[IMesenteric and mesocolic cystic lymphangiomas. Diagnostic and therapeutic management]. / Les lymphangiomes kystiques du mésentère et du méso-côlon. Prise en charge diagnostique et thérapeutique.

STUDY AIM: Study of clinical, diagnostic and therapeutic aspects of mesenteric and mesocolic cystic lymphangiomas. MATERIAL AND METHODS: 15 cases were retrospectively analysed: 5 adults (mean age 36.8 years, range 26 to 46) and 10 children (mean age 23 months, range 0 to 5 years). Diagnosis was prenatal in 1 case. Symptoms were: abdominal pain (80%), fever (20%), abdominal mass (46%), occlusive syndrome (33%), chylous ascitis 1 case. Tumours were mesenteric (86%) or mesocolic (13%). RESULTS: Complete resection was performed in 11 cases (including 10 bowel resections), incomplete resections in 3 and doxycycline sclerotherapy once. Mean follow-up is 5 years. One recurrence occurred 6 years after complete resection and 1 tumour increased after incomplete resection. Patient treated by sclerotherapy was non symptomatic with a 3.5 years follow-up after last injection. CONCLUSION: Mesenteric and mesocolic cystic lymphangiomas are congenital benign tumours. Complete resection should be performed whenever possible. Intracystic sclerotherapy with doxycyclin is possible for unresectable lymphangiomas.

Carcinoid tumors of the appendix in children.

We report ten cases of carcinoid tumor of the appendix observed in children from 1988 to 1996. The patients included six females and four males with an average age of 13 years at presentation. They were admitted after complaining of pain in the lower abdominal quadrant. In eight children who presented with symptoms of acute appendicitis, the tumor was located at the tip of the appendix. Diagnosis was performed after appendicectomy (AE) and pathologic examination, which revealed a tumor slightly under 1 cm in size. Two other children were admitted with clinical signs of peritonitis due to larger tumors measuring more than 2 cm on the base of the appendix. One patient underwent a cecectomy, the other a right hemicolectomy. For all patients follow-up was 3 years, and all recovered fully. According to these findings and a review of the literature, we suggest conservative surgical procedures in children. More than 70% of these tumors are localized at the tip of the appendix and represent an incidental pathologic finding during AE; AE alone is curative. Patients with a bulky tumor of the appendicular base measuring 2 cm and invading the serosa and mesoappendix without metastases may be treated with a cecectomy; ileocecal resection may be indicated in cases where the tumor has infiltrated tissue beyond the cecum with localized metastases and in patients with incomplete gross resection. Right hemicolectomy is questionable in this age group and restricted to rare conditions.

Does nephroblastomatosis influence the natural history and relapse rate in Wilms' tumour? A single centre experience over 11 years.

The presence of multifocal or diffuse nephrogenic rests (NRs) in one or both kidneys is termed nephroblastomatosis (Nbm). Nbm may be a predisposing factor for Wilms' tumour (WT). The aim of this retrospective study was to evaluate the impact of Nbm on the outcome of WT in children. We assessed the outcome of 81 children with Wilms tumours and practical implications of Nbm in the treatment and follow-up. All the pathology slides have been reviewed in 1997. 63 had WT without Nbm (group A) and 18 had WT associated with Nbm (group B). There was no statistical difference between the two groups according to the age at diagnosis and histology. Clinical abnormalities were more frequent in group B (33 versus 8%). There was no statistical difference between the percentage of stage IV in both groups, but bilaterality (stage V) was present only in the group B. Relapse was observed in 20/81 patients (25%): 11 (17%) in group A and 9 (50%) in group B. Mean delay of relapse was longer (25 months) in group B than in group A (10 months). For the whole population, with a median follow-up of 9 years, the event-free survival (EFS) and the overall survival (OS) probabilities were respectively 74%+/-10 and 83%+/-9 at 120 months. The difference in EFS between groups A (82+/-9%) and B (38%+/-29) was significant (P=0.004). The discovery of Nbm in the non-tumoral part of the kidney with WT can be an adverse factor and in particular favours the subsequent development of a new Wilms tumour. It justifies separate follow-up guidelines.

Is the appendix graft suitable for routine biliary surgery in children?

BACKGROUND/PURPOSE: The appendix graft (AG) is used widely for urinary tract replacement in children. Biliary tract replacement is less common. The purpose of this retrospective multicentric study was to evaluate the safety of appendix grafting for biliary reconstruction. METHODS: The files of 33 patients treated at 7 European pediatric centers were reviewed. Indications included choledochal cyst (CC) in 5 cases, biliary trauma (BT) in 1, and biliary atresia (BA) in 27. In CC and BT patients, the graft was inserted isoperistaltically between the proximal biliary duct and second duodenum. In all but one of the BA patients, the graft was placed antiperistaltically by patching its cecal end onto the porta hepatis. RESULTS: Postoperatively, all CC and BT patients initially became asymptomatic but developed laboratory evidence of anicteric cholestasis within 1 year. The most common manifestation was increased gamma-glutamyl-transpeptidase level (GGT), whereas histologic findings showed liver damage (mainly fibrosis). Reoperation has been carried out in 4 CC and 1 BT patients within a mean period of 19 months after appendix grafting. The graft procedure was converted to hepaticojejunostomy (HJ) in 4 and to choledocoduodenostomy in 1. Surgical exploration showed kinking in 1 patient and stenosis in 1. In the remaining 3 cases, there was no discernible cause of cholestasis, and appendix histology findings were normal. In all 5 reoperated patients, liver function findings returned to normal within 1 month. Reoperation is scheduled for the remaining CC patient who currently requires ursodesoxycholic medication to maintain normal liver function and presents histologic evidence of "de novo" sclerosing cholangitis. Results of appendix grafting also were poor in the 27 BA patients. Procedure-related perioperative complications occurred in 4 (15%) including 1 early death from graft necrosis. Another early death resulted from intestinal hemorrhage. Jaundice cleared in only 8 (28%). CONCLUSIONS: The findings of this study suggest that the AG is unsuitable for routine biliary repair in children. It should be used only as a salvage technique when conventional HJ repair is contraindicated. Because of the high risk of graft dysfunction, we recommend screening tests to detect biochemical or histologic cholestasis in any patient previously treated with appendix grafting.

Bilateral cystic pleuropulmonary blastoma in early infancy.

We report 2 cases of multifocal cystic (type 1) pleuropulmonary blastoma, diagnosed during the first 6 months of life. This rare entity must be recognized before evolution into the prognostically unfavorable type 2 or type 3 pleuropulmonary blastoma.

[Prenatal diagnosis of congenital diaphragmatic hernia: evaluation of the prognosis]. / Diagnostic prénatal de la hernie des coupoles diaphragmatiques: évaluation du pronostic.

OBJECTIVE: The aim of this study was to evaluate prognostic prenatal factors of congenital diaphragmatic hernia. MATERIAL AND METHODS: We designed a retrospective study of 34 patients with congenital diaphragmatic hernia. The infants were delivered at the Edouard Herriot Hospital between September 1, 1994 and June 30, 1998. We excluded cases of pregnancy termination. After eliminating 4 cases, we studied 30. The factors studied were: polyhydramnios, transverse abdominal diameter, hepatic and umbilical vein deviation, mediastinal deviation, intrathoracic location of the stomach. Prenatal karyotype and echocardiography were systematically obtained. RESULTS: Total perinatal mortality was 53% and was 48% in case of unique diaphragmatic hernia. Prognosis was poorer if the diaphragmatic hernia was associated with another malformation (perinatal mortality: 80%, 4/5 cases), or in case of preterm delivery (83%, 5/6 cases). Factors associated with poor prognosis were: polyhydramnios, transverse abdominal diameter below the 5(th) percentile and major hepatic deviation. Diagnosis before 25 weeks was not associated with poor prognosis. Intrathoracic stomach was a good diagnostic sign, but did not allow an assessment of prognosis. CONCLUSION: Diagnosis of congenital diaphragmatic hernia was made before 25 weeks in 77% of the cases. It was not a factor of poor prognosis. As other authors, we found that prenatal association with another malformation (especially cardiac malformation) polyhydramnios, deviation of the liver, and abdominal transverse diameter below the 5(th) percentile were factors of poor prognosis. But it was difficult to determine the prenatal prognosis. Improvement is needed.

Surgical treatment of diaphragmatic agenesis by transposition of a muscle flap: report on 15 cases.

Between 1987 and 1996 the transposition of a muscle flap using the anterolateral abdominal wall (the internal oblique and transversus abdominus muscle) has been used for the surgical treatment of fifteen cases of diaphragmatic agenesis. There are several advantages of this technique: a more accurate reconstruction of the diaphragmatic dome, a better adapted muscle flap, good tolerance and evolution as a result of the use of autologous material with thus less risk of recurrence and/or infection. The disadvantages are represented by the nine cases of abdominal herniation at the site of the muscle flap, three of which necessitated secondary surgical treatment. Nine children survived this treatment, six died following severe cardio-pulmonary complications associated with this malformation.

Abdominal expansion using a polytetrafluoroethylene prosthesis in the treatment of Pepper syndrome.

The authors report three cases of stage IV-S neuroblastoma in infants aged 4, 6, and 8 weeks, who despite chemo- and radiotherapy required surgical intervention to urgently relieve major thoracoabdominal compression secondary to massive hepatomegaly. The results were successful, with abdominal expansion being achieved by the introduction of a polytetrafluoroethylene prosthesis, which was removed during the 2nd, 3rd and 7th postoperative month, respectively, after tumor regression. Two children were in complete remission 32 and 38 months later, the 3rd died after 16 months of tumor progression.

Isolated deficient alpha6beta4 integrin expression in the gut associated with intractable diarrhea.

BACKGROUND: An infant born with pyloric atresia had development of intractable diarrhea and was found to have total epithelial detachment of gastric and small and large bowel mucosa. She had no skin abnormalities. Parental consanguinity and pyloric atresia in a sibling who died without autopsy suggest an inherited origin for this disorder. The purpose of this study was to examine defects in intestinal and skin cell adhesion. METHODS: Histologic, immunohistochemical, and ultrastructural characteristics of the skin and gut of the patient were compared with that of normal control subjects. Distribution of adhesion molecules was determined. RESULTS: Immunofluorescent analysis of the digestive mucosa showed alpha6beta4 integrin expression deficiency at the epithelial cell-lamina propria junction. Ultrastructural examination of the digestive mucosa revealed a complete epithelial detachment with a cleavage plane lying between the lamina densa and the basal pole of the enterocytes. Consistent with the absence of skin blistering, integrin alpha6beta4 was expressed at the dermal-epidermal junction. Electron micrographs of skin biopsy specimens showed the presence of normal hemidesmosomes and the absence of dermal-epidermal dysadhesion. CONCLUSION: It was postulated that this patient had protracted diarrhea related to epithelial detachment of the digestive mucosa as a consequence of a deficiency of an integrin alpha6beta4 isoform specific to the gut.

Cystic adenomatoid malformation of the lung: neonatal management of 21 cases.

Currently, congenital cystic adenomatoid malformation of the lung (CCAM) is often diagnosed antenatally by ultrasound, allowing prompt and appropriate medical and surgical management after birth. The authors report 21 cases of CCAM admitted from 1988 to 1997 to a neonatal intensive care unit and treated by high-frequency oscillation (HFO) and early surgery. Six infants developed respiratory distress, of whom 4 required ventilation by HFO. HFO was also the mode of ventilation used in all cases except 1 during the perioperative period. There was no death from respiratory failure. The authors emphasize the usefulness of antenatal diagnosis, the efficiency of HFO in cases with severe respiratory failure, and well-tolerated early surgery.

Case report: a preterm infant with an extradural myxopapillary ependymoma component of a teratoma and high levels of alpha-fetoprotein.

The sacrococcygeal region may be the origin of germinal tumors, of paragangliomas, and, rarely, of extradural myxopapillary ependymomas (MPE) in the newborn and child. A case is presented of a preterm child with an abdominal tumor, originating from the precoccygeal area, that turned out to be a teratoma with a component of an MPE. The high levels of alpha-fetoprotein in this preterm baby were initially misleadingly interpreted as a tumoral marker. The differential diagnosis and the difficulties in interpreting tumoral markers in infants are discussed.

Gastric necrosis in newborns: a report of 11 cases.

Eleven neonates ranging in gestational age from 34 to 40 weeks presented with gastric necrosis. The 4 full-term neonates showed sudden-onset hemorrage and "coffee-ground" vomiting; in the 7 premature babies the initial clinical finding was abdominal distention. The criteria for diagnosis were: perinatal distress in prematures and transient neonatal respiratory distress in full-term babies. Radiographic evidence of gastric distention was typical and preceded clinical signs of hematemesis and gastric perforation. Surgery was performed in 8 patients; 3 received medical treatment. At surgery 1 total and 3 subtotal gastrectomies and 4 segmental gastric resections were performed. Three of these patients died post-operatively as a consequence of multiorgan failure; a second look was necessary in one patient 1 week after surgery because of prepyloric perforation due to ulcers. Biopsy specimens taken from the site of perforation demonstrated extensive necrosis; ulceration was disseminated in the surrounding gastric mucosa; no signs of phlogosis were detected. The diagnosis, treatment, and physiopathologic considerations are reviewed.

Rhabdoid tumor of the kidney associated with a tumor of the posterior fossa.

We report a case of a malignant rhabdoid tumor of the kidney (MRTK) associated with a cerebellar tumor. Diagnosis was confirmed before neoadjuvant chemotherapy by a percutaneous fine-needle biopsy of the abdominal tumor. The clinical and radiologic features of this rare association of childhood neoplasms are reviewed.

[Pheochromocytoma: pediatric features]. / Le phéochromocytome: particularités pédiatriques.

Pheochromocytoma is a rare tumor in children which explains, together with its miscellaneous symptomatology, why the diagnosis may be delayed. The localization of the tumor(s) rests mainly on MIBG scintigraphy, and CT scan and/or magnetic resonance imaging. Thanks to a systematic preoperative treatment of hypertension and major progress in anesthesia, the operative mortality of pheochromocytomas in children is nowadays very low. Nevertheless two major problems remain: 1) the difficulty of diagnosing and treating the malignant forms, 2) the high frequency of recurrences, sometimes many years after the removal of the primary tumor. A regular long term supervision is therefore necessary after the surgical treatment.

One-stage tracheal reconstruction of congenital tracheal stenosis.

Congenital tracheal stenosis (CTS) is a condition difficult to manage and associated with a high mortality rate. The principles of one-stage laryngotracheoplasty have been adapted to the surgical management of CTS. Three children underwent a one-stage anterior tracheoplasty with costal cartilage grafting and without cardiopulmonary bypass. Extubation was performed within 13 days. One child developed granulation tissue one month postoperatively, and this was removed endoscopically. The three children were free of respiratory obstruction signs during follow-up ranging from 8 months to 3 years. Postoperative endoscopies have shown growth of the grafted trachea.

Preoperative stabilization using high-frequency oscillatory ventilation in the management of congenital diaphragmatic hernia.

OBJECTIVES: a) To assess the efficiency of preoperative stabilization with the use of high-frequency oscillatory ventilation in the treatment of congenital diaphragmatic hernia; b) to determine early prognosis factors. DESIGN: Prospective, consecutive patient study. SETTING: A tertiary neonatal intensive care unit in a university hospital. PATIENTS: All patients admitted to the neonatal intensive care unit with a diagnosis of congenital diaphragmatic hernia between April 1990 and June 1993 (n = 18). INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Eleven infants had an antenatal diagnosis. Ventilatory settings, blood gas values, arterial-alveolar oxygen ratio, and oxygenation index were recorded on admission and every 3 hrs thereafter until surgery. Surgery was performed if the FIO2 was < 0.3 and mean airway pressure was approximately 9 cm H2O, while the infants were ventilated with high-frequency oscillation. Mean duration of high-frequency oscillatory ventilation was 57 +/- 52 hrs before surgery and 60 +/- 104 hrs after surgery. Overall survival rate was 72%. Infants were divided into two groups, according to the time of surgery. Group 1 (n = 12) patients were operated on in the first 48 hrs of life; on admission, all group 1 patients had an arterial-alveolar oxygen ratio of > or = 0.3 and an oxygenation index of < or = 10, and all recovered. Group 2 (n = 6) consisted of patients for whom preoperative stabilization was difficult to achieve. One infant died before surgery. Four other infants had congenital malformations and subsequently died. Only one infant survived. In this group, the arterial-alveolar oxygen ratio and oxygenation index on admission were 0.08 +/- 0.05 and 33.2 +/- 14.6, respectively (p < .01 vs. group 1). CONCLUSIONS: a) This study demonstrated the efficiency of preoperative stabilization using high-frequency oscillation in the treatment of congenital diaphragmatic hernia. b) An arterial-alveolar oxygen ratio of > or = 0.3 and an oxygenation index of < or = 10 on admission are associated with a rapidly completed surgical procedure and a good outcome.

Epignathus: clinical and sonographic observations of two cases.

The epignathus or mature pluritissued teratoma of the mid-line of the palate is a benign tumor for which the neonatal prognosis is related to the obstruction of the superior airway. This paper concerns observations of two cases, which developed in different ways. Echography enables diagnosis of this type of malformation which classically combines a heterogeneous polylobed mass, polyhydramnios and the absence of other morphological anomalies. It enables an accurate differential diagnosis to be made by eliminating the other masses of the cephalic region. Echographic diagnosis also allows a rational management decision to be made, i. e. termination of pregnancy if prognosis is poor, or continuation with multidisciplinary (neonatologist, obstetrician, surgeon) intervention at birth.

[Current aspects of cystic lymphangioma in the neck]. / Aspects actuels du lymphangiome kystique cervical.

Cystic hygroma of the neck (CHN) is the result of a defect in the embryonary development of the lymphatic system. In many cases foetal ultrasound allows an early diagnosis before birth, the prognosis being very different according to the gestational age at the time of detection. When discovered before 30 weeks of gestation it is almost always associated with chromosomal abnormality and/or polymalformation leading to spontaneous or therapeutic abortion. On the contrary when appearing after the 30th week of gestation, CHN is usually an isolated malformation as when discovered during infancy or childhood. Approximately 2/3 of these "late" CHN are present at birth; 90% are discovered before 2 years of age. A spontaneous regression occurs in about 15% of the cases. In 70% of cases the CHN is simple without extension to the oropharynx or mediastinum and its complete surgical resection is usually easy. Extension to the oropharynx is present in about 20% of the cases; there is a risk of neonatal respiratory distress and the treatment is difficult. Extension to the mediastinum is found in about 10% of the cases; respiratory distress is rare and a large surgical resection is necessary. Surgery is the primary treatment of CHN after a careful evaluation of the extension of the tumor by ultrasound, scanography or nuclear magnetic resonance, and oropharyngeal endoscopy. It allows a "macroscopically complete" resection in about 80% of the cases, but a recurrence is observed in approximately one every five cases. Following partial resection or important recurrence, treatment includes according to the cases; new attempt of surgical resection, sclerosing therapy, and laser therapy for the oropharyngeal forms.