Careful search for associated anomalies is important to obtain better outcome.

Hydatid disease of the liver in children.

Between 1986 and 1997, 21 children (ten boys and 11 girls) had surgery for hydatid disease of the liver. Their mean age was 6.5 years (range 3-12). Abdominal distention with a mass was the commonest presenting symptom (71.4%), followed by abdominal pain (38%). Hepatomegaly with a palpable mass was present in 12 (57%). Three children had concomitant pulmonary and brain hydatid disease. The diagnosis was established clinically and by skin testing, serology and imaging techniques. All patients received a pre-operative course of mebendazole (50 mg/kg/day) for between 1 and 8 weeks. At surgery, 11 children had a single cyst, eight of which were in the right lobe of the liver. Ten children had multiple cysts occupying both liver lobes. Three forms of surgical treatment were used: capitonnage + partial excision of fibrous capsule; total excision of the cyst; and external drainage of the cyst cavity. Three children required re-operation. Mean follow-up time was 24 months. There were no deaths, but five children developed post-operative complications. Surgical treatment in the form of primary closure of the cyst cavity without drainage seems to offer the best therapeutic option for patients with large hydatid cysts.

Congenital H-type anourethral fistula with severe urethral hypoplasia: case report and review of the literature.

Congenital H-type anourethral fistula with severe urethral hypoplasia and normal anus is an extremely rare variant of anorectal malformations among boys. The authors report a case of a 5-year-old boy who underwent successful management of severe urethral hypoplasia with progressive augmentation by dilating urethra anterior gently and achieving a functionally normal urethra with minimal morbidity. H-type anourethral fistula was excised subsequently through anterior perianal approach.

Implantable central venous access devices in children with metabolic disease.

We have inserted 20 totally implantable central venous devices in 17 patients with severe metabolic disease over a 43-month span. Patient ages ranged from 2 months to 17 years (mean, 4.2 years). The underlying pathology was Gaucher's disease in six patients, vitamin D-dependent rickets type II in five, propionic acidemia in two, and methylmalonic acidemia, 3-hydroxyl-3-methylglutaryl coenzyme A (CoA) lyase deficiency, fructose 1,6 diphosphatase deficiency, and urea cycle disorder in one child each. There were seven complications (six due to catheter-related infection and one due to occlusion of the system) during a total of 7,278 patient-catheter days. The infection rate was 0.8 per 1,000 days. Six catheters were removed due to complications and two due to completion of treatment. There were no operative complications or deaths. Our experience demonstrates that a totally implantable device may be useful in children with metabolic disease who need long-term venous access. Attention should be given to minimize the infection rate to reduce the rate of catheter removal.

Leiomyoma of the esophagus and bronchus in a child.

Leiomyomas of the esophagus and/or the bronchus have rarely been reported in children. To our knowledge, the simultaneous presence of this tumor in both the esophagus and a bronchus in a child has not been previously reported. A 7-year old boy presented with respiratory and esophageal symptoms and was found to have a leiomyoma of the esophagus and right main bronchus. The esophageal leiomyoma was treated with limited myotomy, but bronchoscopic resection was possible for the bronchial lesion. The postoperative result was excellent, with normal swallowing and no residual respiratory problems at 1-year follow-up.

Transverse testicular ectopia: report of three cases.

Two patients who presented with a history of right inguinal hernia and a third with an impalpable left testis were found to have transverse testicular ectopia. Both testes were fixed in their own scrotum, through a modified Ombredanne operation in two patients and a subdartos pouch in one.

Superior ectopic thoracic kidney.

A 9-month-old boy with a respiratory tract infection was found to have a mass in the left chest posteriorly. This proved to be a superior ectopic thoracic kidney, which was easily identifiable on ultrasonography. The case is reported to stress the importance of recognizing thoracic kidneys in order to avoid unnecessary invasive investigations and/or thoracotomy.

Ten years' experience in the management of cervical teratomas.

Five patients with cervical teratoma were seen between 1982 and 1992 in the Maternity and Children's Hospital, Riyadh, Saudi Arabia. All presented with a cervical mass at birth. Their weights ranged between 2.6 and 3.5 kg (average 3.1 kg); all were full-term babies, three girls and two boys. Three cases were diagnosed antenatally; three had a history of maternal hydramnios; three presented with severe respiratory distress at birth and needed endotracheal intubation. All patients underwent surgical treatment except one who died before surgical intervention because of severe respiratory distress. Surgery included complete resection of the tumor. The postoperative courses were uneventful. The histopathology of the tumor showed tissue from all three germ layers. Over 3-11 years of follow-up, none of the patients showed signs of recurrence. Postoperative thyroid function tests and serum alpha-fetoprotein showed no abnormalities. We conclude that antenatal diagnosis help to reduce the morbidity and mortality due to upper airway obstruction. Surgery is safe and represents the only way of treating this tumor.

Clinical significance of preoperative nutritional status in pediatric surgical patients.

This prospective study was undertaken to correlate the preoperative nutritional status in pediatric surgical patients with postoperative morbidity. The prevalence of protein-calorie malnutrition in our patients is also evaluated. Sixty-two consecutive pediatric surgical patients were admitted elective to King Khalid University Hospital for different reasons. Seventy-four major and intermediate surgical procedures were carried out. The patients were assessed nutritionally on the day of admission by using anthropometric and biochemical methods. Patients were monitored for complications during their hospital course. Eighty percent of patients have one or more abnormal values suggestive of protein-calorie malnutrition. Six patients had 8.1% complication rate. Five of them showed moderate to severe malnutrition. The correlation between malnutrition and postoperative morbidity was evident but was not of clinical or statistical significance. And the predictive value of nutritional variables in predicting postoperative complications were difficult to assess in this study but they generally were of little help.