Amenorrhea after Endoscopic Third Ventriculostomy for a Failed Shunt in Spina Bifida: Case Report and Review of the Literature.

BACKGROUND: Secondary endoscopic third ventriculostomy (ETV) for the management of shunt failure may be efficacious, though it may be followed by more frequent complications (including endocrinological impairment, e.g., amenorrhea) compared to primary ETV. These complications are usually underreported in the literature. AIM: We report a case of secondary amenorrhea after ETV for the management of shunt failure in a young woman with hydrocephalus associated with myelomeningocele. METHODS: A 25-year-old woman affected by hydrocephalus and myelomeningocele was admitted for secondary ETV for the management of shunt failure. The endoscopic procedure was preferred over shunt revision based on good results of secondary ETV, especially in patients with hydrocephalus associated with Chiari II malformation and spina bifida. RESULTS: Despite the surgery being uneventful, the patient had early (postoperative seizure) and late (secondary amenorrhea) complications. In the early postoperative period, she received external ventricular drainage followed by VP shunt reimplantation 2 weeks later. There was no neurological morbidity, but 1 month after the ETV she reported secondary amenorrhea and weight gain. Laboratory investigations ruled out hyperprolactinemia, which had been treated with cabergoline administration with no efficacy since the patient was still without regular periods 1 year later. CONCLUSION: ETV may be followed by endocrinological complications like amenorrhea that are rarely reported.

Long-term growth hormone treatment in a boy with 45,X/46,X,idic(Yp) mixed gonadal dysgenesis: comparison with growth pattern of an untreated patient.

BACKGROUND: Mixed gonadal dysgenesis (MGD) is a rare disorder. Short stature is a well known feature of this condition. Although growth hormone (GH) treatment has been suggested to treat growth impairment, conflicting data surround this issue. CASE REPORT: We report on long-term growth hormone (GH) therapy at pharmacological doses (0.33 mg/kg/week) in a boy (age 4.6 years) with MGD [karyotype 45,X/46,X,idic(Yp)]. An untreated boy of similar karyotype and growth delay served as control. The treated boy showed a progressive improvement of stature during GH administration. His height completely normalized after 6.5 years of treatment and he reached his target height centile before puberty onset. In the untreated boy, no improvement of growth pattern was found. CONCLUSIONS: We conclude that short boys with MGD and 45,X/46,X,idic(Yp) karyotype may benefit from early GH therapy at pharmacological doses. Evaluation of larger patient samples and additional follow-up till final height are needed to reach definitive conclusions as to the optimal growth-promoting therapy for this disorder of sex development.

Effectiveness of primary endoscopic incision in treatment of ectopic ureterocele associated with duplex system.

OBJECTIVES: To report our experience with the endoscopic treatment of ectopic ureterocele to demonstrate its long-term effectiveness. Endoscopic treatment is often recommended as the initial and definitive treatment in patients with ureterocele. METHODS: A total of 46 children with ectopic ureterocele in a duplex system underwent primary endoscopic incision from January 1998 to January 2006. The mean follow-up was 3.8 years. Of the 46 children, 35 had been diagnosed prenatally and 11 had been diagnosed after birth because of a urinary tract infection. Low-dose antibiotic prophylaxis was administered to all children and was maintained until voiding cystourethrography showed no reflux. The pre- and postoperative evaluation included clinical assessment, ultrasound evaluation, diethylenetriaminepentacetic acid renography, and cyclic voiding cystourethrography. RESULTS: Ureterocele decompression was achieved in 43 patients (93%). Three patients required additional surgery for persistent ureterocele (1 underwent ureteroureterostomy and 2 ureteropyelostomy). None of our patients showed deterioration of renal function after the procedures. Vesicoureteral reflux was seen in the lower moiety of the ipsilateral kidney in 14 patients (30%). Of the 14 patients with vesicoureteral reflux, 10 had spontaneous resolution. The remaining 4 underwent endoscopic correction. Five patients (10%) developed de novo vesicoureteral reflux in the ipsilateral ureterocele moiety. Of these 5 patients, 3 were treated with endoscopic injection and 2 had spontaneous resolution after 6 months of follow-up. CONCLUSIONS: Our data have shown that primary endoscopic puncture of a ureterocele is a simple, long-term, effective, and safe procedure, avoiding complete reconstruction in most patients.