[Cardiac failure by major arterial hypertension secondary to nephroblastoma]. / Insuffisance cardiaque par hypertension artérielle majeure secondaire à un néphroblastome.

The authors report on the case of a 7 week-old boy, in whom a renal mass was discovered after general symptoms were observed. Within 48 h, cardiac failure secondary to systemic arterial hypertension occurred, requiring intensive care. After a few days of mechanical ventilation and alternating elevated and low blood pressure, improvement was obtained with captopril and frusemide enabling further investigations to be carried out which lead to the diagnosis of Wilms tumor. During left-sided nephrectomy, elevated renin from the left renal vein was found. The post surgical course was excellent. Several authors have reported on the association between arterial hypertension and nephroblastoma as being the result of hyperreninism due to hilar compression; however severe hypertension was uncommon. Renin activity determination from the tumoral tissue had led to a different interpretation, ie primary hyperreninism: in the case of mesoblastic nephroma, only the non tumoral but compressed tissue contains a large quantity of renin; in the case of nephroblastomas, only the tumoral tissue contains renin. The question now is whether all or only certain nephroblastomas secrete renin.

Proteus syndrome in 7 patients: clinical and genetic considerations.

The Proteus syndrome is a congenital hamartomatous disorder delineated in 1983. Because of its polymorphic appearance, the syndrome was named after the greek god Proteus whose name means much less than the polymorphous much greater than. Major clinical findings include hemi hypertrophy, macrodactyly, exostoses, scoliosis, epidermal nevi, haemangiomas, deeply rugated soles of the feet and a variety of deep and subcutaneous masses. We report on 7 new cases of Proteus syndrome. All reported cases have been sporadic. Therefore this syndrome could be due to the action of a dominant lethal gene surviving by mosaicism.

[Necrotizing enteritis during the therapeutic induction phase in leukemia. Value of surgical treatment]. / Entérite nécrosante à la phase d'induction thérapeutique d'une leucémie. Intérêt du traitement chirurgical.

A case of typhlitis in a 3,5 year old girl, during induction therapy for acute lymphoblastic leukemia is reported. This typhlitis, or necrotizing enterocolitis involving the coecum and right colon resulted in stercoral peritonitis during the neutropenic phase. After surgery, the patient had a favorable outcome with complete recovery. Knowledge about this uncommon but severe complication of hemopathies leads to follow clinical, microbiologic and radiologic rules of prophylaxis and screening. Typhlitis requires early treatment by supportive care and surgical cure if necessary.

[Antenatal diagnosis of 2 cases of intra-abdominal solitary cyst]. / Diagnostic anténatal de deux cas de kyste solitaire intra-abdominal.

On the occasion of 2 cases, torsion of an ovarian cyst and volvulus of a cystic intestinal duplication, the authors emphasize the usual benignity of fetal intra-abdominal solitary cystic pictures, among which the most frequent are the ovarian cysts. Antenatal diagnosis allows for detection of complications, especially torsions and for early neonatal management always leading to surgical treatment.

[Blunt injuries of the pancreas in children. Diagnostic and therapeutic approach apropos of 8 cases]. / Traumatismes fermés du pancréas de l'enfant. Approche diagnostique et thérapeutique à propos de 8 cas.

From 1970 to 1985, eight severe blunt pancreatic traumas were admitted. There is significant difference in morbidity between early pancreatectomy with or without splenectomy (a mean hospital stay of 23 days, low loss of weight) and initial simple external pancreatic drainage with delayed partial pancreatectomy or pancreato-cystojejunostomy Roux-en-Y (mean hospital stay of 45 days, 24% loss of weight, one death two years later). Thus it seems essential to diagnose the pancreatic injury and particularly rupture of the pancreatic duct. Aiming to evaluate these lesions, biology, ultrasonography or computerized axial tomography proved insufficient. As a result, in the last two patients, an endoscopic retrograde pancreatography was performed confirming total transection of the pancreatic duct, one case with fistula, the other with a contrast fluid stop. The surgical approach was guided by these X-ray findings and a distal pancreatectomy performed preserving spleen. The authors propose the following protocol: endoscopic pancreatography if an evident improvement in recent pancreatic injury is not obtained in 48 hours, or in the case of former complicated pancreatic trauma; in the event of total rupture of pancreatic duct, operation should be carried out: abdominal exploration guided by the X-rays findings, distal pancreatectomy or, rarely, repair of the pancreatic duct; when no pancreatic duct lesion is found, but ultrasonographic blunt trauma patent, the surgical decision depends on the amount of peripancreatic reaction: medical treatment or external drainage; when lesion of the head of pancreas is detected, conservative treatment is to be preferred to pancreatoduodenectomy.

[Use of the Pavlik harness before the age of 3 months in congenital hip dislocation]. / Utilisation du harnais de Pavlik avant l'âge de 3 mois dans la luxation congénitale de hanche.

The authors report 128 cases of reduction of congenital dislocation of the hip by the Pavlik harness with more than one year of follow-up. They studied 60 patients who had less than 3 months of age at the beginning of the treatment. They obtain 59 reductions (98%) without necrosis of femoral heads. The only case of unsuccess, treated after by continuous extension, has doubtful femoral heads at 15 months of age. The femoral heads appear in normal delay and walk was normal between 10 and 18 months. Only a strict watchfulness, as strict as a continuous extension, can obtain good results with progressive flexion. Bath is not authorized and harness is not removed before stable reduction. Parents are taught how to hold their infant without bringing his knees together. The most important thing is the X-ray control on D8, D21, and D60, the prevent the trap of too much flexion, too nice penetration or persistent eccentration. Under these conditions, the authors think that the treatment of congenital dislocation of the hip by the Pavlik harness is the safest method especially before 3 months of age.

[Has prenatal ultrasonic diagnosis modified the prognosis of intestinal atresia? Results of a multicenter study]. / Le diagnostic échographique prénatal a-t-il modifié le pronostic des atrésies intestinales? Résultats d'une étude multicentrique.

A multicentric investigation concerning intestinal atresia diagnosed in french university hospitals from 1979 to 1983, has been done. Out of 96 exploitable cases coming from 8 centers, 28 correspond to intestinal atresias which had subject to prenatal echographic diagnosis: that is 29%. 15 of the 96 atresias were affected with trisomy. 21 which had not been recognised in utero. Concerning the isolated duodenal atresia, not accompanied by any other malformation, the post surgical evolution has been appreciated during the 12 months following the surgical operation. There was no statistically significant difference, in favour of the group of children whose malformation has been recognised in utero, concerning the number of post surgical complications. This result, apparently deceitful, can be explained by the early management of these malformations in specialised centers which have participated in this study.

[3 case reports of lumbosacral agenesis; role of the lumbo-iliac bone graft]. / A propos de 3 observations d'agénésie lombosacrée, place de la greffe lombo-iliaque.

From 3 cases (8,2 and 16 years) the two first patients have been followed since birth, and in reviewing the 124 cases published in the literature, the authors analyze: the possible causes with in first the mother's diabetes, the possibility of antenatal diagnosis with ultrasound, the clinical symptoms: paraplegia with stiff joints and deformities of lower limbs, finally the therapeutic possibilities to correct the deformities of lower limbs and find the compromise between straighten the patient to authorize the possible standing and keep mobility between trunk and pelvis to allow sitting. In this case, the lumbo-iliac bone graft can be used if necessary and if the mobility of the hips make that possible. This bone graft was realized once in January 1979 (follow up 5 1/2 years). All the references are in Dr Barnetche's thesis (Bordeaux 1984, n degrees 135).

[Prepuce plastic surgery, known as the Lille operation, in distal hypospadias. Apropos of 138 cases]. / La plastie du prépuce, dite lilloise, dans les hypospades distaux. A propos de 138 cas.

Distal hypospadias with meatus situated on the glans, in the balano-penile furrow, even on the distal part of the shaft, are the most frequent hypospadias. Without chordee, it's only a cosmetic handicap. The aim of surgery is to produce the esthetic appearance of a normal penis. In France, circumcision is not usual and most people prefer a child with prepuce. The best technique in this case is to keep the meatus in its hypospadial situation and get the prepuce circular with a ventral suture. It's an easy technique. The hospital stay is short, 2 or 3 days, without any diversion of the urine. This operation is made when the child is about 3 years. Saint-Aubert described this procedure in 1967. The authors present their technique and results. Since 1971, 138 boys underwent this operation with few complications: 2% of fistulas and 2% of phimosis on the last hundred of cases.

[Priapism in the young adolescent (author's transl)]. / Priapisme du grand enfant.

Priapism is exceptional before puberty and a new case is here related. Facing the failure of the medical treatment, a caverno-saphenous shunt is realized at the 72e hour. In connection to this case, we present a review of etiological and pathological knowledges applying to this anomaly of erection. An early surgical treatment is obviously necessary to prevent fibrosis, ground for later impotence. Likewise, we must insist on the usually temporary practice of this accident which may lead to discuss about the suppression of the shunt in some secondary deficiency of erection.

[Traumatic dislocations of the hip in children under 6. Apropos of 10 cases]. / Luxations traumatiques de la hanche de l'enfant de moins de 6 ans: A propos de 10 observations.

In a group of 11 traumatic dislocations of the hip, treated between 1962 and 1979, the authors find and study 10 cases of pure dislocation in often mild traumatism, about children between 4 and 5 and half yeard old. They remembre the classic tests: the most frequent hit by the boy, frequency of the posterior dislocation, the relative geod pronostic of the hurt in the growth, at this age. In front of theses particular facts of happening, they look for predisposing bone-factors by anamnestic investigations, a coxometric study of the hip, and for 5 children, a tomodensitometric investigation. They cannot have a definitive conclusion, but the results seem to show an association of predisposing articular defects which they go to the dislocation. That would be explain the great frequency of pure dislocations and the rarety of the cephalic necrosis.