[Urinary hydrometrocolpos by persistent urogenital sinus; prenatal diagnosis and neonatal management]. / Idrometrocolpo urinoso da persistenza del seno urogenitale: diagnosi prenatale e management neonatale.

Urogenital Sinus (UGS) malformation can be ascribed to an arrest of normal embryonic vaginal development. Neonates with UGS frequently have ambiguous genitalia, rarely the vulva may be normal. The aim of this work is to define the role of prenatal sonography in the diagnosis of UGS associated with hydrocolpos and/or hydrometrocolpos. The Authors report their experience on 3 cases of UGS without ambiguous genitalia with hydrometrocolpos, in which prenatal sonography had shown a cystic dilatation in the pelvis. After birth the 3 neonates presented with female genitalia and a single orifice between the labia. The pelvis sonography showed in all the cases an hydrometrocolpos with a large vagina and a compressed and anteriorly located bladder. Voiding cystourethrogram, genitography and genitoscopy confirmed the presence of an UGS with urinary retention inside the vagina and stenosis of the distal portion of the vagina itself. An early drainage of the capacious vagina was performed in the three patients. There are very few reports in the literature of UGS with hydrometrocolpos diagnosed in utero. The cystic dilatation of the vagina is always misdiagnosed with a distended bladder. In utero, infact, the bladder can not be identified being displaced anteriorly by the vagina. The presence of a fluid-debris level inside the cystic anechoic mass must be considered a crucial finding. Multiple echoes are due to vaginal secretions. Prenatal ultrasound has then a definitive role in detecting an obstructed genital tract. This allows to rapidly drain the vagina relieving urinary tract obstruction.

Conservative treatment in primary neonatal megaureter.

The authors report their experience on 22 neonates (14 males and 8 females) with primary megaureter. In 18 patients a prenatal diagnosis was available, in the other four the diagnosis was suspected during a neonatal ultrasound screening. The dilatation involved the right ureter 7 times and the left 13 times, in 2 patients it was bilateral. Intravenous urography documented a type I ureteral dilatation in 2 renal units (8.3%), a type II in 9 (37.5%) and a type III dilatation in 13 (54.2%). Radionuclide scan (Tc99m DTPA) demonstrated in all the renal units with megaureter a stable function. From diuretic renal scan, non-obstruction appeared in 19 (83%), obstruction in 2 (8.5%) and an equivocal result in the remaining 2. Antibiotic prophylaxis was administered to all patients. Follow-up period ranged from 18 to 54 months. The 2 obstructed megaureter were surgically treated. Other 2 patients with type III dilatation and non-obstructing megaureter underwent surgery at 18 and 24 months of age respectively. The remaining 17 patients were all conservatively treated. The 2 patients with type I dilatation resolved spontaneously. Of 9 patients with type II megaureter the dilatation resolved in 6 patients and 3 patients had a good reduction. In the remaining 8 patients with type III megaureter, the dilatation resolved in 5 cases and 3 patients had only a fair reduction. The renal function (> 40%) remained stable in all the patients.

Disorders of the esophageal motor activity in atresia of the esophagus.

Esophageal dysfunction has been reported after successful repair of esophageal atresia but its nature has not been clearly defined. We studied esophageal motility in 20 newborns with esophageal atresia by recording intraluminal pressure of both proximal and distal segments. The investigation was made by pressure monitoring of the upper pouch via the mouth and of the distal segment via the gastrostomy. In all cases we found motility disorders. Two patients (12.5%) showed incomplete relaxation of the upper esophageal sphincter. The resting pressure of the esophageal body in both segments was constantly positive in all cases. Lower esophageal sphincter (LES) function was normal in all but two patients (16.7%) in whom the LES pressure was reduced and one case (8.4%) with incomplete relaxation of the LES. These studies suggest that motility disorders are also present in esophageal atresia before surgery.