[Bilateral nephroblastoma. Strategy and prognosis apropos of 12 cases].
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Biomedical subjects
Publications and source records attributed to Y Revillon.
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In order to reduce the respiratory morbidity of thoracotomy in esophageal surgery, several methods have been used, such as blunt dissection and endoscopic dissection through mediastinoscopy. It seems that the latter reduces drastically the morbidity but does not allow full visualization of the esophageal wall, a disadvantage in some circumstances. We describe a thoracoscopic dissection of the esophagus which gives a large and magnified view of the pleural cavity, of the mediastinum, and of the esophagus.
We evaluated 131 patients (6 months-14 years) who experienced 21 deaths before listing, 11 continuing on the waiting list, 38 well on home parenteral nutrition, 6 off parenteral nutrition and 59 transplanted (20 girls) aged 2.5 to 15 years, (18 >7 years). They received cadaveric isolated intestine (ITx, n = 31) or liver-small bowel (LITx, n = 32), including right colon (n = 43; 23 LITx) for short bowel (n = 19), enteropathy (n = 20), Hirschsprung (n = 14), or pseudo-obstruction (n = 6). Treatment included tacrolimus, steroids, azathioprine, or interleukin-2 blockers. After 6 months to 10.5 years, the patient and graft survivals were 75% and 54%. Sixteen patients (10 LITx) died within 3 months from surgery (n = 3), bacterial (n = 5) or fungal (n = 6) sepsis, or posttransplant lymphoproliferative disorder (n = 2). Rejection occurred in 27 patients, including 10 steroid-resistant episodes requiring antilymphoglobulins. The grafts were removed due to uncontrolled rejection in seven ITx recipients. Surgical complications were observed in 38 recipients (25 LSBTx) within 2 months, including bacterial (n = 22) or fungal (n = 11) sepsis, cytomegalovirus disease (n=12), adenovirus (n = 11), or posttransplant lymphoproliferative disorder (n = 12). Forty-two children (19 LSBTx) are alive. Weaning from parenteral nutrition was achieved after 42 days (median). Factors related to death or graft loss were pre-Tx surgery (P < .01), pseudo-obstruction (P < .01), age over 7 years (P < .03), fungal sepsis (P < .03), steroid resistant rejection (P < .05), hospitalized versus home patient (P < .01), and retransplantation (P < .05). Colon transplant did not affect the outcome. Interleukin-2 blockers improved isolated ITx (P < .05). Early referral and close monitoring of intestinal failure and related disorders are mandatory to achieve successful ITx.
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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.
OBJECTIVES: Liver transplantation with living related donor has been recently developed to compensate for the insufficient number of liver grafts for children. The major problem is ethical because it implies voluntary mutilation of a healthy person. This paper report results in 37 living related donors. PATIENTS: Recipients were followed in Enfants-Malades Hospital. Investigations and donor surgery were performed at the Digestive Surgery Unit of Beaujon Hospital. RESULTS: One donor was re-operated for bleeding, and another one a biliary fistula treated with percutaneous drainage for one week. The post-operative course was uneventful in the other donors, with a follow-up of between 2 and 50 months. Thirty-three children are alive (90%), one of them underwent a second transplant for arterial thrombosis. Vascular and infectious complications, and the number of rejection episodes were the same as in transplantations with a deceased donor. Biliary complications were frequent (15 patients out of 37) and significantly increased morbidity. A teenage boy who received a small graft (0.9% of his weight) presented initially with hepatic insufficiency without encephalopathy. CONCLUSION: This technique has been shown to have a good balance between benefits and risks. Our experience confirms this, especially in very young children. Each case should be discussed individually and parental consent should be obtained without external pressure. Experience with this technique should be continued and at the same time the use of cadaveric grafts should be optimized.
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This retrospective study analyzes the management of 83 infants who had undergone extensive small bowel resection as newborns between 1970 and 1987. Resection was performed for atresia (n = 34), volvulus (n = 21), laparoschisis (n = 10), vascular enteropathy (n = 10) and other disorders in 8 cases. The patients were divided into two groups: Group I (33 children) has less than 40 cm and Group II (50 children) 40-80 cm of residual small bowel respectively. Survival depended on the length of residual small bowel (Group I: 63.6%, Group II: 92%) and on their date of birth (born before 1980: 65%, after 1980: 95%). The time required for acquisition of intestinal autonomy depended on the intestinal length (average time, 29.5 months for Group I and 14 months for Group II) and especially on the presence of the ileocecal valve. The residual ileal and/or colon length also influenced adaptation. Artificial parenteral and/or enteral nutrition ensured normal height/weight increases. Home parenteral nutrition allowed children to be returned to their families during intestinal adaptation.
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Seventeen children with extended resection of the small intestine, chronic intestinal adynamia or Crohn's disease were given prolonged parenteral nutrition through an arteriovenous fistula, exclusively of associated with enteral nutrition. This new technique allowed the patients to lead a normal life, with a normal growth. In more than 22 patient-years no infectious complication occurred. Thrombosis of the fistula remains the most important complication; il can be prevented thanks to a controlled infusion technique, including adjunction of platelet aggregation inhibiting drugs and the maintenance of proper hemodynamics.
From 1957 to 1978, 211 intestinal obstructions were treated at Hôpital des Enfants malades, Paris: 103 coming from our own surgery, 108 from other surgical centers. 24,6% cases are observed in children less than one year old, after neo-natal surgery. 41% are observed between 5 and 9 years of age, at the "appendicitis period". 61% obstructions follow an appendicectomy. 74% of obstructions happen in the first year after surgery, of which 32% in the first month (the early mechanical intestinal obstruction between 8 days and 7 years after the first obstruction: 72% in the first year, of which 30,5% within the first month. Some precisions are given about clinical symptoms, site and intestinal lesions. The study of the preventive surgical procedures (Noble's, or Child-Philip's technics) are not included in this paper.
A 20 month-old girl with Thalassemia major presented with a pseudotumoral mesenteric lymphadenitis due to Yersinia enterocolitica. The authors stress the rarity of this type of Yersinia infection. The clinical and bacteriological diagnosis is difficult and is based on the early isolation and identification of the bacterial strain, thus allowing an efficient antibiotic treatment. The frequency of such a Yersinia infection in thalassemic children is further discussed.
The only curative treatment for enzymatic deficiency such as Ornithine transcarbamylase (OTC) deficiency is liver transplantation. We have studied hepatocyte transplantation as alternative therapy in spfash mouse, the animal mode of OTC deficiency. Hepatocytes were obtained from C57BL/6J strain mice (normal OTC activity). About 2 x 10(6) hepatocytes in 0.1 ml were injected in the spleen of recipient mouse. We then studied: ammonemia, urinary orotate, spleen OTC activity, liver OTC activity, histological presence of hepatocytes in the spleen, immunohistochemical OTC staining in liver and spleen hepatocytes. In syngeneic hepatocyte transplantation (C57 > C57) functional transplanted hepatocytes were found in the spleen over 5 months after transplantation. In allogeneic transplantation (C57 > spfash) without immunosuppression, hepatocytes were rejected in 3 days. With cyclosporine immunosuppression, hepatocyte survived 12 days and showed OTC activity. A few transplanted hepatocytes with OTC activity migrated from spleen of liver. Metabolic disorder were not corrected. This technically easy method (compared to whole liver transplantation) demonstrated 2 problems: reject in allogeneic transplantation and the small amount of injected enzyme.
Two boys (two years and a half old) were admitted in this hospital for an abdominal tumor. In both cases a pelvic abcess was present, secondary to a pelvic appendicitis. Symptoms have been deeply modified by previous antibiotherapy. Radiological examinations including opacification of the urinary tract by a intravenous pyelogram and opacification of the recto-sigmoid by a baryum enema allowed in both cases to localize the mass and to lead to the diagnosis with the clinical data.