Anorectal atresia. An experimental model in the rat.

Aiming to assess the development of the anorectal sphincteric apparatus and whether it is influenced by the presence of an intestinal conduit through the muscular complex, we seek to develop an experimental model of anorectal atresia in foetuses of female pregnant rats, given orally the herbicide ethylenethiourea (ETU). Six 3-months-old, 300 g female Wistar rats were included in the study group (SG). The foetuses of another unexposed rat formed the control group (CG). In the SG 54, foetuses were alive, all had hypoplasia of the tail and 47 (87%) had no visible anal orifice. The 18 foetuses of the CG were normal. The histological study was performed at the level of the pubic symphysis. Type I anorectal atresia was defined when this section demonstrated an intestinal conduit and Type II when only a muscular cord was found, without any intestinal tube. This study was completed in 15 (32%) of the SG, 9 (60%) with anorectal atresia Type II and 6 (40%) with atresia Type I, and in 11 animals (61%) of the CG. The thickness of the sphincter muscle was larger in the CG (260.47+/-35.32 micro) than in the SG (208.98+/-55.01 micro), p<0.05, but this difference was due to the animals showing atresia Type II. To conclude: ETU had a teratogenic effect. The foetuses with atresia, particularly Type II, had a decreased development of the muscular complex. The presence of an intestinal conduit does not significantly influence the development of the muscular complex.

Gastroesophageal reflux in association with the Sandifer syndrome.

Eight children presenting with the Sandifer syndrome (with neck contorsion, radiologic studies of the cervical spine and normal neurologic exploration) have been studied in relation to gastro-esophageal reflux (GER). In the eight cases barium swallow, 24 h pH-metering, manometry, endoscopy and biopsy were made, presenting pathological GER. The barium swallow was pathologic in 62% of them. The pH-metering in 37%. The lower esophageal sphincter pressures were decreased in 37%, with esophageal motility alteration in 75%. Signs of macro and/or microscopic esophagitis were found in 62%. Three patients received surgical treatment and the rest medical treatment, with improvement of the neck contorsion in all cases. We have attempted to evaluate which one of the functional explorations for the GER diagnosis is better in this kind of patients, and we have demonstrated that the most frequently found alteration is the esophageal dysmotility.

[Syringoceles of Cowper's glands in children]. / Siringoceles de las glándulas de Cowper en niños.

Some tubular or cystic diverticula observed in the ventral wall of the male bulbous urethra that originate from dilatation of Cowper's gland ducts have been termed "syringoceles". They can cause dysuria, hematuria and obstruction. We report 14 syringoceles and 1 true diverticulum from segmental absence of spongious urethra. We review embryology, anatomy and diagnosis in order to determine the best therapeutic approach. We performed endoscopic resection of the distal valve-like border of the diverticulum in 6 patients (42.85%) with good results. The remaining 8 patients, in whom surgery was not indicated, were followed clinically and radiologically.

[Pilomatrixoma: a frequent and little known pediatric tumor]. / Pilomatrixoma: un tumor pediátrico frecuente y poco conocido.

Pilomatrixoma is a benign dermal tumor which is quite frequently observed, but although 50 % of the cases are treated during childhood, it is not well known among paediatricians and paediatric surgeons. Authors report 12 cases localized mainly in the face, neck and upper limbs. Tumors were located in deep dermal layers, they were small, hard and brown or yellow-colored. Microscopic examination revealed basophilic cells which were quite similar to those of basocellular carcinoma, and took origin from hair matrix, and momified or "ghost" cells embedded in a giant cell stroma; lesion was well limited and extended down to subcutaneous fat. In two cases there was a history of infection or insect bites in the site of the lesion and in another one two pilomatrixomas were successively removed from a child whose family had von Recklinghausen's disease. In the majority of patients, preoperative diagnosis was not made, a fact witnessing that this is badly known and justifies this report.

Hydatid renal cyst with characteristic sonographic pattern. A case report.

The kidney is a rare localisation of the hydatid cyst even in countries with a high rate of infestation. We report a case of renal hydatid cyst in a five-year-old girl. Conventional radiology did not yield any diagnosis, whereas in this case renal ultrasonography showed characteristic echographic signs pointing to the origin of the disease.

Cryptorchidism and persistence of Müllerian remnants in a normal male.

A four-year-old boy was explored for bilateral cryptorchidism. Operation led to the discovery of two intraabdominal testes with their corresponding vasa, two Fallopian tubes and a small uterus. No urological anomalies were found except for a vesico-ureteral reflux. Cystourethroscopy revealed a normal male tract. Sexual chromatine was negative and karyotype was 46, XY. A normal rise in testosterone serum level was recorded after stimulation with CGH. At operation both gonads could be placed extra-abdominally and the female internal structures were biopsied and left in place. This very unusual condition is derived from the absence of regression of Müllerian structures during fetal life due to a deficit in testicular secretion of anti-Müllerian hormone. The current knowledge on this topic is updated.

[Mesenteric cystic lymphangiomas (report of three cases) (author's transl)]. / Linfangiomas quísticos del mesenterio. Estudio de tres casos.

Three cases of mesenteric cystic lymphangiomas in children are reported. All of them were found during laparotomies for acute abdomen, and their pathology was rather similar, except for the contents which was chylous in the two cases located in the jejunum and serous in the remaining ileal case. One of these tumours contained calcified material, a fact which makes diagnostic suspicion possible. The literature on this topic is up-dated.

[Rectal agenesis with absent bladder in a female newborn (author's transl)]. / Agénésie rectale avec absence de vessie chez une fille.

This paper reports a very unusual variety of female ano-rectal agenesis without fistula associated with severe urogenital malformations. The patient died of renal failure and, at autopsy, both ureters draining dysplastic ectopic kidneys and two vaginas, either with its corresponding uterus and annexa were found opening to s single perineal orifice through a very narrow conduit. Bladder and urachus were absent and a single left umbilical artery was found. This infant had also thoracic vertebral anomalies.

[Gallstones in infancy (author's transl)]. / Litiasis biliar en la primera infancia.

Authors present a 22 month old female, suffering from biliary lithiasis, diagnosted before surgery by endovenous cholangiography, associated to cataracts and neurological impairment. Current etiopathogenic views are briefly exposed, pointing out that incidence of gallstones has ceased to be exceptional.