[Superior mesenteric artery syndrome: a cause of vomiting in children. Report of 3 cases]. / Syndrome de l'artère mésentérique supérieure: une cause à évoquer devant des vomissements isolés chez l'enfant. A propos de 3 observations.

Duodenal obstruction by Superior Mesenteric Artery (SMA) is a misdiagnosed vomiting syndrome in children. Several factors are involved, including rapid weight loss, rapid statural growth without weight augmentation. Diagnosis is suspected when an improvement is achieved by ventral decubitus and it is confirmed by plain films of the abdomen, GI study with barium and echography, measuring the aortomesenteric angle (inferior to 25-30 degrees ). Patients must at first be treated conservatively. Surgery is indicated for occlusive episodes with unsuccessful conservative therapy. The authors report 3 cases with different clinical presentation. However, all the patients presented important weight loss and vomiting.

[Intestinal volvulus after enterocystoplasty: report of three cases in children]. / Volvulus intestinal après entérocystoplastie: à propos de trois cas chez l'enfant.

Enterocystoplasty complications related to the presence of intestinal mucous presence in the bladder are well known and well reported in the literature. Mechanical problems in the intestinal tract due to using gut for bladder augmentation are not as well recognised. We analyzed three cases of children treated by ileocystoplasty who presented an intestinal volvulus in two cases and a cecal volvulus in one case around the vascular pedicle of the intestinal patch used for bladder augmentation.

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.

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.

[Intravesical morphine analgesia in pediatric urology]. / Analgésie par morphine intravésicale en Urologie Pédiatrique.

OBJECTIVE: Evaluation of the technique and value of intravesical morphine instillation after ureterovesical reimplantation in children. MATERIAL AND METHODS: From July 1997 to June 1998, the Duckett protocol (Philadelphia, USA), comprising slow intravesical infusion of morphine for the first three prosoperative days, was applied in 25 children aged five months to twelve years after ureterovesical reimplantation surgery, without bladder catheterization. Postoperative pain was evaluated by CHEOPS and VAS scales and by all nursing staff by an anonymous questionnaire. RESULTS: Analgesic assessment tests confirmed a significant improvement of postoperative comfort, despite the absence of a bladder catheter. No adverse effects or complications related to intravesical morphine instillation were observed. CONCLUSION: Intravesical morphine instillation is an easy, effective postoperative analgesia technique, allowing good tolerance of the absence of postoperative bladder catheterization.

Idiopathic varicocele in children and adolescents--which therapeutic choice?

61 children were studied and treated between January 1986 and September 1993 for idiopathic varicocele. The aim of our study was to evaluate the advantages and disadvantages of the different techniques and to show the progression to a greater efficacy. The mean age at the time of therapy was 14 years, ranging from 7 to 16 years. All children presenting with pain or testicular asymmetry were treated. Four asymptomatic children were followed for 2 years before treatment. 36 children were treated by surgical ligature via the inguinal approach; 8 with a resection of the varicose veins as far as the tunica vaginalis. 14 children were treated by percutaneous sclerotherapy including 1 patient following unsuccessful classical surgical treatment. 12 children were treated by surgical inguinal ligature associated with peroperative phlebography and thrombosis. 56 children were reviewed postoperatively over a period which varied from 2 months to 4 years (5 lost to follow-up). For the 36 classical ligatures: 25 good results, but 9 hydroceles (5 out 8 varicose resections): 70% good results. 7 failures and 4 lost to follow-up. For the 14 percutaneous sclerotherapy: 4 technical failures (impossibility to catheterize the spermatic vein): 10 good results. For the 12 ligatures with peroperative phlebography and thrombosis: 11 good results and 1 lost to follow-up. All the above procedures were carried out at our out-patient clinic. The therapeutic choice will therefore have to take into consideration a procedure which produces the lowest morbidity rate and proves to be the most effective. The association of surgical ligature, phlebography and thrombosis meets these requirements.

[Clinical study of a mivacurium-propofol combination for laparoscopic surgery in children]. / Etude clinique de l'association mivacurium-propofol pour la chirurgie coelioscopique chez l'enfant.

Coelioscopic surgery in children is today in constant progress and requires pharmacological agents which provide excellent surgical conditions for variable and unpredictable durations. The mivacurium-propofol association was clinically studied in this context in 30 ASA I patients aged from 6 to 16 years and appeared safe, efficient and easy to use. The orbicularis oculi and pollicis adductor stimulation allows simple and adapted neuromuscular blockade monitoring. Double-burst stimulation at the ulnar nerve improves the detection of a residual curarization.

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.

The structure of the dart and dart sac of the snail Eremina ehrenbergi, Roth. 1839.

The dart of Eremina ehrenbergi is a small white delicate structure. It consists of funnel-shaped corona with two blades; a short flare and an extended shaft which is surrounded by two lateral vanes. Dart formation was investigated by examining the histological structure of the dart sac of snails before and after copulation. Dart sac is a finger like muscular protrusion. Its internal epithelial lining forms at its blind edge an extended tubercle which gives its lumen a Y-shape. Dart formation begins at the tip of the tubercle in which the epithelial cells secrete a crystallite and homogeneous material which forms the mineralized part of the corona. The subsequent secretory activity of the tubercle epithelium results in an increase in the diameter and anterior lengthening of the corona and the flare. The continued secretory activity of the dart sac epithelium forms the layers of the shaft and the lateral vanes. The fine sculpturing structure of the dart had been investigated by using SEM.

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.

Continent urinary diversion using an artificial urinary sphincter.

We report a new and simplified method of continent urinary diversion employing a modified AMS 800 artificial urinary sphincter (AUS). Our aim in using this artificial valve is to make a stoma continent, while allowing intermittent catheterisation. The AMS 800 pump is replaced by a subcutaneous injection port. This allows, by direct puncture, the accurate setting of the closing pressure by varying the volume of the intra-prosthetic liquid, with subsequent adjustment of this pressure as necessary. The cuff is placed on the subcutaneous part of the intestinal loop diversion. The pressure-regulating balloon is implanted within the area of abdominal pressure, retroperitoneally. After first confirming the efficacy of the system in 3 dogs, the device was placed in 2 patients. The first had a neuropathic bladder treated initially by enterocystoplasty with an appendicocutaneous stoma. Secondary leakage was subsequently controlled by placement of the device, with continuing excellent results at 32 months. The second patient was a girl in whom a urogenital rhabdomyosarcoma had been treated by anterior exenteration, radiotherapy and a sigmoid conduit diversion. This was subsequently converted to a continent reservoir by simple augmentation of the conduit and placement of the device, with a good result being maintained after a follow-up of 20 months. These two cases illustrate the best indications for this procedure, namely primary or secondary leakage from a supposedly continent urinary diversion, and conversion of a freely draining conduit into a continent reservoir. Although long-term results are still pending, our experience thus far encourages us to recommend this technique as a simple means of achieving a continent urinary diversion.

The gonad of the desert snail Eremina ehrenbergi Roth, 1839 (Stylommatophora-Gastropoda) and its role in the production of the male gametes.

Specimens of Eremina ehrenbergi Roth, 1839, were collected in the El-Omayed area of the Sahara Desert, west of Alexandria. This simultaneously hermaphrodite snail has a comparatively small gonad enclosed in a thin membrane. The gonad is composed of a mass of branched tubules, each differentiated to a generative proximal half and a conducting distal half. The primitive germ cells proliferate from germinal cells forming a discontinuous layer in the germinal wall of each tubule; they undergo spermatogenesis or oogenesis at the original site of their proliferation. A cluster of spermatogonia may be derived from one primitive germ cell and it develops round a "Sertoli" cell. The division and differentiation of the cells in each cluster are strictly synchronized. The role of the gonad in formation of the spermatophore is restricted to the production of clusters of mature spermatozoa, which are discharged, without the "Sertoli" cell, via three efferent ductuli connecting the conducting portions of the gonadal tubules with the hermaphrodite duct. The sperm has the same appearance as in vertebrates and can be studied in smears prepared from the gonad or the middle of the hermaphrodite duct, where they are collected and stored for a time.

Achalasia of the esophagus in childhood. Surgical treatment in 35 cases, with special reference to familial cases and glucocorticoid deficiency association.

Achalasia of the esophagus is a relatively rare problem in children, but it may be the cause of severe lung disease, growth retardation and respiratory death in young infants. Surgical esophago-cardio-myotomy remains the treatment of choice, and this article details 25 years of experience with 35 children with achalasia of the esophagus and their late post-operative follow-up. The occurrence of achalasia in the first six months of life, the existence of a familial factor, the prevalent possible association with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem, somewhat different from the adult form, which is considered to be an acquired disease.

Achalasia of the esophagus in childhood: surgical treatment in 35 cases with special reference to familial cases and glucocorticoid deficiency association.

Achalasia of the esophagus is a relatively rare problem in children, but it can result in severe lung disease, growth retardation, or respiratory death in young infants. Surgical esophagocardiomyotomy remains the treatment of choice, and this report details a 25-year experience with 35 children with achalasia of the esophagus and their long-term postoperative follow-up. Occurrence of achalasia in the first 6 months of life, the existence of familial cases, and the association of achalasia with genetic diseases (familial dysautonomia, glucocorticoid insufficiency, Rozycki syndrome) suggest that achalasia in childhood may in certain cases represent a congenital problem.

[Surgical abstention in splenic injuries in children. Under what conditions?]. / L'abstention chirurgicale dans les traumatismes spléniques de l'enfant. A quelles conditions?

The authors report a series of 33 cases of documented splenic lesions which were not operated on. In their opinion non-surgical management is justified, thanks to the progress in ultrasonography and intensive care monitoring. They define out the modalities of this therapeutic choice.

[Surgical indications for hydronephrosis discovered antenatally]. / Les indications opératoires dans les hydronéphroses de découverte anté-natale.

The authors report series of 82 hydronephrosis (in 70 infants), detected antenatally by ultrasound. The cases were classified in 3 groups according to the severity of pelviureteric junction obstruction. Only the more severe cases are operated at once. In the remaining cases the observation help to decide the indications. In this series 35 renal units among 82 have been operated at date. The 47 remaining are annually followed up by ultrasound.

[Conservative treatment with high anastomosis in duplications with lesions of the upper kidney pelvis]. / Traitement conservateur par anastomose haute dans les duplicités avec pathologie du pyélon supérieur.

High anastomoses (ureteropyelic or uretero-ureteral) were performed in 14 patients for either ectopic ureter (7 cases) or uterocele (7 cases). This simple operation (one benign complication) allows conservation of a still functioning upper segment. In cases of ureterocele, aspiration of the latter eradicates it but the high operation does not treat the possibly associated reflux. Certain secondary operations are therefore necessary (2 of the 7 cases). Means for assessing upper segment function (particularly renal scan) are discussed, the presence of function justifying the conservation of this segment even if it is dysgentic and sometimes the site of nodular renal blastema. The respective indications for high anastomosis and total bladder surgery are discussed.

[Undescended testicles: 2-stage reduction or Fowler's technic. Apropos of 2 comparative series]. / Testicules inabaissables: abaissement en deux temps ou technique de Fowler. A propos de deux séries comparatives.

The testicular vessel transection allows a one-stage orchidopexy for high undescended testes. The authors have been using this procedure (as described by Fowler) since january 1984. 29 operations have been performed in this way. This series is compared to another series of 28 staged orchidopexies, a procedure which was used in Rouen until december 1983. The number of cases in each series is not high enough to allow a significant comparison; no conclusion can be drawn as for the long term result concerning the testicular function. However the procedure described by Fowler has the great advantage of being performed in one-stage. But the surgeon must comply with strict rules and the authors point out the difficulties of the operation.