A rare complex bladder exstrophy variant.
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Bladder Exstrophy↗
Biomedical subjects
I Valioulis
Publications and source records attributed to I Valioulis.
Enterocystoplasty complications in children. A study of 30 cases.
BACKGROUND/PURPOSE: Bladder augmentation for small vesical capacity represents an important aid to these patients, but the appearance of complications is not rare. We analysed the complications of the enterocystoplasty techniques used in our Department. METHODS: From 1983 to 1997 sixteen girls and fourteen boys were treated. We performed 28 bladder augmentations (in 13 cases using small bowel, in one case using ileum and caecum and in 14 cases using sigmoid colon) and 2 bladder substitutions (in one case with ileum and sigmoid colon and in the second using a colonic segment). RESULTS: Twelve children present recurrent urinary infections. In five children bladder stones were formed (among them a case of familial cystinuria-lysinuria). Two children presented intestinal volvulus and another one a caecal volvulus. In two children a perforation of the augmented bladder was treated operatively. An 11-years-old child presented severe electrolyte disturbances. CONCLUSIONS: The complications presented in our patients confirm the view that they are associated with intestinal mucosa in the bladder and our study proves, once more, the current necessity to seek alternative tissues for bladder augmentation.
Adolescent↗
A complex chromosomal rearrangement associated with Hirschsprung's disease. A case report with a review of the literature.
The authors present a de novo complex chromosomal rearrangement 46,XY,t(2q;3p;4q;13q) associated with Hirschsprung's disease. They review the literature concerning genetic aspects of Hirschsprung's disease focusing on genetic studies and recent molecular research. Genetic aspects of intestinal neuronal dysplasia are also briefly discussed.
Abnormalities, Multiple↗
[Torsion of the spermatic cord. Diagnosis, emergency treatment].
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Adolescent↗
Intrapericardial teratoma diagnosed prenatally in a twin fetus.
Prenatal diagnosis at 32 weeks' gestation of an anteromediastinal tumor in a twin fetus allowed immediate neonatal intensive management after delivery at 34 weeks' gestation. At 48 h of age the patient underwent a median sternotomy; complete resection of the tumor was possible. Histologically, it was a mature teratoma. At age 1 year both twins are well.
Adult↗
Cricopharyngeal achalasia--a cause of major dysphagia in a newborn. A case report.
Cricopharyngeal achalasia is a rare cause of neonatal dysphagia. Its treatment is based on surgical myotomy, which is to be avoided in the presence of prematurity and neurological diseases. The authors report a case of cricopharyngeal achalasia in a full-term four-month-old female baby. Coughing and choking during feeds were the major symptoms. The diagnosis was made peroperatively although barium meal and endoscopic findings were suggestive. After cricopharyngeal myotomy, symptoms took several weeks to disappear. Seven months after surgery, she feeds normally and weighs 7,700 g. Neonatal cricopharyngeal achalasia could be a foregoing state to cricopharyngeal diverticula in adult. Some cases may remain undiagnosed either due to lack of symptoms or sudden infant death.
Cricoid Cartilage↗
Reversed midgut rotation in a neonate: case report with a brief review of the literature.
The authors report a case of reversed rotation of the midgut in a neonate and review the embryology, the clinical and radiographic findings, and the operative techniques. They also present radiographs of their case, which are the first ever published of a neonate with this retroarterial type of reversed rotation.
Congenital Abnormalities↗
Abdominoscrotal hydrocele.
Hydrocele is among the commonest inguinal anomalies in children. One of its most unusual variants is the abdominoscrotal hydrocele, of which no more than 22 cases have been reported in the world literature. The present case is the first one in our country. In this patient the coexistence of small bilateral scrotal hydrocele and cystic abdominal mass at the right side is described. Abdominoscrotal hydrocele is an unusual cause of lower abdominal cystic tumour in association with an inguinoscrotal hydrocele in infancy.
Abdomen↗
Gallbladder disease in patients with cystic fibrosis.
We retrospectively studied 18 patients with cystic fibrosis, who underwent surgery for gallbladder disease from 1975 to 1990. A long delay between the onset of symptoms and the diagnosis was noted (mean 8.7 months). This delay was attributed to masking of the symptoms of biliary disease by the malabsorption and pulmonary symptoms seen in this patient population. We do not recommend routine intraoperative cholangiography in patients with cystic fibrosis and gallbladder disease. Cystic fibrosis is a disease with progressive pulmonary deterioration. Cholecystectomy can be performed in these patients with relative safety if careful preoperative and postoperative care is provided. We recommend early operative intervention in the patient with gallbladder disease and cystic fibrosis.
Adolescent↗
[Anatomic abnormalities of the genital tract in ano-rectal malformations].
Of 152 patients with imperforate anus, 16 girls -25%), 10 boys (15%) have an abnormality of the genital tract and real frequency seems underevaluated. In girls, vaginal septum, bicornuate and didelphys uterus are mostly diagnosed, associated with severe unilateral upper urinary tract malformations. Absent vagina and uterus (Rokitansky syndrome) must be seen at birth, to preserve perineal fistula used to fashion a vagina at the time of the abdomino-perineal fistula pull through. In boys hypospadias, ectopia and uretero-vas anastomosis are the most frequent malformations.
Abnormalities, Multiple↗