Management of children with disorders of sexual development [DSD]: a retrospective analysis

We retrospectively reviewed data of children who were managed for Disorders of Sexual Development [DSD] presenting after one year of age with a view to understand gender assignment issues in these children. Patients were managed at NICH Karachi, NIRM and Shifa International Hospital Islamabad. All patients were investigated on standard lines to make a proper diagnosis. Karyotyping was performed in all patients. Hormonal essays included, serum testosterone levels, 17-OH progesterone levels, FSH, LH etc. Ultrasonography, urogenital endoscopy and laparoscopy were also performed in selected cases. The aim was to assign the genetic sex to the patient when possible. Male gender was advised to all male DSD responding to exogenous hormonal therapy. Patients with complete androgen insensitivity syndrome were advised female gender. Children having Gonadal dysgenesis [GD] responding to androgen therapy were also advised male gender. Female gender was advised to all children with congenital adrenal hyperplasia [CAH]. Gender assignment was performed after a detail consultation with the family and children if they were old enough to comprehend the issue. Male gender was assigned to CAH patients reared as male if child/family insisted to keep the gender of rearing. Of the 61 patients, 23 were undervirilized male [UVM], 29 had congenital adrenal hyperplasia [CAH], 4 had clitoromegaly, four gonadal dysgenesis and one aphalia. The mean age of presentation of UVM was 8.4 years and CAH was 7.06 years. Clitoromegaly without CAH mean age was 5.6 years. It was not possible to definitely establish the true nature of male DSD in few patients due to limitation of available investigations. Twenty two male DSD patients responded to exogenous testosterone therapy and had male gender assignment. One had female conversion as non-responder. Of the 29 cases of CAH, 27 decided for female assignment and had feminizing genitoplasty. Two children aged 13 and 16 years refused for a female gender assignment and were assigned male gender and reconstructions performed accordingly. Clitoral recession was performed in all the four patients with clitoromegaly. Four patient having MGD was assigned male gender. Single aphallia patient was assigned male gender however adequate phallus reconstruction is still awaited. Older UVM children reared as female accepted male gender happily but CAH children more than 10 years of age reared as male were not happy for a female conversion. Genital reconstruction was most satisfactory in CAH patients. Patients with male gender assignment had multiple procedures and patient's satisfaction response was variable according to the size of the phallus and severity of androgen deficiency. Most patients were however happy for the masculine appearance after chordee correction. Long-term results need to be evaluated. Older female children reared as male find it extremely difficult for female conversion whereas male children reared as female accept male conversion well. Gender re-assigment in younger children is well accepted by the family

Anterior sagittal approach for anorectal malformations in female children: early results

To determine the technical applicability and early postoperative outcome of anterior sagittal approach for anorectal malformations in female children. Study Design: Case series. Place and Duration of Study: Surgical Unit B of National Institute of Child Health [NICH], Karachi, from April to November 2007. Female patients with congenital anorectal malformation who underwent anorectoplasty through anterior sagittal approach were included in the study. Surgery was done either as primary or staged procedure [with initial colostomy or cut back]. Operative details were recorded. Follow-up was done in OPD. Thirty patients with mean age of 11.5 months underwent anorectoplasty through anterior sagittal approach. Eighteen patients had ASARP as a primary procedure. Staged procedure with initial colostomy was done in 9 patients. Initial cut back was done in 2 cases and one redo surgery done. Vaginal tear occurred in one, while partial tear of most distal part of fistula occurred in 4 children. At follow-up, 2 patients with primary ASARP developed wound infection with superficial disruption. Bleeding with wound disruption occurred in one case. Anal mucosal prolapse, anastomotic stricture and recurrent fistula occurred in one patient each. Cosmetic appearance of perineum was good in 10, satisfactory in 5 and poor in 3. Among patients staged with colostomy, bleeding with wound disruption, anal stenosis and retraction occurred in one case each. Cosmetic results were good in 7, satisfactory and poor in one case each. Two patients with initial cut back did not have any complication and one operated for disrupted wound developed disruption again. Anorectoplasty can satisfactorily be done through anterior sagittal approach in females with anorectal malformations. Primary ASARP has almost the same results as staged procedure, which should be done in selected patients

Splenic hydatid cyst: a rare site of involvement

A case of splenic hydatid cyst is reported in which the only complaint was left sided abdominal pain. Hydatid disease was diagnosed on ultrasound abdomen and CT scan. IHA was also significantly raised. Patient was kept on albendazole pre-operatively. On splenic exploration due to the presence of good splenic tissue cystectomy and capitonnage was done