[Pseudo-"isolated" intestinal perforation in a very low birth weight infant: exceptional presentation of Hirschsprung's disease]. / Perforation intestinale du grand prématuré en apparence "isolée": mode de révélation exceptionnel de la maladie de Hirschsprung.

Intestinal perforations in preterm newborn are characterized with high morbidity and mortality rates. They often are associated with necrotizing enterocolitis and seldom correspond to idiopathic spontaneous intestinal perforation. Perforations upstream of an intestinal organic obstruction (atresia), or of a functionnal obstruction (meconium-ileus, Hirschsprung disease) have been considered to be rare in preterm newborns. We report a case of caecal perforation with a pneumoperitoneum which occurred at 5 days of life, in a 28-week gestational age infant, that was treated by cecostomy. There were no signs of necrotizing enterocolitis. At 43 days of life, the preoperative contrast enema study revealed a left colon transition zone, suggesting a Hirschsprung disease, which was confirmed by rectal biopsies. Transanal pull-through was performed. There were no postoperative complication. This case demonstrates that an isolated intestinal perforation in very preterm newborn can reveal a Hirschsprung disease, especially if it occurs in the caecum.

Successful use of recombinant factor VIIa for severe surgical liver bleeding in a 5 month-old baby.

A 5 month-old baby developed non-ceasing intra-peritoneal bleeding after extensive surgical biopsy for an hepatoblastoma. A single recombinant activated factor VII injection following enlarged hepatectomy helped to resolve quickly this life-threatening haemorrhagic syndrome.

[Surgical aspects of intussusception due to lymphoma in children]. / Aspects chirurgicaux des invaginations intestinales sur lymphome chez l'enfant.

BACKGROUND: Intussusception due to lymphoma is a challenging condition for pediatric surgeons. The aim of this study is to report seven cases of this entity and to discuss its management. CASE REPORT: Six boys and one girl, 3-15-years-old, were admitted for intussusception secondary to a lymphoma. All patients underwent laparotomy: biopsy of massive abdominal tumor 6 and 8 weeks following resection of an intussusception (two cases), ileal resection of non-reductible intussusception (one case), right hemicolectomy for tumor of the appendix (one case), tumorectomy of localized ileal tumor (two cases), enlarged mesenteric lymph node biopsy associated with simple reduction of intussusception (one case). All children were successfully treated with protocol chemotherapy with a 15-month to 13-year follow-up. No relapse was observed. CONCLUSION: Surgeons should be aware of operative sights of ileal lymphomas. Diagnosis of lymphoma may be difficult after manual reduction of intussusception. A sample of any abnormality (mesenteric lymph node, peritoneal fluid) should be taken. Intestinal resection allows to reduce the intensity of chemotherapy but must be as limited as possible: ileal resection in cases of complicated intussusception, tumorectomy "in sano" in cases of ileal parietal isolated tumor. Reduction of intussusception alone (with no resection of ileal tumor) seems to be effective if diagnosis of lymphoma is possible from peripheral samples (peritoneal fluid, pleural effusion, mesenteric lymph node, bone marrow biopsy...).

Anal canal duplication in infants and children--a series of 6 cases.

The authors present a series of six anal canal duplications (ACD), duplications of the alimentary tract located along the posterior side of the anal canal, with a perineal opening just behind the anus. Five asymptomatic duplications were diagnosed before the age of one year, by simple perineal inspection. A twelve-year-old girl presented with perineal and anal pains and diarrhoea. Fistulography revealed a tubular structure in five cases and a cystic structure in one case, behind the normal anal canal, in one case communicating with it. A presacral sacrococcygeal teratoma was found in two children and in one case it was visualised by preoperative US in an infant with a lumbosacral myelomeningocele. Surgical excision was performed by a perineal approach in 5 cases, by a combined sacral and perineal approach in the last case, because of the associated teratoma. Non-invasive preoperative investigations, consisting of a pelvic X-ray, US examination, barium enema and fistulography, are sufficient in most cases; MRI is reserved for special indications. Surgical treatment restores a normal perineal aspect, without sequelae, and avoids complications like those described in other types of digestive duplications: infection, ulceration, bleeding, malignant changes during later adult life. Associated anomalies are frequently described in the literature, especially presacral tumours (16%) and anorectal malformations (21%); they can influence the management, the surgical approach and the functional prognosis.

[First-intention surgical treatment of thyroglossal duct cysts in children: apropos of 99 cases]. / Traitement chirurgical de première intention des kystes du tractus thyréoglosse chez l'enfant: à propos de 99 cas.

INTRODUCTION: Thyroglossal duct cyst (TGDcs) is the most common malformation of the neck. The risk of infection and malignant transformation impose its treatment. OBJECTIVES: The purpose of our study were: 1) to specify some points about the symptomatology and preoperative evaluation necessary for TGDcs diagnosis; 2) to analyse the factors who can explain an unsuccessful surgical treatment. PATIENTS AND METHODS: Our study is based on a retrospective review of cases and a review of the literature. From 1981 to 2000, 99 children with a mean age of five years were treated for a TGDcs with a surgical procedure in the Grenoble University Hospital. 3 excision and 96 Sistrunck's procedure were performed. In all cases a histological study was made. RESULTS: Ultrasonography was the most frequent preoperative evaluation. We have had 7 complications: 3 haematoma, 2 abscess and 2 desunited scar. 6 children have had recurrent disease. Among these 6 children, 3 have had an excision and 3 a Sistrunck's procedure. No case of malignant transformation was reported. CONCLUSION: Ultrasonography is the first preoperative evaluation to obtain before surgical treatment of a TGDcs. Sistrunck's procedure is the best surgical treatment with a value of recurrence from 1% to 10%. Areas of surgical failure included breaking of cyst during the dissection, inadequate hyoïd bone resection, an anatomical variation with many ductuli in the base of tongue, inadequate muscles of tongue resection.

[An unusual care of intestinal invagination: jejunojejunal invagination]. / Un cas rare d'invagination intestinale: l'invagination jéjunojéjunale.

BACKGROUND: Jejunal intussusception is uncommon in comparison with ileocolic form. It is more frequent in children over 2 years of age and has an atypical subacute presentation. An underlying anatomical cause is usually found. CASE REPORT: A 14-year-old boy was admitted for abdominal pain with bilious vomiting. The physical examination was normal, with only the ultrasonography showing an intussusception in the left hypochondrium. At laparotomy the diagnosis of jejunal intussusception was made; its reduction was impossible. A resection and end to end anastomosis was performed. The anatomopathology examination found a polyp in ectopic gastric mucosa. CONCLUSION: Jejunal intussusception must be better understood as its diagnosis could be made too late. Surgical exploration is the treatment of choice because of the usual underlying anatomical cause.

Pheochromocytoma and paraganglioma in children: a report of 24 cases of the French Society of Pediatric Oncology.

Pheochromocytoma and paraganglioma of childhood are rare neuroendocrine tumors. Urinary catecholamine measurements, metaiobenzylguanidine (MIBG) scanning, computed tomographic scanning, and magnetic resonance imaging have greatly facilitated diagnosis. Prognosis after surgical resection is excellent. In this retrospective series collected from French oncology centers, the risk of tumor progression was studied in order to assess prognostic factors and the optimal diagnostic and therapeutic management. Medical records of 24 children with paraganglioma were reviewed. This tumor occurred at a median age of 12.5 years and in most cases was revealed by arterial hypertension. The diagnosis was made by the demonstration of urinary excretion of catecholamines and their metabolites. Six patients had bilateral adrenal pheochromocytomas; two patients had extra-adrenal paragangliomas. In eight patients, the paraganglioma occurred as a familial disease. Surgical excision was the only therapeutic procedure. With a follow-up of 5.2 years, 14 of the patients are still in first complete remission and 6 have developed metastases or shown tumor progression. Despite a high long-term survival rate, the risk of malignancy and of multifocal involvement is of concern and is associated with a significant rate of late events. The outcome depends on adequacy of tumor resection and must be serially assessed.

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.

Malignant peripheral nerve sheath tumor arising in a "de novo" ganglioneuroma: a case report and review of the literature.

A case of a "de novo" ganglioneuroma showing a large area of malignant peripheral nerve sheath tumor (MPNST) is described. The tumor arose in an 11.5-year-old girl with neither stigmata nor family history of von Recklinghausen's neurofibromatosis. In addition, the patient had no previous history of a neuroblastoma or radiation therapy. This report provides new evidence that, although rare, the spontaneous development of an MPNST in a benign ganglioneuroma can occur. Immunohistochemical and electron microscopy studies supported the finding that the spindle cell component was of nerve sheath origin.

Feasibility of retroperitoneal pelvic lymph node exploration in cervical carcinoma. Assessment of morbidity in 33 cases treated by combined radio-surgery or definitive radiotherapy.

BACKGROUND AND PURPOSE: Laparoscopic modalities by selective biopsies permit a better assessment of the pelvic lymph nodes status than imaging procedures in cervix carcinoma. They could enable the radiation oncologist to adapt the target volume of external irradiation, provided the feasibility of such procedures is good and the toxicity reduced as much as possible. MATERIAL AND METHODS: From June 1980 to May 1993, 52 women with a mean age of 49, underwent a retroperitoneal pelvic lymph node laparoscopic procedure for cervix carcinoma classified according to FIGO as stages IA2 (14), IB (12), IIA (6), IIB (14), IIIB (3) and IVA (3). Two techniques were used: retroperitoneoscopy (RPS) in 16 cases, and a panoramic retroperitoneal pelviscopy (PRPP) in 36 cases. RESULTS: Intra-operative and post-surgical morbidity were equivalent in the two procedures. Among the 33 patients who had external irradiation, one Grade 3 urinary late morbidity (3%) due to an overtreatment was observed; no Grade 3/4 morbidity of the gastro-intestinal tract, no lymphoedema of the lower extremities, no parietal tumor cells implantation were noticed. CONCLUSIONS: These procedures can be used safely to better know the prognosis and to define the pelvic lymph node planning target volume and its radiation management with accuracy.

[Granulosa cell tumors of the ovary in children and adolescents. Multicenter retrospective study in 40 patients aged 7 months to 22 years]. / Tumeurs de la granulosa de l'ovaire chez l'enfant et l'adolescente. Etude multicentrique rétrospective de 40 patientes âgées de 7 mois à 22 ans.

BACKGROUND: Juvenile granulosa cell tumors (JGCT) of the ovary are rare in children. The over-all outcome after surgery is relatively good, but the indication and type of complementary treatment for severe forms are still unclear. POPULATION: A retrospective survey of the majority of patients with JGCT of the ovary admitted between 1965 and 1990 to 11 French oncologic pediatric centers was carried out. Medical records including surgical and histological data, were analyzed and each tumor was retrospectively classified by the same pathologist according to the Wollner classification. RESULTS: There were 40 patients aged 7 months to 22 years (mean: 6 years); 28 were less than 10 year old at diagnosis. Three had enchondromatosis (Ollier's disease). At diagnosis, all patients presented with an abdominal tumor, 23 had developed manifestations of precocious pseudopuberty, 2 had signs of virilization after a normal puberty and 2 had secondary amenorrhea. Surgery was always the primary treatment: unilateral ovariectomy in 35 cases, bilateral in 4 and biopsy alone in 1 case. There were 21 stage I, 1 stage II, 16 stage III and 2 stage IV cases. 13 patients received combined chemotherapy and 2 abdominal radiotherapy. 34 patients were alive and disease-free 10 months to 26 years after surgery and 6 died. All 23 patients with precocious pseudopuberty had a favorable outcome. CONCLUSIONS: This study confirms earlier reports. Unilateral ovariectomy is the first-choice therapy. There is no evidence that tumors complicated by rupture and hemoperitoneum require chemotherapy. Combined chemotherapy does not appear to improve the prognosis for the rare malignant forms. The factors of good prognosis are age less than 10 years and the presence of precocious pseudopuberty.

[Mediastinoscopy]. / La médiastinoscopie.

Mediastinoscopy was initiated by E. Carlens in 1959 and enabled the exploration of the mediastinal tissues, above all the lymphatics with both a diagnostic and a prognostic aim. Surgical intervention was performed most often under general anaesthesia and cervical mediastinoscopy was carried out under direct visual inspection thanks to a thoracoscope with its own light source so that the mediastinal axis, the thymic area and also the peripheral mediastinum could be examined. The examination can be completed or even replaced by antero-lateral mediastinoscopy. Biopsies with forceps are in general of good quality. Side effects or complications are rare (1-3.8%) and the mortality is practically nil (0-0.5%). Diagnostic mediastinoscopy is indicated in cases of isolated or multiple mediastinal adenopathy, mediastinal tumours in particular those of the thymus whose extent is not evident. And finally certain miscellaneous pulmonary conditions. The sensitivity is 95-99% in sarcoidosis or metastatic lymphadenopathy and a little less in malignant lymphomas or thymic tumours. Mediastinoscopy is used to assess prognosis as regards the extension of broncho-pulmonary cancer. The existence and the site of metastatic nodes can be precisely assessed possibly on the contralateral side too, and influences the therapeutic orientation. The rare failures of mediastinoscopy leave a remaining 5% for an exploratory thoracotomy. Computered tomography does not seem to replace mediastinoscopy but rather guides its application. It is also part of the assessment of malignant lymphomas in certain situations.

[Parameningeal cervical rhabdomyosarcoma in the neonatal period]. / Rhabdomyosarcome cervival paraméningé en période néonatale.

A case of parameningeal cervical rhabdomyosarcoma with severe bone destruction is reported in a 3 month-old infant; symptoms were present at birth. The treatment consisted of exclusive intensive chemotherapy. The outcome was favourable with complete tumor regression and vertebral bone reconstruction. The child was on complete remission without sequellae two years later.

[Iron salt poisoning complicated by gastric stenosis]. / Intoxication par le perchlorure de fer compliquée d'une sténose gastrique.

Iron salt poisoning has been reported in a 7-year-old child. Desferioxamine was used for 6 days; systemic toxicity remained moderate. The clinical course was marked by gastro-intestinal symptoms and gastric stricture. The treatment principles for iron salt poisoning are reviewed.

Single-stage correction of posterior hypospadias (178 cases). Comparison of three techniques: free skin graft, free bladder mucosal graft, transverse pedicle preputial graft.

Three techniques have been developed for single-stage repair of posterior hypospadias: free skin grafts, free bladder mucosal grafts, and pedicle preputial grafts. This multicenter retrospective study of 178 children who underwent surgery for posterior hypospadias was designed to compare the results achieved with these procedures. Free skin grafts (15 cases) resulted in the most frequent complications, and in particular the most severe strictures; in our opinion this technique should be abandoned. Pedicle preputial grafts (133 cases) gave the greatest number of successes from the outset, and should be preferred whenever the dimensions of the prepuce are sufficient for urethroplasty. Results with bladder mucosal grafts (30 cases) were not as good as with preputial grafts, but this technique remains the only solution when the meatus is in a very posterior position, and for children who have already undergone multiple operations.

[A rare case of bladder exstrophy with a completely malformed complex in a girl]. / Un cas inhabituel d'extrophie vésicale avec ensemble malformatif complexe chez une fille.

Report of the observation of a female newborn presenting a complex malformative set: distal anorectal malformation without fistula; bladder exstrophy without epispadias, with cutaneous bridge; congenital urethrovaginal fistula; malformative sexual ambiguity with bulky genital tubercle, whole urethra, urethral meatus on the apex of the genital tubercle, associated with a mid labial genital joint and a high genital orifice; unilateral multicystic kidney; vertebral abnormalities with supernumerary thoracolumbar vertebrae; no karyotype abnormalities and no familial pathological history; no endocrine abnormalities. The therapeutic multistage program permitted a good reconstruction of the different lesion, particularly with bladder functioning witch in hope a good continence with two years of follow-up. No similar case is reviewed in the literature.

[Prenatal diagnostic imaging of abdominal cysts. Report of 9 cases]. / Images kystiques abdominales de diagnostic anténatal. A propos de 9 cas.

The authors report on 9 cases of cystic lesion detected by fetal ultrasound echography, uropathies and bowel dilatations having been excluded. The lesions were detected at a fairly advanced stage of pregnancy, during the 3rd trimester and involved 8 girls and 1 boy. Investigation of possible chromosomal and associated anomalies was carried out in each case. Analysis confirmed the frequency of ovarian cysts in the female subjects (6), but also demonstrated the following: 1 case of bowel duplication, 1 cystic Meckel diverticulum, 1 liver biliary cyst (boy). The etiology and diagnostic difficulties and the approach to be adopted are noted. The authors emphasize the frequent signs of complications, especially as regards ovarian cysts. A thorough clinical examination and echographic detection and confirmation of the lesions is indicated followed by early surgical intervention if the cyst persists; so that, particularly in the case of ovarian cysts, the organ in question is preserved to the maximum.

[Left pulmonary artery sling associated with right pulmonary hypoplasia and ventricular septal defect]. / Artère pulmonaire gauche aberrante associée à une hypoplasie pulmonaire droite et á une communication interventriculaire.

The pulmonary artery sling is a congenital malformation where the left pulmonary artery forms a vascular sling that bends around the right border of the lower trachea. Its clinical manifestations are signs of tracheal compression in the first months of life. The diagnosis of this rare anomaly is suspected on radiological and echocardiographic grounds and confirmed by oesophagography, tracheoscopy and angiocardiography. Diagnostic problems may be encountered when the condition is associated with tracheobronchial (50% of the cases) or cardiovascular malformations. In the case reported here respiratory symptoms and heart failure were present in a 1-month hypotrophic infant who also had ventricular septal defect and dextrocardia due to right lung hypoplasia. The pulmonary artery sling was diagnosed by angiography. The severity of the clinical signs precluded all attempts at surgical repair of the cardiovascular anomalies. The child died at the age of 2 1/2 months.

[Solid tumors of the adrenal gland in children (excluding neuroblastomas). A study of a series of 18 cases]. / Les tumeurs solides de la surrénale chez l'enfant (neuroblastomes exclus). Etude d'une série de 18 cas.

This is a survey of the French South-East Group of Research in Pediatric Surgery (GRECPSE) concerning 18 Solid Adrenal Tumors, observed during a 20-years period (1969-1989) in 11 boys and 8 girls. 13 are tumors of the adrenal cortex (9 adenomas and 4 carcinomas). Clinical virilization is found in 7, cushing's syndrome in 3; while feminizing tumor, Conn's syndrome and non hormone-secreting form are quite exceptional (1 case each, respectively). 5 are adrenal pheochromocytomas (4 benign tumors and 1 malignant with local invasion). Ultrasonography, CT Scan and MIBG Scintigraphy are modern procedures which permit localization of the small and non palpable tumors with high accuracy. They make easier surgical management. Differentiation between benign and malignant tumors of the adrenal glands still remains the main problem. The course of the disease is surely the only distinguishing parameter. However weight tumor and selective histopathological characteristics are useful in predicting the prognosis and behavior of such tumors = Mitotic activity for adrenocortical tumors, extensive areas of necrosis and small cells for pheochromocytomas have the highest discriminating value.