Ischemic necrosis of bile ducts complicating Schönlein-Henoch purpura.

Gastrointestinal complications of Schönlein-Henoch purpura are frequent and sometimes severe. However, there seem to be no reports of liver involvement. A child is described in whom Schönlein-Henoch purpura was complicated by bile duct lesions, resulting in biliary cirrhosis and requiring liver transplantation. At surgical removal, the liver had lesions of bile ducts and of adjacent small blood vessels in the hilum, very similar to those complicating hepatic artery thrombosis after liver transplantation. These findings suggest that Schönlein-Henoch purpura can be complicated by vasculitis of the peribiliary vessels resulting in ischemic necrosis of the bile ducts. Schönlein-Henoch purpura can be added to the list of causes of ischemic cholangiopathies.

[Neonatal rhinopharyngeal obstruction due to craniopharyngioma]. / Obstruction rhinopharyngée néonatale par craniopharyngiome.

BACKGROUND: Craniopharyngioma, the most common tumor of the sellar and suprasellar regions in childhood, can exceptionally develop in the infrasellar area. CASE REPORT: A premature girl (GA: 30 weeks) weighing 1240 g required intubation and ventilation because she suffered from a neonatal respiratory distress syndrome. Nasal endoscopy showed total obstruction of rhinopharynx by a tumor resembling adenoid tissue. This tumor was successfully excised and its histological examination showed a craniopharyngioma. CONCLUSION: A wide range of intranasal tumors has to be examined, endoscopy and CT scan are necessary for a precise localization and to eliminate meningocele.

[Cancers in children in the Auvergne area: retrospective study from 1986 to 1991]. / Les cancers de l'enfant dans la région Auvergne: étude rétrospective de 1986 à 1991*.

BACKGROUND: The specificity of childhood cancers led to the creation of regional childhood cancer registries. An epidemiological study of childhood cancers in the Auvergne area was carried out over a 6 year-period (1986-1991) in order to create a registry. POPULATION AND METHODS: The population of our study was 252,820 children (0-15 years old), living in the Auvergne region. All malignant neoplasms were included together with brain tumours (whatever grading). Data were collected from medical and administrative sources. RESULTS: The data of 153 cases were collected during this period. World age standardized overall incidence rate was 120.5 cases/milion/year. Age standardized incidence rates were: leukemias 37.6 (ALL 28.01), central nervous system tumours 18.34 (medulloblastomas 4.6, astrocytomas 4.6, ependymomas 3.8), lymphomas 10.0, neuroblastomas 18.6, soft tissue tumors 8.3, bone tumours 6.1 (Ewing's sarcomas 4.1, osteosarcomas 2.0), nephroblastomas 5.5, retinoblastomas 3.1, liver tumours 0.5 and others 0.5. CONCLUSIONS: Our data base in the Auvergne area might be a source of information for epidemiological studies on the role of etiological factors, the survival, the sequelae and the incidence trends.

[Abdominal cystic lymphangiomas in children. Apropos of 6 cases]. / Lymphangiomes kystiques abdominaux chez l'enfant. A propos de six observations.

Cystic lymphangiomas are rarely observed in the abdomen, even in a paediatric surgery ward. From 1977 to 1991, we diagnosed and treated six such lesions in our paediatric surgery department in Clermont-Ferrand. Most of the lymphangiomas were seen in boys. Mesenteric localizations were seen in 4 out of 6 cases with the characteristic chylous contents in 3 cases. There were very many different presenting signs, although two acute episodes of intestinal vuvulus occurred. The clinical features and the usefulness of exploratory techniques are discussed. The frequency of abdominal cystic lymphangiomas is probable underestimated although the number of cases diagnosed will undoubtedly rise with routine echography, especially in the ante-natal period. Surgical exeresis is the standard therapy but other alternative or complementary methods such as sclerosis techniques could be of interest.

[Rapid efficacy of preoperative chemotherapy in nephroblastoma involving cardiac cavities]. / Efficacité rapide de la chimiothérapie préopératoire sur un néphroblastome envahissant les cavités cardiaques.

BACKGROUND: Tumors involving the inferior vena cava and cardiac cavities are rare in nephroblastoma. When they do occur, the standard treatment is primary surgery; but this is often technically difficult. CASE REPORT: A 3 year-7-month-old girl was admitted because of an abdominal mass and signs of heart failure. Ultrasonography showed that this mass involved the right renal vein and the inferior vena cava including the right atrium. A CT scan showed no metastases. Echocardiography showed that the mass occupied two thirds of the right atrium and had blocked the tricuspid valve. The patient was given heparin (2 mg/kg/day), vincristine (1.5 mg/m2 on day 1) and actinomycin D (15 micrograms/kg on day 1 to 3). The heart failure disappeared and the abdominal mass decreased in volume. This improvement was confirmed by successive ultrasonographies. Chemotherapy was then completed and the patient was operated on six weeks later: right nephrectomy and removal of the cavoatrial tumoral thrombus. Pathological examination confirmed the nephroblastoma and the patient was given radiotherapy and chemotherapy for 28 weeks. She was also given aspirin for 17 weeks. Pulmonary metastases were detected 1 year after onset and were treated by a combination of surgery and chemotherapy. The patient is well 20 months after the onset of the disease. CONCLUSION: Preoperative chemotherapy seems to be effective in nephroblastoma extending to cardiac cavities; it allows subsequent surgery and facilitates postoperative radiotherapy.

Malignant pleural tumor in a child mimicking a mesothelioma.

Malignant tumors of pleural origin are rare in childhood and difficult to diagnose. We report a case in a white 11 1/2-year-old girl who presented with a bulky tumour in the basal left lung field. There was an area of osteolysis at the ventral extremity of the 9th left rib allowing excision of the tumor by lateral thoracotomy. Macroscopically the operation was satisfactory. Despite two complementary excisions, chemotherapy with Vin-Caepi and consolidation chemotherapy followed by bone-marrow autograft, left pleural metastases appeared two years later. A second operation, a new course of chemotherapy with stage IV SIOP MMT, followed by a second bone-marrow graft and mediastinal radio-therapy, resulted in remission. At the time of this report, after three and a half years of evolution, the child's condition is satisfactory and she leads a normal life without treatment. A probable diagnosis of pleural mesothelioma was arrived at by elimination. This kind of tumour, without specific markers, is difficult to distinguish from small-round-cell tumors. Evolution is unpredictable and often rapidly fatal. Although these tumors generally have a weak response to chemo- and radiotherapy, we think that close follow-up and active therapy, rapidly initiated at the slightest sign, can improve the quality of life of the patients and increase their survival.

[Neuroblastoma in the Auvergne region from 1986 to 1991: epidemiological data]. / Le neuroblastome dans la région Auvergne de 1986 à 1991: données épidémiologiques.

An epidemiological study of neuroblastoma in the Auvergne area of France (departments: Puy-de-Dôme, Allier, Cantal, Haute-Loire) was carried out over a 6 years period (1986-1991). Nineteen cases of neuroblastoma were registered during this period. The annual incidence rate is 1.2 among 100,000 children under the age of 15 years. Similar incidence rates from other studies have been reported in the literature. In the department of Rhône, France, a screening program for neuroblastoma was initiated in 1990. The Auvergne area may constitute a population-based control group.

[Mucopolysaccharidesis Type VII resulting from beta-glucuronidase deficiency. Report of one family]. / Mucopolysaccharidose Type VII par deficit en beta-glucuronidase: etude d'une famille.

The patient is a north african female, fourth born child in a family with consanguinity. Facial dysmorphia, clubfeet, swollen extremities and heel borne ponctuate calcifications are observed soon after birth. beta glucuronidase activity is very low in serum, leukocytes and skin fibroblasts. At 18 months, gorwth and psychomotor development are normal. Flat facies and dorsolumbar cyphosis are striking. There is no clinical sign of storage disease, neither ocular or cytologic (blood, bone-marrow) abnormalities. Squeletal abnormalities are predominant on cervical and lumbar column and pelvis. Urinary excretion of chondroitin 4 sulfate and chondroitin 6 sulfate is increased. A 4 year old sister is affected. Facial dysmorphia, mild squeletal abnormalities are observed. Again, growth is normal and there is no symptom of storage disease. Enzymic expression of parent heterozygotism is marked in serum studies, but less marked in leukocytes and fibroblasts. The last two children are heterozygotes. At the time of a 5th pregnancy, enzymic activity studies of amniotic fluid and amniotic cells have shown that the foetus was an inaffected female. This child was normal at birth.