[Stenosis of the upper renal pelvis: rare complication of an incomplete ureteral duplicity. Embryological investigation]. / La sténose du pyélon supérieur : complication rare d'une bifidité urétérale. Enquête embryologique.

A stenosis of the upper pole of an incomplete renal duplication is presented. The prenatal diagnosis of a right renal ureteropyelic junction syndrome, isolated, with a normal amniotic liquid was confirmed at birth. Intravenous pyelogram 8 days after birth showed three right dilated calical groups with a dilated renal ureteropyelic junction, but an normal inferior calical group suspected a renal bifidity. Renal MagIII scintigraphy evaluated the anatomical and functional stenosis and indicated surgery. Postoperative followings were simple and results good 3 years after. From this rare case, embryogenesis is discussed.

[Congenital prenatal hemangioma: diagnosis and treatment]. / Hémangiome congénital à révélation anténatale.

An echogenic tumor (22 x 22 mm) isolated from the anterior part of the left leg at 27 WA of pregnancy suggested an angiomatous structure. This mass appeared to be well vascularized on Doppler examination. Three-dimensional imaging showed connections with adjacent tissues. The limbs were mobile. Delivery of a 3990-g-boy occurred normally. On the anterior part of the left leg, there was a plate-shaped mass with a pink hole at the superior part. Excision under general anesthesia was performed at the first month because of the risk of rupture and bleeding. The dissection was easy with the deep plane. Healing was complete 10 days after the surgical procedure and the aesthetic aspect was good 1 year later. Histological findings of the suspect mass emphasize the vascular aspect of the tumor. A hemangioma capillary with venous structures in the dermal hypodermic area was found.

Anatomical and sonographical studies on the development of fecal continence and sphincter development in human fetuses.

The aim of this study was to specify the sonographic, anatomical and morphological aspects of the fetal anal sphincter and to compare them with pathological and physiological findings. The sphincter was examined by serial sectioning and staining of embryo and fetal tissue and by real-time ultrasound. Its function was analysed using amniotic fluid digestive enzyme assays in cases of anorectal atresia and cystic fibrosis. Morphological findings indicate that the functional components of the anal sphincter do not differentiate before 30 weeks and therefore do not account for the observed anal continence at 22 weeks. Ultrasound measurements of the sphincter indicate three developmental phases: 1) slow growth from 14 to 19 weeks; 2) rapid growth from 19 to 30 weeks; 3) subsequently, no further increase, but contractions indicative of peristaltism. Amniotic fluid digestive enzyme assays indicate that anal sphincter maturation begins with perforation of the anal membrane at 12 weeks. Comparison of pathological cases (anorectal atresia and cystic fibrosis) suggests two possible explanations of fetal anal obstruction: increasing viscosity of digestive secretion or the presence of the three anal sphincter muscles, even if still immature. Our results clarify the evacuation and retention of meconium during fetal life and the role of the terminal part of the digestive tract, notably the anal sphincter. Prenatal diagnosis of anorectal atresia is therefore possible before 20 weeks of gestation by measurement of amniotic fluid digestive enzymes and ultrasonography, thus enabling better neonatal management.

[Role of laparoscopic surgery in the treatment of abdominal pain in children]. / Miesto laparoskopickej chirurgie v liecbe bolestí brucha u detí.

Principal indications of the coelioscopy in pediatric surgery is the laparoscopic appendectomy. In compare with classic techniques it has several advantages but as well as some limitations. During decision about eventual laparoscopic intervention in children is important to take into the consideration personal and material facilities of the unit or hospital for pediatric coelioscopic interventions, their accessibility as well as longer operating time and higher price of the operation. It is amoral to use the laparoscopic appendectomy as a method of teaching surgeon and team to the technique. The authors consider the laparoscopic exploration of the peritoneal cavity with subsequent appendectomy to be a very suitable technique for examination and treatment of pre-pubertal and pubertal girls or young women with chronic or repeated abdominal pain of the uncertain origin, however, its indication in indubitable acute appendicitis of a boy is questionable.

Principal modifications of the Duhamel procedure in the treatment of Hirschsprung's disease. Analysis based on results of an international retrospective study of 2,430 patients.

In the literature, many articles refer to the DUHAMEL technique or some of its modifications in the treatment of Hirschsprung's disease, however, no complete review of all of them has been published to date. The authors present an analysis of the principal modifications of this technique based on the results of an international retrospective survey that evaluated a series of 2,430 patients as well as data from the literature. The important role of the internal anal sphincter in the physiology of continence is emphasized; understanding its function is an imperative prerequisite for a successful Duhamel procedure. The original technique, which used two crushing Kocher clamps as proposed by Duhamel in 1956, as well as a majority of the modifications with various spur-crushing instruments are no longer in use. At present, surgeons generally use staplers for the colorectal anastomosis.

Duhamel operation 40 years after: a multicentric study.

The authors present the results of an international retrospective study evaluating the present situation in using Duhamel's technique or its modifications in the treatment of Hirschsprung's disease on the basis a series of 2430 patients. After evaluation of all important criteria and discussion with data from the literature it is concluded that the techniques is simple, fast, less shocking, logical, reliable, respecting the rectal anatomy and physiology, ingenious in its principle, without serious complications. The rectum is preserved in place with all its vascular drainage and innervation. This technique can be performed by every sufficiently experienced surgeon.

Incarcerated umbilical hernia in children.

Over a period of 5 years, four cases of incarcerated (one strangulated) umbilical hernia (UH) in children were observed and treated in Saint-Denis, France. In a review of the literature, only 45 descriptions of complicated UHs in children were found worldwide. Incarceration of UHs is considered to be very rare in children, however, it appears to occur more frequently than it is generally believed. Therefore, a more active therapeutic approach is recommended even in smaller hernias, from more than an aesthetic point of view.

[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.

Arterial vascularisation of the extrahepatic biliary tract.

The aim of this study was to establish a plan of the arterial distribution to the main supraduodenal biliary tract in order to draw practical conclusions for biliary surgery, especially in bilio-alimentary and bilio-biliary anastomoses and liver transplantation. It was based on a study of 60 fresh subjects and was carried out using four different methods: dissection after injection of colored latex into the superior mesenteric a. (10 subjects), radiography and dissection after injection of Micropaque into the celiac trunk (10), dissection after injection of Indian ink into the common hepatic a. (10) and radiography and dissection after selective injection of the right hepatic a. or the gastroduodenal a. (30). Analysis of the results leads to definition of three types of vascularisation and two territories, superior and inferior, overlapping at the level of the mouth of the cystic duct. The practical implications for biliary surgery and liver transplantation are discussed.

[Appendicitis in children]. / L'appendicite de l'enfant.

In 1992, appendicitis is still common among children. Mortality due to a belated or erroneous diagnosis is far from being nil. The disease must always be present in the clinician's mind, and its diagnosis is purely clinical. No significant advance has been made on this subject, and clinical findings remain of paramount importance. Hospitalization for observation at the slightest doubt and emergency surgery when the diagnosis is probable are the best means of improving the management of appendicitis in children.

Fetal development of the urethral sphincter.

A morphological study was carried out on 25 human embryos (3-60 mm CR) and 20 human fetuses (15-40 weeks). The anatomical analysis was completed by immunocytochemical studies of different markers of muscle differentiation (vimentin, desmin, titin and isoforms of the myosin heavy chains). A mesenchymal condensation forms around the urethra after the division of the cloaca (E.H. 12-15 mm CR). The m. pubo-rectalis appears in 20-30 mm CR embryos, following the opening of the anal membrane. Striated muscle fibers can be clearly differentiated at 15 weeks. At this time, the smooth muscle layer also becomes thicker at the level of the bladder neck and forms the inner part of the urethral musculature. The urethral sphincter is a functional unit composed of central smooth muscle fibers and peripheral striated muscle fibers. In the human fetus, this musculature mainly develops in the anterior wall of the urethra. We analysed the expression of smooth and skeletal markers as an original approach to the muscular development in this region.

[Ano-rectal sphincter myectomy in children. Indications and results]. / La sphinctéromyectomie ano-rectale chez l'enfant. Indications et résultats.

Ano-rectal myectomy, described by B. Duhamel in 1965, is the logical therapy of rectoanal achalasia; an experience of 117 cases is reported. Clinical examination and radiological investigations have been made prior to surgery. This surgical procedure is simple, without complication. Three kinds of rectoanal achalasia are described: achalasia with structural change of the internal sphincter (fibrosis, sclerosis); achalasia with a functional sphincteric disorder (with normal histology); achalasia with intrinsic innervation abnormality. Excellent results have been observed in the two first groups but more variable successful results in the third group which concerns short segments of Hirschsprung's disease.

[Complications of the ritual circumcision]. / Complications de la circoncision rituelle.

Over the last 8 years, 23 cases of complications following ritual circumcision in home surroundings have been admitted for treatment. Patients were seen either as emergencies for immediate complications, sometimes serious, requiring recovery surgery or at a later stage for sequelae of variable severity.

[Teratoma strictly of the buttocks in a 13-year-old girl]. / Tératome purement fessier chez une fille de 13 ans.

A 13-year old girl was admitted to our hospital because of an ulcerated gluteal tumor. This tumor was present since birth. A diagnostic procedure was performed. This tumor was strictly limited to the gluteal region, without pelvic extension, but with sacral malformation. Total surgical excision was completed. Pathology report revealed a histologically benign teratoma. The origin of the tumor is discussed.

Surgical treatment of malignant esophagotracheal fistulas.

Malignant esophagotracheal fistulas represent the fatal evolution of esophageal cancer. Since October 1974, 18 patients were seen who had confirmed esophagotracheal fistula, and in half of these patients, the fistula was the revealing symptom. Upper gastrointestinal studies (using hydrosoluble dye) and bronchoscopic examination confirmed the diagnosis, which was already strongly suspected in 16 of the patients due to the specific nature of the cough triggered by swallowing. The average age of the patients was 57 years, (range, 32 to 78 years). In 12 patients, the nutritional repercussions were moderate, and in only 6 patients, were the bronchopulmonary consequences serious. Therapeutically, we adopted a palliative surgical attitude since the physical and psychological condition of these patients was considered fatal the month after discovery of the fistula. In addition, the results of intubation were uncertain and transitory. Our surgical methods evolved in three stages: first, we performed bipolar exclusion of the esophagus coupled with gastrostomy (four patients), second, we performed retrosternal coloplasty (five patients), and third retrosternal gastroplasty with drainage of the inferior portion of the esophagus by Roux-Y jejunal anastomosis (six patients). The latter technique was inspired from that described by Kirschner in 1920. It is moderately aggressive and has the advantage of dealing with several objectives: exclusion of the fistula, reestablishment of normal alimentation, drainage of the inferior portion of the esophagus, and permitting postoperative radiotherapy. In our series, when only the patients who underwent coloplasty or gastroplasty were considered, 3 of 11 died, and the longest postoperative survival times were 16 and 23 months. Regarding gastroplasty alone, our results were in agreement with those in the literature. In the 19 patients who underwent gastroplasty, the operative mortality was 31.5 percent and the average length of survival was 9 months. Six patients survived from 8 to 26 months. Therefore, this "last resort" surgery seems legitimate in light of the fact that the results are sometimes superior to those of radical curative surgery for extirpative esophageal cancer.

[Therapeutic approach to bilateral hepatoblastoma]. / Approche thérapeutique d'un hépatoblastome bilatéral.

The treatment of a case of bilateral hepatoblastoma by continuous infusion of adriamycin in the hepatic artery accompanied by ligature of the right hepatic artery enabled the authors to carry out six weeks later an extensive right lobe hepatectomy. Unfortunately the patient died seven weeks after the partial hepatectomy from complication of surgery. Thus it was possible to demonstrate the effectiveness of the intra arterial chemotherapy. No residual tumor could be found at autopsy. As in the case with the adult, arterial ligature combined with carefully targeted chemotherapy would seem to be potentially important therapeutic option in the treatment of malignant liver tumors in children.

[Value of neuroleptoanalgesia in preventing recurrence of fetal shunts during treatment of diaphragmatic hernias]. / Intérêt de la neuroleptanalgésie dans la prévention du retour aux shunts foetaux lors de la cure des hernies diaphragmatiques.

A leptanalgesia (Chlorpromazine-morphine) was used in the treatment of postero lateral diaphragmatic hernia in six neonates. The treatment which was begun immediately after the arrival of the child and continued during and after surgery seemed to contribute to the favorable outcome observed in five of the babies. In spite of the empirical nature of their argument, the authors suggest the possible mechanism of action of the treatment. However they recognize that its effectiveness can only be assessed after careful hemodynamic studies.

[Epidermoid cyst of the spleen in children. Apropos of 2 cases]. / Kyste épidermoïde de la rate chez l'enfant. A propos de deux observations.

Two cases of epidermoid cyst of the spleen are reported. A splenectomy was necessary because the topography of the lesions was not consistent with a conservative procedure for this children fourteen and ten years old. The histological criteria and pathogenic hypotheses of this infrequent lesion are analysed. Ultrasonography and eventually computerized tomography bring the best findings concerning the topography and are useful for the surgical treatment. When it is possible, the partial conservation of the spleen is to be wished.