Biomedical subjects
Y Revillon
Publications and source records attributed to Y Revillon.
Thoracoscopic treatment of mediastinal cysts in children.
BACKGROUND/PURPOSE: The development of thoracoscopic surgery has made many procedures possible, including the treatment of mediastinal cysts in children. The authors report their experience with this procedure between 1992 and 1997. METHODS: Surgery was performed on 22 children aged from 1 month to 9 years (median, 27 months), weighing 5 to 49 kg (median, 12.5 kg). Diagnosis was made by antenatal ultrasound scan in six cases (27%), with a chest x-ray performed for respiratory symptoms in 14 cases, and with a chest x-ray performed for positive tuberculin intradermoreaction in two cases. Decision to resect the cyst was determined by thoracoscopy in 21 of the 22 cases, and by open surgery in one case only (subcarinal compressive cyst with left lung distension and a mediastinal shift). RESULTS: Eighteen of the 21 (86%) cases were treated successfully by thoracoscopy. In three cases of bronchogenic cysts, we performed an associated thoracotomy because the dissection was too difficult and dangerous. In three cases, a small part of a common wall between the cyst and the bronchus was not removed. The pathological diagnosis was bronchogenic cysts in 15 cases (71%), pleuropericardiat cysts in three cases (14%), esophageal duplication in two cases (10%), and cystic hygroma in one case (5%). Two postoperative complications were observed: one esophageal wound and a case of recurrent pneumothorax after chest tube removal. Patients were discharged after 2 to 11 days (median, 3 days). Follow-up was uneventful. CONCLUSIONS: Treatment of mediastinal cyst by thoracoscopy is feasible in most cases. Compressive cysts with lung distension and mediastinal shift remain a contraindication. If the cysts have a common wall with the bronchus or esophagus, or if they are subcarinal, the dissection may be difficult and dangerous, and thoracotomy may be preferable.
Small bowel transplantation alone or with the liver in children: changes by using FK506.
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Preliminary experience with combined liver and small bowel transplantation in children.
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[What is new in pediatric pneumology?].
Have been selected for this review: 1) the recent and impressive development of high-resolution and spiral CT scan in pediatric thoracic imaging; 2) the emerging of new and promising therapies for asthma (long acting inhaled beta 2-agonists, leukotriene antagonists, anti-IgE monoclonal antibodies); 3) the multifactorial origin of asthma in childhood; 4) the development of thoracoscopic surgery, a minimal-invasive approach beneficial in numerous circumstances.
Crystalluria: a clinically useful investigation in children with primary hyperoxaluria post-transplantation.
Primary hyperoxaluria type I (PH I) is a congenital error of metabolism that can be manifested by an increased oxalate production, and ultimately result in kidney failure. After a combined liver/kidney transplantation, children with PH I have persistent excretion of oxalate that causes crystal formation in the urinary tract, and could result in systemic oxalosis and eventual graft failure. We speculated that crystalluria may be predictive of this nephrolithogenic tendency and thus investigated the effect of an intensive therapeutic strategy to prevent crystal formation in 13 children at our hospital. Oxalate crystal volume (OCV) measurements were performed at regular intervals for 36 months, and compared with urine supersaturation measurements. We found that crystalluria with the OCV measurement is non-invasive, easily performed, and gives feedback on the efficacy of PH I therapy within one hour. Further study is needed to determine whether this method is a better predictor of nephrocalcinosis than is supersaturation alone.
Combined liver and small bowel transplantation in a child with epithelial dysplasia.
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[Laparoscopic manual intestinal anastomosis: experimental study in a pig model].
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Successful bronchotracheal reconstruction in esophageal bronchus: two case reports.
Esophageal bronchus is the most common congenital bronchopulmonary foregut malformation. Current surgical treatment is resection of anomalous pulmonary tissue, which is often hypoplastic and destroyed by infection. The authors report two cases of bronchotracheal reconstruction. The diagnosis was early, before 15 days of age. The anomalous pulmonary tissue had a pulmonary arterial supply and venous drainage as assessed by angiography and a good functional capacity on selective ventilation. Bronchotracheal anastomosis was successful in both cases: a right main bronchus at 25 days of age and a left main bronchus at 13 days of age. One child underwent reoperation 1 year later for bronchomalacia of the reimplanted bronchus. Both children are well with normal growth 3 and 7 years after surgery. Chest roentgenograms showed normal and symmetrical lung aeration. Tracheal reimplantation may be preferred to pulmonary resection when the anomalous pulmonary tissue is not destroyed. The pulmonary functional capacity is increased and the complications of pneumonectomy avoided.
Intestinal transplantation in pediatric patients: the European experience.
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Small-intestinal transplantation.
The management of patients with intestinal failure has benefited from progress in parenteral nutrition (PN), especially home-based PN. Intestinal transplantation is therefore possible and is now, in some conditions, the logical therapeutic option. Since 1985, more than 180 small-bowel grafts have been done, involving the isolated small bowel with or without the colon (38%), the liver-small bowel (46%) or several organs (16%). Two-thirds of recipients were under 20 years of age, and indications were short-bowel syndrome (64%), severe intractable diarrhoea (13%), abdominal cancer (13%) or chronic intestinal pseudo-obstruction syndrome (8%). Of the patients, 51% survived > 2 years after the graft. Patient and graft survival depends on the type of immunosuppression, i.e. cyclosporine or FK506. The results must be interpreted carefully as they represent the first experience in numerous centres using different immunosuppressive protocols, without any randomization. The results from the largest of these centres reflect the current situation more closely. Functional grafts lead to gastrointestinal autonomy (weaning of PN) while maintaining satisfactory nutritional status and normal growth in childhood. Intestinal transplantation is theoretically indicated for all patients permanently or dependent for a long time on PN. However, as PN is generally well tolerated, even for long periods, each indication for transplantation must be carefully weighed up in terms of the iatrogenic risk and quality of life. When PN has reached its limits, especially in those associated with vascular, infectious, hepatic or metabolic complications, intestinal transplantation must be undertaken. Transplantation of the small bowel alone remains the first option, as combined liver-small bowel grafting is only indicated in the case of life-threatening progressive cirrhogenic liver disease.
Intestinal transplantation.
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[Cystic adenomatoid malformation of the lung revealed in a newborn infant by an image of a lung abscess].
BACKGROUND: Cystic adenomatoid malformation, a rare pulmonary malformation, usually appears as a cystic mass, radiologically. It may be infected and confusion has also arisen in distinguishing it from pneumonia with pneumatoceles. CASE REPORTS: A full-term boy suffered from severe neonatal respiratory distress. Pregnancy had been uneventful despite the fact that his mother had insulin-dependent diabetes. Prenatal ultrasonographies did not reveal any abnormality. On day 2, X-rays showed a right pulmonary mass that appeared solid. The patient was treated for E Coli sepsis. Subsequently, the pulmonary mass became lacent, cystic, fluid-filled, resembling an abscess; the CT scan confirmed these features. As the lesion increased in volume, a limited resection was performed. Histologic examination showed adenomatoid proliferation of bronchiolar elements with formation of cysts and necrosis. CONCLUSION: Infection of cystic adenomatoid malformation may supervene the first days of life resulting in a lung abscess appearance.
Management of oxalosis.
Oxalosis is the final stage of primary hyperoxaluria type I (PHI) when reduction of GFR produces oxalate accumulation. It involves bones, arteries, eyes, heart, nerves, etc. The management of oxalosis starts with prevention of nephrocalcinosis and renal failure by diluting urine and by inhibiting oxalate crystal formation either by increasing the urinary citrate or the urinary pyrophosphate. At endstage renal disease (ESRD) there is no dialysis modality for avoiding the progression of oxalosis. Combined liver/kidney transplantation (LKT) represents the most effective approach. The European PHI transplant registry recently reported 64 LKT with a five-year patient survival of 80% and progressive healing of oxalosis. Four children who received LKT in our unit are reported, all of whom are alive with a follow-up of three months to five years. Bone disease completely healed in one case after three years, but retinal deposits persisted despite improvement of visual acuity. A special perioperative protocol must be applied for protecting the graft from oxalate released from the stores. Laboratory follow-up including oxalemia and urinary crystal volume helps to adjust the individual prescriptions. This intensive management must be continued as long as oxalate stores persist.
Potential candidates for small bowel transplantation in pediatric patients on home parenteral nutrition.
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Isolated and combined liver-small bowel transplantation in Paris: 1987-1995.
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Evaluation of a hydrocolloid dressing.
A hydrocolloid dressing was compared to adhesive skin tapes on children's postoperative wounds. A total of 170 children of varying ages were randomised in two parallel groups, in nine centres of plastic, thoracic, gastrointestinal, urogenital and orthopaedic surgery. Skin closure was satisfactory in both groups, with 76 (89.4%) healthy closures without dehiscence in the hydrocolloid group and 81 (95.3%) in the control group; a relationship was found between partial closures/dehiscence and the type of surgical procedure. No product-related maceration, infection or adverse event was reported during the study and both groups showed very satisfactory cosmetic results.
Liver transplantation in two cases of propionic acidaemia.
Orthotopic liver transplantation (OLT) was performed in two patients with propionic acidaemia, a 7-year-old boy and a 9-year-old girl, diagnosed with a severe neonatal form with high risk of metabolic decompensation. In both cases the metabolic liver functions recovered within the 12 postoperative hours; no clinical symptoms of propionic acid toxicity, metabolic acidosis, severe hyperammonaemia, hyperglycinaemia or haematological abnormalities were observed. In both cases insulin-dependent diabetes mellitus occurred early after OLT (persisting in the boy's case). Severe post-transplantation complications were observed (acute rejection and CMV infection in both patients) which did not trigger metabolic decompensation. The boy developed chronic rejection and vanishing bile duct syndrome due to incomplete hepatic arterial thrombosis. He required permanent in-patient care with chronic hyperammonaemia and neurological sequelae involving the basal ganglia and died 15 months after OLT. The girl left hospital after 2 months and is presently leading a normal life with almost no dietary protein restriction (40 g protein per day). Urinary urea excretion and daily protein intake increased after liver transplantation. Propionyl- and tiglylglycine disappeared immediately after OLT. Urinary methylcitrate and 3-hydroxypropionate remained at concentrations corresponding to those before OLT. However, the total of all characteristic metabolites of organic acid analysis was reduced to 50-60% of the values before OLT in both patients. Propionylcarnitine was still detected at significant concentrations. Plasma odd-chain fatty acid concentrations decreased continuously after OLT only in the girl's case. Tissue of both transplanted livers showed increased odd-chain fatty acid concentrations 9 and 15 months after OLT, respectively, in both patients. We consider that at present OLT should only be performed in severe forms of propionic acidaemia.