RESUMO
The increased survival rate reached in infants with congenital diaphragmatic hernia (CDH) has shown a concomitant increase in late morbidity. Many complications including pulmonary damage, cardiovascular disease, gastro-intestinal disease, failure to thrive, neurocognitive defects and musculoskeletal abnormalities have been described. Long-term pulmonary morbidity in CDH consists of obstructive and restrictive lung function impairments due to altered lung structure and prolonged ventilatory support. Long-term consequences of pulmonary hypertension are unknown. Gastro-esophageal reflux disease (GERD) is also an important contributor to overall morbidity. Failure to thrive may be caused by GERD, insufficient intake due to oral aversion and increased caloric requirements due to pulmonary morbidity. Neurological outcome is determined by an increased risk of perinatal and neonatal hypoxemia in the first days of life of CDH patients. Many studies have addressed the substantial impact of the health problems described above, on the overall well-being of CDH patients, but only a few studies focus on the health-related quality of life. Considering the scattered data indicating substantial morbidity in long-term survivors of CDH, follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.
Assuntos
Hérnias Diafragmáticas Congênitas , Qualidade de Vida , Doenças Cardiovasculares/etiologia , Transtornos Cognitivos/etiologia , Insuficiência de Crescimento , Seguimentos , França/epidemiologia , Refluxo Gastroesofágico/etiologia , Gastroenteropatias/etiologia , Hérnia Diafragmática/complicações , Hérnia Diafragmática/mortalidade , Hérnia Diafragmática/fisiopatologia , Hérnia Diafragmática/cirurgia , Humanos , Lactente , Recém-Nascido , Pneumopatias/etiologia , Doenças Musculoesqueléticas/etiologia , Fatores de Risco , Taxa de Sobrevida , Resultado do TratamentoRESUMO
Congenital diaphragmatic hernia (CDH) is a life-threatening anomaly associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care, and new postnatal treatment strategies, the rates of mortality and morbidity in the newborn with CDH remain high as the result of severe respiratory failure secondary to PH and PPH. Later, lung function assessments show obstructive and restrictive impairments due to altered lung structure and lung damage due to prolonged ventilatory support. The long-term consequences of pulmonary hypertension are unknown. Other problems include chronic pulmonary aspiration caused by gastro-oesophageal reflux and respiratory manifestations of allergy such as asthma or rhinitis. Finally, failure to thrive may be caused by increased caloric requirements due to pulmonary morbidity. Follow-up studies that systematically assess long-term sequelae are needed. Based on such studies, a more focused approach for routine multidisciplinary follow-up programs could be established. It is the goal of the French Collaborative Network to promote exchange of knowledge, future research and development of treatment protocols.
Assuntos
Hérnia Diafragmática/complicações , Hérnias Diafragmáticas Congênitas , Displasia Broncopulmonar/etiologia , Insuficiência de Crescimento/etiologia , Refluxo Gastroesofágico/complicações , Humanos , Hipertensão Pulmonar/etiologia , Recém-Nascido , Pulmão/irrigação sanguínea , Circulação Pulmonar , Insuficiência Respiratória/etiologia , Lesão Pulmonar Induzida por Ventilação Mecânica/etiologiaRESUMO
Congenital diaphragmatic hernia (CDH) is a 1 out of 3500 live-born malformation with persistent 30-40% mortality rate, related to severe pulmonary hypoplasia and hypertension. Better knowledge on the mechanisms inducing failure of adaptation at birth is a prerequisite for improving CDH prognosis. CDH is also associated with longterm morbidity, including prolonged respiratory failure, failure to growth, oral aversion, and scoliosis. Early prevention starting as soon as the first hours of life are required to reduced long term morbidity. The aims of the management are not only to reduce early mortality, related to persistent pulmonary hypertension, but also to prevent late morbidity.
Assuntos
Hérnias Diafragmáticas Congênitas , Doenças do Recém-Nascido/terapia , Doenças do Sistema Digestório/etiologia , Doenças do Sistema Digestório/prevenção & controle , Hérnia Diafragmática/complicações , Hérnia Diafragmática/terapia , Humanos , Recém-Nascido , Doenças Respiratórias/etiologia , Doenças Respiratórias/prevenção & controleRESUMO
AIM: The treatment of achalasia consists of reducing distal esophageal obstruction by either Heller myotomy surgery or endoscopic pneumatic dilatation. The aim of the present study was to evaluate the short- and middle-term results of these procedures in children. METHODOLOGY: For technical reasons, children under six years old (n=8) were treated by surgery only, whereas patients over six years old (n=14) were treated by either Heller myotomy or pneumatic dilatation. RESULTS: Of the children aged under six years, 75% were symptom-free at six months and 83% at 24 months of follow-up. Of the patients aged over six years, complete remission was achieved by Heller myotomy in 44.5% vs. 55.5% by pneumatic dilatation after six months, and in 40% vs. 65%, respectively, after 24 months. Both pneumatic dilatation and Heller myotomy showed significant rates of failure. CONCLUSION: These results suggest that pneumatic dilatation may be considered a primary treatment in children over six years old. Also, where necessary, Heller myotomy and pneumatic dilatation may be used as complementary treatments.
Assuntos
Cateterismo , Acalasia Esofágica/terapia , Esfíncter Esofágico Inferior/cirurgia , Esofagectomia/métodos , Adolescente , Criança , Pré-Escolar , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Acalasia Esofágica/fisiopatologia , Acalasia Esofágica/cirurgia , Feminino , Humanos , Lactente , Masculino , Manometria , Qualidade de Vida , Estudos Retrospectivos , Resultado do TratamentoAssuntos
Cateterismo/métodos , Atresia Esofágica/cirurgia , Estenose Esofágica/terapia , Gastrostomia/métodos , Anastomose Cirúrgica/efeitos adversos , Anastomose Cirúrgica/métodos , Constrição Patológica/diagnóstico , Constrição Patológica/terapia , Atresia Esofágica/diagnóstico , Estenose Esofágica/etiologia , Esôfago/cirurgia , Seguimentos , Gastrostomia/instrumentação , Humanos , Íleo/cirurgia , Lactente , Masculino , Medição de Risco , Resultado do TratamentoRESUMO
BACKGROUND AND OBJECTIVES: Tissues derived from the colon, stomach, and jejunum have been used to replace the esophagus in childhood to cure esophageal atresia or stricture secondary to gastroesophageal reflux or the ingestion of corrosive agents. The outcome in adulthood of colon interposition performed at an early age has yet to be satisfactorily assessed. The aim of this single-center retrospective study was to evaluate the long-term nutritional, digestive, and respiratory outcome of all patients (n = 32) who underwent colon interposition during childhood in our hospital (1970-2001). PATIENTS AND METHODS: Medical records of these subjects were reviewed and their nutritional (weight, height, 24-hour food diary), digestive (questionnaire), and pulmonary function status evaluated. RESULTS: Of the patients, 17 had esophageal atresia (7 males, median age at surgery 11 months, range 0.5-61) and 15 had ingested corrosive substances (10 males, median age at surgery 50 months, range 22-113). Complications occurred less than 1 year postoperatively in 53% and long-term complications (occurring >1 year after surgery) in 84%. Long-term complications were common: digestive symptoms were found in 85% (most frequently observed during the first 5 years of follow-up), abnormal lung function in 7 (58%) of those tested (n = 12), feeding difficulties in 50%, scoliosis in 35%, and nutritional complications in 25%. CONCLUSIONS: Our study showed a high rate of sequelae following esophageal replacement. This highlights the need for multidisciplinary long-term follow-up into adulthood, and research into alternatives to colon interposition as treatment for esophageal strictures.
Assuntos
Colo/transplante , Atresia Esofágica/cirurgia , Estenose Esofágica/cirurgia , Esofagectomia/efeitos adversos , Estado Nutricional , Adolescente , Cáusticos/toxicidade , Criança , Pré-Escolar , Colo/patologia , Colo/cirurgia , Atresia Esofágica/complicações , Estenose Esofágica/induzido quimicamente , Estenose Esofágica/complicações , Esôfago/patologia , Esôfago/cirurgia , Feminino , Seguimentos , França/epidemiologia , Humanos , Lactente , Masculino , Complicações Pós-Operatórias/epidemiologia , Complicações Pós-Operatórias/etiologia , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
We report a case of neuroblastoma diagnosed after adrenal haemorrhage following a minor trauma in a thirteen-month-old boy. Minor trauma is not commonly described as a cause of AH in the literature. Therefore when no accepted cause for AH can be found in a young child below the age of 5 years, it is important to look for a neuroblastoma and discuss the necessity of surgical exploration.
Assuntos
Neoplasias das Glândulas Suprarrenais/diagnóstico , Hemorragia/etiologia , Neuroblastoma/diagnóstico , Acidentes por Quedas , Neoplasias das Glândulas Suprarrenais/complicações , Neoplasias das Glândulas Suprarrenais/genética , Neoplasias das Glândulas Suprarrenais/cirurgia , Glândulas Suprarrenais/irrigação sanguínea , Evolução Fatal , Humanos , Lactente , Masculino , Neuroblastoma/complicações , Neuroblastoma/genética , Neuroblastoma/cirurgia , Tomografia Computadorizada por Raios XRESUMO
OBJECTIVES: Analysis of our experience and propositions on the diagnosis and treatment of congenital bronchopulmonary malformations (BPM). POPULATION AND METHODS: Retrospective study of BPM diagnosed between 1997 and 2001. Analysis of clinical spectrum, diagnosis tools, treatment, and clinical outcome. RESULTS: Thirty-two cases of BPM have been investigated (11 cystic adenomatoid malformations, 7 pulmonary sequestrations, 7 bronchogenic cysts, 4 congenital lobar emphysema, and 3 complex emphysematous malformations). Nineteen patients had a prenatal diagnosis. For 9 others, symptoms occurred before 4 years of age. Evaluation included a CT-scan in all patients (BPM involution in one). Surgical treatment was performed in 30 patients (lobectomy in 18), with a mean age of 7 months for asymptomatic patients. During the follow-up (mean: 3 years), respiratory symptoms were reported in 10 cases, 3 of them were related to the BPM. DISCUSSION: Improvement in prenatal ultrasound diagnosis modified the management strategy. Considering the risk of pulmonary complications, surgical treatment is required during the first months of life. For congenital lobar emphysema, and some pulmonary sequestrations or small cystic adenomatoid malformations (<3 cm), conservative attitude may be preferred. BPM justify a multidisciplinary management.
Assuntos
Pulmão/anormalidades , Anormalidades do Sistema Respiratório/diagnóstico , Anormalidades do Sistema Respiratório/terapia , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Diagnóstico Pré-Natal , Prognóstico , Anormalidades do Sistema Respiratório/cirurgia , Estudos Retrospectivos , Tomografia Computadorizada por Raios XRESUMO
The orthotopic liver transplantation (OLT) allows survival of children followed for severe hepatic injury, provided that the immunosuppressive treatment is prolonged. The nephrotoxicity of cyclosporine predicts the long-term outcome of the adult patients receiving a liver transplant. The aim of this study was to determine the long-term outcome of renal function in children receiving OLT. This study included 12 children, with a median for age of 7.1 yr (2-15 yr) at the time of OLT. The duration of follow-up was at least 4 yr, being 7 yr in 10 patients and more than 10 yr in seven. Renal function was evaluated with the serum level of creatinine, calculated glomerular filtration rate (cGFR), and measurement of glomerular filtration rate using chrome 51 ethylenediaminetetraacetate ((51)Cr EDTA) clearance performed at least once during follow-up. The doses and the serum concentrations (C(0)) of cyclosporine were reported at each study time. The cGFR decreased significantly 2 yr after the OLT [median (range): 106 mL/min/1.73 m(2) (71-150) at the time of OLT vs. 85 mL/min/1.73 m(2) (57-128) 2 yr after the OLT, p = 0.03], and decreased again between 7 and 10 yr after OLT [median (range): 99 mL/min/1.73 m(2) (76-125) 7 yr after OLT vs. 81 mL/min/1.73 m(2) (66-140) 10 yr after OLT, p = 0.04]. Six patients developed chronic renal failure (cGFR from 57 to 80 mL/min/1.73 m(2)) 2 yr after OLT associated with high doses of cyclosporine [median (range): 8.8 mg/kg/day (3.5-13)]. The cGFR overestimated renal function by 16% compared with the isotopic measurement of GFR (p = 0.03). Using the (51)Cr EDTA measurement, six of seven patients followed up more than 10 yr after OLT presented mild (n = 3) or moderate (n = 3) chronic renal failure. In our study, the majority of OLT recipients developed a chronic renal failure 10 yr after transplantation. Cyclosporine seems to be the most important factor responsible for the impairment of renal function. The use of the mycophenolate mofetil, a new immunosuppressive agent, allowing a reduction in the dose of cyclosporine, could minimize renal dysfunction. While awaiting the results of a prospective long-term study, close drug monitoring is advised.
Assuntos
Atresia Biliar/cirurgia , Degeneração Hepatolenticular/cirurgia , Rim/fisiologia , Transplante de Fígado/fisiologia , Adolescente , Criança , Pré-Escolar , Ciclosporina/efeitos adversos , Ciclosporina/uso terapêutico , Feminino , Taxa de Filtração Glomerular , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Falência Renal Crônica/induzido quimicamente , Falência Renal Crônica/epidemiologia , Testes de Função Renal , Masculino , Resultado do TratamentoAssuntos
Pneumopatias/congênito , Pneumopatias/diagnóstico , Pulmão/anormalidades , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Pulmão/embriologia , Gravidez , Diagnóstico Pré-Natal , PrognósticoRESUMO
In children, the watery diarrhoea-hypokalemia-achlorhydria (WDHA) syndrome is uncommon and usually due to a neuroblastic tumour hypersecreting the vasoactive intestinal peptide (VIP). We report a case of WDHA syndrome secondary to hypersecretion of VIP that revealed a neuroblastoma in a 13-month-old girl. A secretory diarrhoea, characterised by the persistence of diarrhoea despite the cessation of oral feeding, led to the search of a neuroblastic tumour in the patient. The serum concentration of VIP decreased to normal values soon after the surgical excision of the tumour.
Assuntos
Diarreia/etiologia , Neuroblastoma/complicações , Peptídeo Intestinal Vasoativo/análise , Vipoma/complicações , Diagnóstico Diferencial , Feminino , Humanos , Lactente , Neuroblastoma/diagnóstico , Peptídeo Intestinal Vasoativo/farmacologia , Vipoma/diagnósticoRESUMO
A mesenchymal hamartoma (MH) of the liver in a seven-month-old girl was associated with slightly elevated alpha fetoprotein (AFP) concentrations (320 microg/L, normal < 25 after six months of age). Hepatocytes were abundant at the periphery of the tumour at histopathological examination. These were slightly atypical and expressed AFP immunohistochemically. In two other girls, aged 14 months and two months respectively, with MH and increased serum AFP concentrations (320 microg/L and 7500 microg/L, respectively), hepatoblastomas were misdiagnosed preoperatively. MH of the liver with increased serum AFP concentrations may thus mimic hepatoblastoma radiologically if cysts are lacking, and foetal hepatoblastoma pathologically if a biopsy samples only the peripheral hepatocellular component of the MH.
Assuntos
Hamartoma/diagnóstico , Hepatoblastoma/diagnóstico , Hepatopatias/diagnóstico , Neoplasias Hepáticas/diagnóstico , alfa-Fetoproteínas/análise , Diagnóstico Diferencial , Feminino , Hamartoma/sangue , Hepatócitos/patologia , Humanos , Lactente , Fígado/patologia , Hepatopatias/sangueRESUMO
UNLABELLED: Prognosis of extrahepatic biliary atresia depends on an early surgical treatment. This survey studied the delay to surgery of infants treated for biliary atresia and analysed the causes of late diagnosis and referral. MATERIAL AND METHODS: Medical files of 21 infants treated for biliary atresia between 1988 and 1998 were retrospectively analysed. RESULTS: Median age at biliary operation was 57 days and did not change during time. In only 3/21 cases, surgery was performed before 45 days of age. The first clinical or biological sign of cholestasis was noted at a median age of 12 days. In eight cases, the first medical visit for cholestasis (median age of 21 days) was not followed by a blood test. In 11 cases, infants presenting with biological cholestasis were referred to hospital more than seven days later. False diagnosis was noted in seven cases and delayed significantly the operation. CONCLUSION: The delay to surgical treatment is too long and does not decrease whereas a majority of infants visit their physician early. Medical information is mandatory for all physicians taking care of infants.
Assuntos
Atresia Biliar/diagnóstico , Atresia Biliar/cirurgia , Colestase/etiologia , Diagnóstico Diferencial , Erros de Diagnóstico , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Tempo , Resultado do TratamentoRESUMO
UNLABELLED: Anastomotic stricture is the most common complication following the surgical repair of esophageal atresia, and is usually treated by esophageal dilation. OBJECTIVES: The aims of this study were to assess in an infant population operated on at birth for type III or IV esophageal atresia: 1) the frequency of esophageal stenosis following the repair of esophageal atresia, and associated factors; 2) the efficacy of esophageal dilation by the Savary-Gaillard bougie technique. MATERIALS AND METHODS: The medical records of 52 children presenting with esophageal atresia over a 5-year period were retrospectively reviewed. Gestional age and birth weight, duration of mediastinal and transanastomotic drainage, and anastomotic complications including leakage, stricture, and the presence of gastroesophageal reflux were recorded and analysed. Patients presenting with anastomotic stricture were compared with a group of children without stricture. The number of esophageal dilations, their efficacy and the complication rate were analyzed. RESULTS: Anastomotic stricture developed in 20 (40%) of the 50 patients undergoing primary repair for esophageal atresia. The occurrence of anastomotic stricture was related to anastomotic tension during esophageal surgical repair (p < 0.03). Young children required esophageal dilation at a mean age of 142 days (24-930 days). Stricture resolution occurred after a mean of 3.2 dilations (1-15) over an average period of 7.9 months (range: 0-30 months). Dilation was successful in 90% of the 20 patients. Seven patients required only one dilation. Perforation of the esophagus occurred in one case, and this severe complication led to the death of the child. Esophageal dilation was unsuccessful in two patients, who presented prolonged severe dysphagia. CONCLUSION: Anastomotic stricture following repair of esophageal atresia is connected with the length of the gap that has to be repaired, and tension during suture. Esophageal dilation by the Savary-Gaillard bougie technique is an effective method for treating esophageal stricture. Several dilations are usually needed before the disappearance of dysphagia.
Assuntos
Anastomose Cirúrgica/efeitos adversos , Atresia Esofágica/cirurgia , Estenose Esofágica/etiologia , Estenose Esofágica/terapia , Fatores Etários , Peso ao Nascer , Dilatação/métodos , Feminino , Idade Gestacional , Humanos , Incidência , Recém-Nascido , Masculino , Estudos Retrospectivos , Fatores de Risco , Técnicas de Sutura , Resultado do TratamentoRESUMO
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.