ABSTRACT
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
ABSTRACT
To determine the commonest cause of genitourinary fistulae and evaluate the experience of the surgical management. The study was carried out at the Urology Department, Jinnah Postgraduate Medical Centre [JPMC], Karachi. Twenty two patients were operated including 14 of Vesicovaginal Fistula [VVF], and 8 of Ureterovaginal Fistula [UVF]. In one patient of VVF fistula was extending up to proximal urethra. Out of 14 VVF cases, 9 patients underwent transvaginal repairs, 1 extraperitoneal transvesical repair and 1 transperitoneal repair. Three urinary diversions including 1 Mitrofanoff and 2 ileal conduits were also made. Ureteroneocystostomy was done in cases of UVF with adjuvant procedures in 3 cases including Psoas Hitch in 1 and Boari's flap in 2 patients. The mean age was 35 [range 21-50] years. VVF due to obstetric causes was seen in 71.4% patients while 28.6% developed VVF secondary to gynaecological procedures. Majority of [91%] VVF were repaired successfully in the first attempt in patients subjected to the procedure [78.6% cases of VVF]. There was only one recurrence in transvaginal repair, which was successfully treated with extraperitoneal transvesical approach in second attempt, thus augmenting the success rate to 100%. However 3 [21.4%] cases of VVF required urinary diversion as there was complete loss of sphincter mechanism in one case, in which fistula was extending up to the urethra, and in two cases of complex VVF with complete loss of posterior bladder wall surrounded by extensive scarring. All cases of UVF were successfully treated with ureteroneocystostomy alone or with adjuvant procedure in 37.5% cases. Uncomplicated VVF can be successful repaired transvaginally by refreshing the fistulous edges without excising it. Transabdominal route should be reserved for complicated VVF. Ureteroneocystostomy with antireflux mechanism with or without adjuvant procedure is the treatment of choice for UVF resulting from complete transaction or ligation of ureter