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). When checked (11 cases), the karyotype was normal. We made a cesarean section in 11 cases: the indication was a complication for 6 (fetal distress, PROM, polyhydramnios, large dilatation of the gut). We noted growth retardation in 7 newborns and prematurity in 5/12 (mean gestational age of 36.8 weeks). The preoperative study of the gut noted 5 cases with intestinal damage and one case of complete necrosis of the gut. The global prognosis is not as good as usual, with a perinatal mortality of 41.6% (5/12). We discuss this latter point and examine the literature.

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. Symptoms of infection and impaired renal function led to a diagnosis of fungal bezoar. In the second patient the development of the bezoar was more insidious and occurred after surgical correction of the obstructive pelvi-ureteral junction on the left side. Candiduria was the first sign in both cases. Ultrasonography is the best method to visualize fungal masses within the collecting system. In most cases, percutaneous nephrostomy allows relief of the obstruction, sampling of urine for culture and irrigation with amphotericin B. However, additional surgical intervention may be necessary. Systemic antifungal treatment using mainly 5-flucytosine is also given.

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. In 2 patients a laparotomy was carried out, followed by biopsy of a mesenteric lymph node. The diagnosis of Yersinia infection was confirmed by bacterial culture of the biopsied material and also by serology. In the third patient, serological studies and ultrasonic imaging of the abdomen led to early diagnosis and surgery was avoided. We suggest that a diagnosis of mesenteric adenitis due to Yersinia pseudotuberculosis should now be considered in all patients presenting with an abdominal mass, and in whom there is an appropriate clinical and epidemiological history. The diagnosis should be confirmed by abdominal ultrasound (alternatively Computerised Axial Tomography or Magnetic Resonance Imaging) and serological studies. In this way, unnecessary surgery can be avoided.

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. Progress in imaging techniques allows a better analysis of cysts and septa. If no solid part can be detected in the tumour, total nephrectomy is sufficient to obtain a favourable outcome.

A new method of ventilation with a mixture of helium and oxygen in the management of congenital diaphragmatic hernia. A preliminary study.

To prevent the severe prognosis of pulmonary hypoplasia in congenital diaphragmatic hernia, the authors developed a new method of ventilation, using a modulated mixture of helium and oxygen. Associated with a leptanalgesia (Chlorpromazine-Morphine), the authors observed with this method two good results. Efficiency of this technique has been confirmed by X-rays and successive pulmonary scintigraphies (Xenon 133).

[Digestive stenoses following ulcero-necrotizing enterocolitis]. / Les sténoses digestives post-entérocolite ulcéro-nécrosante.

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. The stricture was treated surgically in 9 patients (resection-anastomosis in 6 cases, resection with gastrointestinal diversion in 2 cases and isolated gastrointestinal diversion in 1 case). A follow-up barium enema in 1 patient showed spontaneous resolution of the stricture. With a mean follow-up of 5 years, the over all result was considered to be good in 7 cases and poor in one case. Two deaths were observed, one on the 10th day and the other at the 3rd postoperative day. These complications appeared to be related to the delayed diagnosis and treatment of the stricture.

[Glomerular nephropathy with IgA mesangium deposits and Crohn disease]. / Néphropathie glomérulaire à dépôts mésangiaux d'IgA et maladie de Crohn.

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. The surgical procedure was followed by parietal abcess and cutaneous fistula. One year later, a purulent secretion came out of the fistula associated with fever, hematuria and acute renal failure. Renal biopsy confirmed IgA nephropathy. The course was favorable under parenteral nutrition and after surgical closure of the sigmoido-cutaneous fistula. The microscopic hematuria only persisted but the nephropathy did not relapse even during a further digestive exacerbation. CONCLUSION: IgA nephropathy has been reported in association with chronic inflammatory bowel disease. Its mechanism remains unclear: increased mucosal IgA production in inflammatory bowel, increased serum IgA and/or immune complex deposition in the renal mesangium appear the most relevant hypotheses.

[Skin lesions and joint involvement in purpura fulminans (author's transl)]. / Séquelles cutanées et ostéoarticulaires majeures du purpura fulminans. Quatre observations.

Until recently, purpura fulminans had a very prognosis. It can now be treated and the patient may survive but extensive skin necrosis may lead to septic arthritis. The authors have observed 4 cases of this disease. 3 had to suffer amputation of a lower limb. They describe the type of care recommended in order to limit the extent of the necrosis.

[The treatment of upper femoral unicameral bone cysts in children by Ender's nailing technique]. / Indication inhabituelle de l'enclouage de Ender: le kyste osseux fémoral de l'enfant.

The authors have operated on 2 children aged 7 and 8 years who had developed a large unicameral bone cyst in the trochanteric and upper femoral diaphysial region. Ender's nails were inserted into the bone above the lower femoral epiphysial plate. Healing occurred rapidly.

[Coralliform lithiasis in children. Apropos of 20 case reports]. / La lithiase coralliforme chez l'enfant. A propos de vingt observations.

The authors recall the characteristics of infantile staghorn calculus, in relation to twenty personal cases. The complaint is more frequently encountered in boys than in girls. A frequent etiology is proteus urinary infection. The morphology of the staghorn and the state of the parenchyma are determining factors in the treatment. A sinusoid morphology and thickness of the renal parenchyma are operative indications. The bivalving operation is exceptional. Results are generally satisfactory (80% of cases), and recurrences are rare.

[Replacement continent bladder in dogs by opening an antro-pyloric graft onto the abdominal wall (seven cases between 1980 and 1983)]. / Etude d'une vessie continente de remplacement chez le chien par ouverture à la paroi abdominale d'un greffon antro-pylorique (sept cas réalisés de 1980 à 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. The best results were obtained with a gastric pouch supplied by the right gastro-epiploic artery; dissection and reimplantation of the ureters in the gastric wall are difficult. IVP and cystography indicated good function of the antral pouch, with no reflux. The antral bladder controlled by the denervated pyloric sphincter could be a possible alternative in the surgical management of neurogenic or exstrophic bladder.

[Congenital esophagotracheal fistulas without esophageal atresia in children]. / Les fistules oeso-trachéales congénitales sans atrésie de l'oesophage chez l'enfant.

Ten cases are reported of children presenting isolated, congenital esophagotracheal fistulae without esophageal atresia, or "H-type-fistula" of Anglo-Saxon authors. The malformation is rare, of difficult diagnosis and often associated with other malformations. Respiratory symptomatology is sometimes dramatic, but diagnosis is based on data from radiologic imaging of the esophagus and from esophageal and tracheobronchial endoscopy. Treatment is surgical by a cervicotomy approach due to the almost constant high localization of the lesion.

[Ectopic insertions of single ureters in children. Apropos of 9 cases]. / Abouchements ectopiques d'uretères simples chez l'enfant. A propos de 9 cas.

The authors present 9 cases of ectopic implantation of a single ureter in children. Ureteric ectopia is part of a congenital anomaly of the whole of the urinary tract, which explains the incidence of renal ectopia and associated dysplastic lesions of the renal parenchyma. The authors stress the importance of ultrasonography and, more recently, the CT scan for the localisation of the kidney homolateral to the ureteral ectopia. An analysis of the treatment and the results demonstrates the contrast between the unilateral forms which are easily treated and which have a good prognosis and the bilateral forms which are more difficult to treat and have a less favourable prognosis.

Obstructive uropathies diagnosed in utero. The postnatal outcome--a study of 43 cases.

The ultrasonic antenatal diagnosis of obstructive uropathies is now common. This study of 43 cases in which the mean follow-up is more than 2 years already allows us to show the importance of early diagnosis. The importance of a thorough postnatal examination is emphasised as well as the importance of preventing urinary tract infection. Although relief of the obstruction in the neonatal period in many cases allows an excellent recovery due to the exceptional qualities of the urinary tract at this age, there remain serious uropathies associated with significant renal dysplasia, for which ultrasonic diagnosis in utero is still insufficient. The results obtained allow us to be optimistic regarding the prognosis for many of these children.