Cellular mesoblastic nephroma: morphologic, cytogenetic and molecular links with congenital fibrosarcoma.

Congenital mesoblastic nephroma (CMN) is a rare renal tumor of early infancy with a favorable outcome after complete surgical removal. CMN consists of a heterogeneous group of spindle cell tumors subdivided into "classical", "cellular or atypical" and "mixed" forms based on histologic features. We describe a new case of cellular CMN diagnosed by antenatal ultrasonography with complete remission five years after nephrectomy.

Mesenteric adenitis caused by Yersinia pseudotuberculosis presenting as an abdominal mass.

Infection by Yersinia pseudotuberculosis has become of increasing pathological importance. Patients normally present with symptoms similar to those of appendicitis, due to mesenteric adenitis. We present the case of 3 patients infected by Yersinia pseudotuberculosis who in addition to fever and abdominal pain had a palpable abdominal mass, so great was the enlargement of the mesenteric nodes.

Prenatal detection of a congenital pancreatic cyst and Beckwith-Wiedemann syndrome.

We report a case of congenital pancreatic cyst detected prenatally by ultrasound in a fetus with evidence for a diagnosis of Beckwith-Wiedemann syndrome (BWS). Neonatal hypoglycaemia was prevented. The cyst was managed by internal drainage.

[Glomerular nephropathy with IgA mesangium deposits and Crohn disease].

Background: Renal disease is an unfrequent extraintestinal manifestation of chronic inflammatory bowel disease.

Case Report: A 12-year-old girl suffered from recurrent abdominal pain, diarrhea and growth impairment due to Crohn's disease of ileocaecal region. After six months of nutritional rehabilitation, an ileo-caecal resection was performed because of intestinal stenosis.

[Total colonic form of Hirschsprung disease. Treatment and long-term follow-up in 16 cases].

From 1971 to 1994, 16 cases of total colon Hirschsprung's disease were treated at the University Hospital in Rennes. Diagnosis have been at 2 days to 3 months. Two children had a family history of Hirschsprung disease among which one associated megacolon and multiple endocrine neoplasia.

[Replacement continent bladder in dogs by opening an antro-pyloric graft onto the abdominal wall (seven cases between 1980 and 1983)].

To determine the usefulness of antral bladder controlled by the pyloric sphincter denervated for urological applications, bladder replacement was performed in 17 dogs using the antral segment with its own sphincter, initially with the pylorus supplied by the left gastro-epiploic artery, and later by the right gastro-epiploic artery. The smooth pyloric muscle was denervated to obtain permanent spasm and pulled through the striated anterior abdominal muscle. Both ureters were anastomosed by a Cohen's procedure.

Urinary fungal bezoars in children--report of two cases.

We report two cases of urinary obstruction by fungal bezoars in full-term neonates who presented a uropathy detected antenatally. Early percutaneous urinary diversion was performed to relieve renal impairment secondary to a primary megaureter in the first case and to bilateral pelvi-ureteral obstruction in the second. Acute fungal obstruction occurred first on the side of the primary megaureter and then on the healthy side in the first patient.

[Digestive stenoses following ulcero-necrotizing enterocolitis].

Over a 10-year period, the authors followed the course of 112 neonates treated for primary acute necrotizing enterocolitis. Ten cases of gastrointestinal stricture were diagnosed, regardless of initial treatment, after an interval of 20 days to 6 months. The site of the stricture was colonic in 6 patients, ileal in three other patients and ileal and colonic in the remaining patient.

Remarks about the prognosis in case of antenatal diagnosis of gastroschisis.

We report our experience of 15 cases of gastroschisis which occurred between 1981 and 1993. All but one were diagnosed antenatally by ultrasound between 16 and 32 weeks of pregnancy. We made a termination of the pregnancy in 3 cases, for multiple malformations in 2 cases and one case of very early premature rupture of the membranes (PROM).

Renal cystic tumours in children--a diagnostic challenge.

A series of 8 cystic renal tumours is reported in seven-months to four-years-old children. The final diagnosis was cystic nephroma (multilocular cyst) in 4, cystic, partially differentiated nephroblastoma in 3 and partially cystic nephroblastoma in one. Pre-operative distinction between those three types is difficult and inadequate therapeutic approach may result from a wrong diagnosis.

[Cloacal exstrophy: what therapeutic approach for what result?].

The authors report 11 cases of cloacal exstrophy and analyse the anatomical features and the results of surgery. The outcome of three patients aged 20, 18 and 9 years is related. Advances in prenatal assessment of malformations is likely an explanation for the lack of new cases in the recent years.

[Renal agenesis, bicornuate uterus and cyst of the Gartner's duct].

A cyst of Gartner's duct with a bicornuate, unicervical uterus and ipsilateral renal agenesis were seen in a 13-month-old girl. Left renal agenesis had been suggested by antenatal ultrasound findings. After birth, there were no symptoms but ultrasonography demonstrated a multicystic mass in the pelvis.

[Millard's flap for sequelae of high anorectal malformations. Apropos of 6 cases].

Mucosal prolapse is a frequent sequela after surgical treatment of the high type of anorectal malformation whatever was the technic used for the pull-through operation. The authors report six cases treated with the spiral flap described by Millard. The initial procedures had been a Romualdi-Rehbein pull through in three cases with secondary revision of anoplasty and a Stephan's operation in the three other cases.

[Diffuse esophageal leiomyomatosis. Apropos of 3 cases].

Three cases of diffuse esophageal leiomyomatosis are discussed. Two of these are familial one, the mother being affected. These familial cases can occur in association with Alport's syndrome.

[Anterior hypospadias and chordee: a one-stage cure using a tubulated and reversed flap].

A one stage urethroplasty using a tubulated and reversed flap was carried out by the authors in 74 cases of hypospadias with chordee between 1978 and 1988. The procedure is derived from the technic of Mustarde and the flip flap of Devine and Horton. The experience led to limit its indication to the patients who have a sufficient length of ventral skin of good quality between the hypospadic meatus and the peno scrotal junction to build the urethra up to the apex of the glans after correction of the chordee.

[Torticollis in children: do not forget the Sandifer syndrome].

We report a case of Sandifer syndrome with chronic torticollis and gastroesophageal reflux (GER). The infant exhibited regurgitations and vomiting from birth. Torticollis with a permanent tilt of the head towards the right developed at age six months.

[Duplication of the urethra in a young girl. Apropos of a case].

Duplication of the female urethra is quite uncommon. A case of complete urethral duplication is reported in a 2.5 year old girl seen for pyuria.

[Anterior rectal duplication. Value of ultrasonic diagnosis].

A case of neonatal anterior rectal duplication is reported. Abdominal ultrasonography revealed a prerectal cystic mass. The different types of rectal duplications and the main differential diagnosis are described.

[A duplication of the alimentary tract with unusual localization in the submaxillary region].

Duplication cysts developed from the intra-oral digestive tract are exceedingly rare lesions of which only 15 cases have previously been reported. We report an additional case in a neonate with a large cystic lesion in the right submandibular area. Clinico-pathologic findings are described and the literature is reviewed.

[Accidental ingestion of caustics in children. Apropos of 100 cases].

One hundred children (67 boys, 33 girls) underwent early fiberoptic endoscopy (without general anesthesia in 96 cases) for caustic ingestion between January 1985 and June 1988. The intended use of the product was household (83) industrial (4) farm (6) or medicinal (7). Caustic burns were classified as grade 1 (mucosal hyperaemia), grade 2 (ulceration) and grade 3 (necrosis).

[The multiseptate gallbladder. A rare malformation of the biliary tract].

Multi-septate gallbladder is a very rare anomaly. In this 13-years-old girl with a complaint of nausea, vomiting and abdominal pain, sonographic examination and oral cholecystogram demonstrated a normal size gallbladder with many thin septa. The longitudinal section of the removed gallbladder revealed multiple inter-communicating lobules divided by many thin septa.

[Diffuse esophageal leiomyomatosis. Apropos of 5 cases with 2 familial cases].

Diffuse esophageal leiomyomatosis is characterized by diffuse muscular hypertrophy chiefly marked in the lower end of the esophagus looks at a large tumor. It involves the entire length of the esophagus and down to the upper part of the stomach (esogastric leiomyomatosis). Diffuse leiomyomatosis is a extremely rare pathology encountered in children (13 previous report cases) and young adults.

Primary megaureter in the neonate with prenatal or postnatal diagnosis.

Twenty-nine cases of primary megaureter diagnosed before the age of 3 months are grouped in this diagnostic and therapeutic study. Seventy-five per cent were discovered by antenatal sonography due to the presence of pelvicalyceal dilatation without localising precisely the position of obstruction, twenty-five per cent were detected by the occurrence of early urinary tract infection. Four patients were operated during the first few months.

[Oto-palato-digital type I syndrome in five generations. Relationship to the type II form].

Three cases of oto-palato-digital syndrome (OPD) are described. They are from the same family, in which the syndrome is an X linked recessive disorder, transmitted through five generations. These cases are classified rather in the OPD type I.