RESUMO
OBJECTIVE: Morbidity in fetuses affected by gastroschisis is mainly the result of bowel ischaemic and inflammatory processes. Experimental studies on animal models show that clearing amniotic fluid from the digestive secretions by amnioexchange procedures reduces the inflammatory process. We evaluated the benefit of the amnioexchange procedure for fetal gastroschisis in humans. DESIGN: Prospective, interventional, randomised study. SETTING: Eight referral centres for fetal medicine. POPULATION: Pregnant women carrying a fetus with gastroschisis. METHODS: We compared, in utero, amnioexchange with a sham procedure. The protocol included, in both arms, steroid injections at 30 weeks of gestation and the use of postnatal minimal enteral feeding. MAIN OUTCOME MEASURES: The primary outcome was a composite variable based on the duration of ventilation and parenteral nutrition. Secondary outcomes were the effectiveness and safety of the amnioexchange procedure, including the rate of perinatal death, time to full enteral feeding, primary closure, and late feeding disorders. RESULTS: Sixty-four patients were randomised. There was no difference in the composite criteria between the amnioexchange and control groups. Based on an intention-to-treat analysis, there were no significant between-group differences in pregnancy outcome or complications. When studying the relationship between digestive compounds and amniotic fluid inflammatory markers, a clear correlation was found between bile acid and both ferritin and interleukin 1ß (IL1ß). CONCLUSIONS: In humans, amnioexchange, as described in our protocol, is not an option for fetal care; however, we provide supplementary proof of the involvement of inflammation in the pathogenicity of gastroschisis and suggest that future research should aim at reducing inflammation. ClinicalTrials.gov: NCT00127946. TWEETABLE ABSTRACT: A prospective, interventional, randomised study shows no benefit of amnioexchange for fetal gastroschisis in humans.
Assuntos
Líquido Amniótico/química , Cloretos/administração & dosagem , Drenagem/métodos , Doenças Fetais/terapia , Gastrosquise/terapia , Cuidado Pré-Natal/métodos , Cloreto de Sódio/administração & dosagem , Adulto , Biomarcadores/análise , Cloretos/farmacocinética , Drenagem/efeitos adversos , Feminino , Doenças Fetais/diagnóstico , Gastrosquise/diagnóstico , Idade Gestacional , Humanos , Mediadores da Inflamação/análise , Gravidez , Resultado da Gravidez , Estudos Prospectivos , Cloreto de Sódio/farmacocinéticaRESUMO
INTRODUCTION: The first oesophagogastric dissociation was described by Bianchi in 1997 for the treatment of severe gastro-oesophageal reflux (GOR) in neurological patients to avoid serious respiratory complications. The dissociation leads to malabsorption and growth problems. We describe the first two cases of oesophagogastric reconnection long after lung growth. PATIENTS: Case no. 1 was a 12.7-year-old child with a history of type I oesophageal atresia. He required oesophagocoloplasty and gastric dissociation by the Bianchi procedure at the age of 3 months. He has oesophagogastric reconnection at 11 years old because he had a several macrocytic anaemia. Case no. 2 was also a 12-year-old boy, with an unlabelled multiple malformation syndrome with type I oesophageal atresia. He had an oesophagocoloplasty with gastric dissociation at the age of 3 months to protect his lungs. The reconnection was proposed at 10 years old because he developed multiple nutritional deficiencies and growth retardation. RESULTS: The two cases have no clinical symptom of reflux and their post-operative digestive contrasts are normal. Reconnection appears possible with or without preserving the Roux-en-Y loop. It improves absorption and corrects any vitamin deficiencies, allows the reconnected stomach to be monitored in terms of reflux and malignancy and enables enteral nutrition supplementation to be stopped. The possibility of reconnection after the Bianchi procedure therefore extends its indications, mostly in the case of oesophageal atresia complicated by severe GOR with pulmonary repercussions or tracheoesophageal cleft.
Assuntos
Anastomose em-Y de Roux/efeitos adversos , Atresia Esofágica/cirurgia , Junção Esofagogástrica/cirurgia , Esofagoplastia/efeitos adversos , Gastrectomia/efeitos adversos , Complicações Pós-Operatórias/cirurgia , Anemia/etiologia , Anemia/cirurgia , Criança , Seguimentos , Humanos , Masculino , Deficiência de Vitamina B 12/etiologia , Deficiência de Vitamina B 12/cirurgiaRESUMO
The prenatal diagnosis of esophageal atresia is challenging. The length of the defect of the esophageal atretic portion is one of the parameters affecting outcome and prenatal evaluation of this length has not, to our knowledge, been described previously. We report on seven fetuses assessed prospectively which were suspected to have esophageal atresia. Targeted ultrasound examination of both fetal cervical and thoracic structures was performed in each case in order to assess prenatally the atretic portion. The length of the defect was assessed both directly, by visualizing the interruption of the hyperechoic lines representing the walls of the esophagus in a mid-sagittal view (n = 4), and indirectly, by means of the 'tracheal print' (n = 5). Both methods were used in three cases. Prenatal results were compared with postnatal or postmortem findings. The prenatal diagnosis of esophageal atresia was made correctly in six of the seven cases and in all of these there was concordance between prenatal and postnatal estimates of the esophageal defect lengths. Direct or indirect sonographic assessment of the esophagus in cases of suspected prenatal esophageal atresia improves the specificity of its diagnosis and aids prenatal evaluation.
Assuntos
Atresia Esofágica/diagnóstico por imagem , Ultrassonografia Pré-Natal/métodos , Pré-Escolar , Atresia Esofágica/embriologia , Feminino , Humanos , Valor Preditivo dos Testes , Gravidez , Terceiro Trimestre da Gravidez , Estudos Prospectivos , Fatores de Risco , Sensibilidade e Especificidade , Ultrassonografia Pré-Natal/normasRESUMO
Endogenous nitric oxide (NO) mediates pulmonary vasodilatation at birth, but inhaled NO fails to reduce pulmonary vascular resistance (PVR) in newborns with congenital diaphragmatic hernia (CDH). This study was designed to investigate the effects of ventilation, and the nature of its endogenous mediator, in fetal lambs with experimental CDH. Investigations at 138 days of gestation showed that ventilation markedly decreased PVR. Inhibition of NO synthesis reduced ventilation-induced pulmonary vasodilatation in vivo and increased in vitro isometric tension of vascular rings. Ventilation therefore reduces PVR at birth in lambs with CDH, and endogenous NO seems to contribute to this reduction.
Assuntos
Hérnia Diafragmática/terapia , Pulmão/irrigação sanguínea , Óxido Nítrico/metabolismo , Circulação Pulmonar , Respiração Artificial , Vasodilatação , Animais , Modelos Animais de Doenças , Estimulação Elétrica , Inibidores Enzimáticos/farmacologia , Idade Gestacional , Hemodinâmica , Hérnia Diafragmática/fisiopatologia , Hérnias Diafragmáticas Congênitas , Óxido Nítrico Sintase/antagonistas & inibidores , Óxido Nítrico Sintase/metabolismo , Nitroarginina/farmacologia , Circulação Pulmonar/efeitos dos fármacos , Ovinos , Resistência Vascular , Vasodilatação/efeitos dos fármacosRESUMO
BACKGROUND: Lymphangiomas are rare benign lesions of the lymphatic system. The most common symptoms are abdominal tumor or "acute abdomen" in children. The treatment of choice is complete surgical resection, but the recurrence rate with incomplete resection is high, and laparotomy exposes the patient to adhesions. The authors report their experience with the lymphangioma laparoscopic approach. METHODS: This retrospective study examined 15 consecutive operations for lymphangiomas in children, ages 5 months to 14 years, treated during the 5-year period from 1999 to 2004. RESULTS: Six patients were treated using the primary laparotomy approach, and nine patients underwent the laparoscopic procedure, six successfully. Three conversions were necessary (1 case requiring partial colectomy, 1 retroperitoneal case with adherence on the aorta and vena cava, 1 case with partial volvulus). Morbidity included two cases of acute occlusion caused by adhesions after laparotomy. There was no recurrence of lymphangioma during a mean follow-up period of 35 months. CONCLUSION: The laparoscopy procedure could be used successfully for abdominal lymphangioma, even in an emergency. When the laparoscopic resection is impossible, laparotomy or sclerotherapy can be discussed.
Assuntos
Neoplasias Abdominais/cirurgia , Laparoscopia/métodos , Linfangioma/cirurgia , Neoplasias Abdominais/diagnóstico , Adolescente , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Previsões , Humanos , Lactente , Laparoscopia/tendências , Laparotomia/métodos , Laparotomia/tendências , Linfangioma/diagnóstico , Linfangioma Cístico/diagnóstico , Linfangioma Cístico/cirurgia , Masculino , Procedimentos Cirúrgicos Minimamente Invasivos/métodos , Procedimentos Cirúrgicos Minimamente Invasivos/tendências , Estadiamento de Neoplasias , Estudos Retrospectivos , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Ultrassonografia DopplerRESUMO
PURPOSE: Whereas gastrointestinal symptoms such as vomiting, diarrhea and abdominal pain are common in children suffering from the so-called post-diarrheal form (D+) of hemolytic uremic syndrome (HUS), more serious gastrointestinal complications are rare. We tried to define factors predictive of the severity of gastrointestinal complications post D+ HUS. METHODS: We reviewed the files of all children admitted to our hospital for D+ HUS between 1988 and 2000. We retained those cases with gastrointestinal complications and analyzed the consequences of these complications on the evolution of the children's conditions. RESULTS: Sixty-five children with D+ HUS were admitted to our hospital during this period. Sixteen children developed gastrointestinal complications involving one or more digestive organs: necrosis of the colon or ileum, hemorrhagic colitis, pancreatitis, transient diabetes, hepatic cytolysis and cholestasis, peritonitis and prolapse of the rectum. One child died. CONCLUSION: Gastrointestinal complications of D+ HUS are rare, but they can be lethal, and early surgery may sometimes prove necessary. However, we were not able to demonstrate a correlation between the severity of the gastrointestinal manifestations and the clinical or biological signs accompanying D+ HUS.
Assuntos
Diarreia/complicações , Gastroenteropatias/etiologia , Síndrome Hemolítico-Urêmica/complicações , Adolescente , Criança , Pré-Escolar , Diagnóstico Diferencial , Feminino , Seguimentos , Gastroenteropatias/diagnóstico , Gastroenteropatias/epidemiologia , Humanos , Incidência , Lactente , Masculino , Prognóstico , Estudos Retrospectivos , Índice de Gravidade de DoençaRESUMO
Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, associated with a poor outcome. We report the case of a 7 week-old boy with a pleuropulmonary blastoma classified as type I. Disease was discovered at a chest X-ray performed as a work-up for a benign acute viral bronchiolitis. The final diagnosis was brought by pathology: a bronchopulmonary malformation had not been ruled out by clinical, radiological and macroscopic findings. Pleuropulmonary blastoma is a rare childhood intrathoracic neoplasm, for which the poor outcome and tough diagnosis justify a surgical attitude when an intrathoracic bullous lesion is found in an infant.
Assuntos
Neoplasias Embrionárias de Células Germinativas/diagnóstico por imagem , Blastoma Pulmonar/diagnóstico por imagem , Neoplasias do Sistema Respiratório/diagnóstico por imagem , Criança , Malformação Adenomatoide Cística Congênita do Pulmão/diagnóstico por imagem , Humanos , Masculino , Radiografia Torácica , Tomografia Computadorizada por Raios XRESUMO
AIM OF THE STUDY: The purpose of this study is to describe the management of infants with gastroschisis (G) and omphalocele (O) during the first 7 days after surgery. METHODS: A retrospective review of all cases of O or G managed at the ICU of the Robert Debré Teaching Hospital between January 1993 and July 2000 was carried out. PATIENTS: 29 infants with G, 15 with O (12 unruptured O [UO] and 3 ruptured O [RO]). RESULTS: Ventilatory support consisted of conventional mechanical ventilation (46 %) and/or in high-frequency oscillatory ventilation (61 %). After day 4, ventilatory requirements evaluated by mean airway pressure (MAP) differed significantly between G (n = 10/29) and O (n = 7/15; group vs. day of life, p = 0.04). The average of MAP measured on days 5, 6, and 7 was significantly higher in O than in G (14.7 +/- 3.0 versus 10.9 +/- 2.8, p < 0.01, respectively). Volume expansion was required at least once in 90 % of patients. Mean fluid requirements were significantly lower in UO than in G and in RO (41 +/- 31 ml/kg, 91 +/- 73 ml/kg, and 137 +/- 25 ml/kg, respectively; p = 0.02 for each comparison). Patients with G were significantly more likely to receive norepinephrine (59 % vs. 20 %, p = 0.027) than patients with O. Twenty-six infants with G (90 %) and 11 with O (73 %) were discharged alive from ICU. CONCLUSIONS: Haemodynamic instability can be expected in patients with G or RO, and ventilatory requirements were higher in infants with O than in infants with G during the first week after surgery.
Assuntos
Gastrosquise/cirurgia , Hérnia Umbilical/cirurgia , Cuidados Pós-Operatórios , Feminino , Hidratação/métodos , Humanos , Recém-Nascido , Unidades de Terapia Intensiva Neonatal , Masculino , Respiração Artificial/métodos , Estudos RetrospectivosRESUMO
BACKGROUND: Laparoscopic surgery in patients with Crohn's disease (CD) has been demonstrated to have advantages over a conventional approach in children. The aim of this study was to review the children treated for CD with a laparoscopic approach, to report our indications, the surgical procedure, the complications, and to compare the children with pancolitis or ileocaecal (segmental) Crohn's disease. PATIENTS AND METHOD: We reviewed the files of 11 children treated for CD in a single institution between 1999 and 2004 for a retrospective study of clinical and surgical data. Mann-Whitney U-test was used for statistical analysis of nonparametric data. RESULTS: Eleven children were operated. The average age when initial clinical symptoms became apparent was 12.1 years (range 6.6 - 15), and surgery was performed after an average of 3.4 years of disease (range 1 - 7.6). The surgical indications were stenosis in 6 cases, failure to thrive in 1 case (segmental CD, SCD group) and pancolitis refractory to medical treatment in 4 cases (pancolitis group, PCD group). Mean operative time was 207 minutes (range 140 - 270) for the SCD group and 285 minutes (range 260 - 300) for the PCD group (p < 0.05). Three cases needed a conversion to open surgery (2 in PCD group, one in SCD group), mainly in relation to anastomosis performed with an EEA stapler. The average length of surgical unit stay was 6.5 days (range 4 - 8) for the PCD group and 6.4 days (range 4 - 8) for the SCD group; average follow-up was 16 months (range 3 - 38). Two patients had a relapse of CD (stenosis of the anastomosis in one, skin fistula in the other). CONCLUSION: A laparoscopic approach for ileocolic resection in Crohn's disease is a feasible procedure, even in cases of pancolitis. We recommend an extra-corporeal anastomosis because, in relation to the inflammatory bowel, the mechanical anastomosis is not a safe procedure in cases of pancolitis.
Assuntos
Doença de Crohn/cirurgia , Laparoscopia , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos Retrospectivos , Resultado do TratamentoRESUMO
BACKGROUND: In the management of autoimmune myasthenia, thymectomy is recognized as effective surgical therapy. The necessity of complete radical thymectomy to achieve maximal improvement has been emphasized. Video-assisted thoracoscopic surgery has been successfully used for thymectomy in adults, and more recently in children, and has been described as achieving the same radicality and functional improvement as median sternotomy or as transcervical thymectomy. The aim of this work is to report our first thoracoscopic experience in this indication. METHODS: Patients with myasthenia gravis on anticholinesterase drugs and/or steroids are discussed for surgery in case of clinical deterioration despite increasing doses of medication or in case of no improvement. We decided to perform thoracoscopic thymectomies by a left-sided approach. Preoperative localization of thymic tissue is done by a thoracic CT exam. Patients are placed on their right side with a thoracic tilt under the thorax. Four thoracoscopic ports are used, a 10-mm for the camera and three 5-mm operating ports. The left lung was collapsed by selective intubation (double-lumen endotrachial intubation). RESULTS: Two boys, 7.5 and 14 years old, were addressed by the department of neurology for radical thymectomy. They presented an ocular myasthenia gravis for 2 years and a mild general myasthenia gravis for 7 years. The operative times were 120 and 240 min. There was no intraoperative or postoperative complication. Duration of thoracic drainage was 2 days. The children were discharged on the third postoperative day. For the second procedure, an ultrasound exam during surgery was necessary to localize the thymus exactly, thus enabling its complete resection without the need for a conversion. The follow-up is 19 and 7 months with a clinical improvement enabling the diminution of medication for both children, the end of ptosis for the first child, and the general improvement of muscle strength for the second. CONCLUSIONS: Thoracoscopic thymectomy in children with juvenile myasthenia gravis seems to offer a complete surgical resection, as do open techniques. In case of difficulties in finding the thymus, an ultrasound exam is feasible to enable complete resection. The left-sided thoracoscopic approach gives a good mediastinal and cervical exposition. Furthermore, being less painful in the postoperative period, it presents a less pronounced impairment of pulmonary function, and it presents good cosmetic effect.
Assuntos
Miastenia Gravis/cirurgia , Toracoscopia/métodos , Timectomia/métodos , Adolescente , Criança , Humanos , MasculinoRESUMO
BACKGROUND: The aim of this study was to compare the operative outcome in children undergoing open vs thoracoscopic resection of bronchogenic cysts. METHODS: The medical records of children who underwent the resection of bronchogenic cysts from 1990 through 2000 were reviewed. Four cyst resections were performed by the open technique and five using a thoracoscopic procedure. The age of the patients, length of hospital stay, duration of drainage, operating time, and outcome were investigated. RESULTS: The mean age of patients undergoing the open procedure was 3 years and 3 months; the mean age for thoracoscopy patients was 7 years and 10 months (p < 0.05). The operating time for the open procedure was 70 +/- 25 min; for the laparoscopic procedure, it was 78 +/- 6 min (p, NS), except in one case with a main bronchial tail that required conversion (320 min). Duration of surgical drainage was 6.5 +/- 3 days for the open procedure and 2.5 +/- 1 days for the thoracoscopic one (p < 0.05). Hospital stay for open patients was 12 days +/- 0 days; it was 6 +/- 1.6 days for thoracoscopic patients (p < 0.01). There were no deaths. The thoracoscopic procedure failed once due to a main bronchial tail and had to be converted to an open procedure. Other early complications included a bronchopulmonary infection after an open cyst excision and an atelectasis after a thoracoscopic cyst excision. Late complications included one reoperation for incomplete excision in each of the two groups. CONCLUSION: Bronchogenic cyst resection can be performed safely. For complete treatment of these patients, total excision of the wall cyst is needed. In selected patients, the thoracoscopic procedure may decrease the duration of surgical drainage and length of hospital stay without increasing the operating time or MSK for complications.
Assuntos
Cisto Broncogênico/cirurgia , Toracoscopia , Toracotomia , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Recém-Nascido , Masculino , Estudos RetrospectivosRESUMO
UNLABELLED: Despite regular progress in neonatal intensive care, congenital diaphragmatic hernia (CDH) diagnosed antenatally is still associated with up to 80 % mortality. It is impossible to predict which fetus with CDH will survive or not. OBJECTIVE: To identify reliable antenatal predictors of outcome and of pulmonary hypoplasia (PH) in fetuses with CDH. DESIGN: Retrospective study. SETTING: Paediatric intensive care unit of a university children's hospital. PATIENTS AND METHODS: Antenatal parameters and presence of left ventricular hypoplasia in utero were compared retrospectively to outcome and to presence of PH in 32 consecutive newborn infants with antenatally diagnosed CDH. Antenatal parameters included: gestational age at diagnosis, herniated organs, associated malformations and presence of polyhydramnios. Size of the cardiac ventricles, the aorta (Ao) and the pulmonary artery (PA) were obtained by fetal echocardiography, from which we calculated a cardioventricular index (left ventricle/right ventricle, LV/RV) and a cardiovascular index (Ao/PA). Delivery was planned in order to provide ventilatory and hemodynamic management. In case of death, PH was assessed according to the following criteria: the lung weight/body weight index and the radial alveolar count. For statistical comparisons, patients were separated into two groups: the hypoplasia group (H) and the non-hypoplasia group (NH). RESULTS: Thirty-two pregnancies were delivered. Twenty-six newborns died (81 %), 6 survived (19 %). When comparing non-survivors to survivors, predictors of poor outcome were: mean gestational age at diagnosis (23 vs 28 weeks, p = 0.002), intrathoracic stomach (20 vs 1 s, p = 0.01) and associated malformations (6 vs 0). Cardiac ventricular disproportion, expressed by the LV/RV ratio, appeared to correlate well with a poor outcome (0.63 in non-survivors vs 0.93 in survivors, p = 0.03) and with PH (0.63 in the H group vs 0.95 in the NH group, p = 0.03). CONCLUSIONS: Our study confirmed the factors for a poor prognosis associated with CDH previously described in the literature, but none with a consistent demonstration of accuracy. LV hypoplasia may be a more accurate predictor of outcome and of PH but it has to be assessed by prospective studies with larger samples. Further basic science and Doppler-flow studies may be helpful to understand the natural history and pathophysiology of LV hypoplasia in CDH.
RESUMO
UNLABELLED: Despite regular progress in neonatal intensive care, congenital diaphragmatic hernia (CDH) diagnosed antenatally is still associated with up to 80% mortality. It is impossible to predict which fetus with CDH will survive or not. OBJECTIVE: To identify reliable antenatal predictors of outcome and of pulmonary hypoplasia (PH) in fetuses with CDH. DESIGN: Retrospective study. SETTING: Paediatric intensive care unit of a university children's hospital. PATIENTS AND METHODS: Antenatal parameters and presence of left ventricular hypoplasia in utero were compared retrospectively to outcome and to presence of PH in 32 consecutive newborn infants with antenatally diagnosed CDH. Antenatal parameters included: gestational age at diagnosis, herniated organs, associated malformations and presence of polyhydramnios. Size of the cardiac ventricles, the aorta (Ao) and the pulmonary artery (PA) were obtained by fetal echocardiography, from which we calculated a cardioventricular index (left ventricle/right ventricle, LV/RV) and a cardiovascular index (Ao/PA). Delivery was planned in order to provide ventilatory and hemodynamic management. In case of death, PH was assessed according to the following criteria: the lung weight/body weight index and the radial alveolar count. For statistical comparisons, patients were separated into two groups: the hypoplasia group (H) and the non-hypoplasia group (NH). RESULTS: Thirty-two pregnancies were delivered. Twenty-six newborns died (81%), 6 survived (19%). When comparing non-survivors to survivors, predictors of poor outcome were: mean gestational age at diagnosis (23 vs 28 weeks, p = 0.002), intrathoracic stomach (20 vs 1 s, p = 0.01) and associated malformations (6 vs 0). Cardiac ventricular disproportion, expressed by the LV/RV ratio, appeared to correlate well with a poor outcome (0.63 in non-survivors vs 0.93 in survivors, p = 0.03) and with PH (0.63 in the H group vs 0.95 in the NH group, p = 0.03). CONCLUSIONS: Our study confirmed the factors for a poor prognosis associated with CDH previously described in the literature, but none with a consistent demonstration of accuracy. LV hypoplasia may be a more accurate predictor of outcome and of PH but it has to be assessed by prospective studies with larger samples. Further basic science and Doppler-flow studies may be helpful to understand the natural history and pathophysiology of LV hypoplasia in CDH.
Assuntos
Anormalidades Múltiplas , Ventrículos do Coração/anormalidades , Hérnia Diafragmática/diagnóstico , Hérnias Diafragmáticas Congênitas , Diagnóstico Pré-Natal/métodos , Feminino , Idade Gestacional , Ventrículos do Coração/embriologia , Hérnia Diafragmática/mortalidade , Humanos , Mortalidade Infantil , Recém-Nascido , Pulmão/anormalidades , Masculino , Valor Preditivo dos Testes , Gravidez , PrognósticoRESUMO
OBJECTIVES: to evaluate the analytical performances of the Hitachi 911 analyzer (Roche Diagnostics, F) for gamma-glutamyl-transferase, 5'nucleotidase, amylase, aspartate-amino-transferase and total-alkaline-phosphatase assay in amniotic fluid. To establish reference intervals for these five enzymes throughout gestation. To determine their antenatal diagnostic value. DESIGN AND METHODS: amniotic fluid samples were collected between 14 and 35 weeks of gestation. Weekly numbers ranged from 31 to 92. Techniques developed for serum enzyme assays were applied to amniotic fluid. Two pools of amniotic fluid containing low and high marker levels were used. Within-day and between-day variations were calculated, together with the limits of detection and linearity. Reference ranges were established on 508 amniotic fluid samples, including 23 samples from pregnancies with chromosomal aberrations, 14 with gastrointestinal tract defects and 5 with gastroschisis. RESULTS: the assay technique for total-alkaline-phosphatase assay had to be adapted to amniotic fluid, but no adaptation was necessary for the other markers. The within-day CV ranged between 2.2 and 11.2% for low-level samples and from 1.1 to 3.4% for high-level samples. The between-day CV ranged from 6.3 to 13.3% for low-level samples and 1.2 to 4.7% for high-level samples. Total-alkaline-phosphatase activity fluctuated throughout gestation. Amylase levels and aspartate-amino-transferase levels increased whereas gamma-glutamyl-transferase and 5'nucleotidase levels fell until delivery. All trisomy 18 and trisomy 13 pregnancies, 65% of Down's syndrome pregnancies and all pregnancies with digestive tract defects were associated with marked changes in the level of at least one enzyme. CONCLUSION: The Hitachi 911 is suited to rapid, reliable quantification of amniotic fluid enzymes with only minor adaptation. Useful reference intervals can be obtained throughout gestation. Gamma-glutamyl-transferase, 5'nucleotidase, total-alkaline-phosphatase and amylase assay can help to confirm echographic evidence of bowel disorders and thereby improve patient management especially in case of gastroschisis.
Assuntos
Amniocentese/métodos , Líquido Amniótico/metabolismo , Química Clínica/métodos , Diagnóstico Pré-Natal , Espectrofotometria/métodos , Fosfatase Alcalina/metabolismo , Amilases/metabolismo , Aspartato Aminotransferases/metabolismo , Feminino , Idade Gestacional , Humanos , Modelos Estatísticos , Nucleotidases/metabolismo , Gravidez , Fatores de Tempo , Trissomia , gama-Glutamiltransferase/metabolismoRESUMO
Congenital malformations of the tracheobronchal tree and the related arterial blood supply are a complex group of lesions in which there are abnormalities of the venous drainage and lung parenchyma. These malformations are examples of congenital pulmonary venolobar syndrome (CPVS). Tracheal trifurcation is an extremely rare anomaly associated with CPVS. We report on an unusual case of lower right extralobar sequestration connected to the trachea, plus a type I posterior laryngeal cleft, an aberrant systemic artery, and an anomalous route of the phrenic nerve. This paper discusses the place of this unusual abnormality in the spectrum of congenital bronchopulmonary vascular malformations.
Assuntos
Anormalidades Múltiplas , Brônquios/anormalidades , Sequestro Broncopulmonar/patologia , Laringe/anormalidades , Traqueia/anormalidades , Brônquios/irrigação sanguínea , Humanos , Lactente , Masculino , Nervo Frênico/anormalidades , SíndromeRESUMO
BACKGROUND: Solitary ulcer of rectum syndrome occurs rarely in children, and the diagnosis may be difficult. Rectopexy has already been reported with good results in adults and children. We report the first laparoscopic rectopexy in children. METHODS: We report the first case of rectopexy performed according to the Orr-Loygue technique using a laparoscopic approach in children. Three trocars were used. A polypropylene mesh was sutured on each side of the rectum and fixed to the promontary with a nonabsorbable suture. The operative time was 150 min. RESULTS: The postoperative course was uneventful. The boy was discharged on postoperative day 3. The rectoscopy at 4 months was normal. The rectal bleeding and pain had disappeared by the 18-month follow-up assessment. CONCLUSION: We report a successful laparoscopic rectopexy for solitary ulcer of rectum syndrome in a child. The procedure is simple, safe, and reproductible, giving good results in the reported case.
Assuntos
Laparoscopia , Doenças Retais/cirurgia , Úlcera/cirurgia , Criança , Procedimentos Cirúrgicos do Sistema Digestório/métodos , Humanos , SíndromeRESUMO
BACKGROUND: Gallbladder stones are very common in patients with sickle cell disease and are the cause of recurrent abdominal pain. Their management has been highly controversial, especially for children. Nonoperated patients and those treated on an emergency basis have a very high rate of morbidity (>50%). METHODS: We performed a retrospective review of a series of 29 homozygous SS sickle cell children who underwent laparoscopic cholecystectomy between 1991 and April 1998. RESULTS: Only in one case a conversion was necessary (early in the series). Exploration of the common bile duct was done via intraoperative cholangiography. There were no mortalities. The morbidity rate was 17%; (however, of the five patients concerned, four suffered from hyperthermia for 2 days. All of the children were improved and enjoyed resolution of their abdominal pain. CONCLUSIONS: We believe that elective laparoscopic cholecystectomy at the earliest time possible, along with correct perioperative management, is the treatment of choice for cholelithiasis in children with sickle cell disease.
Assuntos
Anemia Falciforme/complicações , Colecistectomia Laparoscópica/métodos , Colelitíase/etiologia , Colelitíase/cirurgia , Dor Abdominal/cirurgia , Acetaminofen/uso terapêutico , Doença Aguda , Criança , Pré-Escolar , Colecistite/cirurgia , Feminino , Humanos , Masculino , Nalbufina/uso terapêutico , Dor Pós-Operatória/tratamento farmacológicoRESUMO
INTRODUCTION: Splenectomy is frequently performed in children for various hematologic and autoimmune disorders. We reviewed our indication for splenectomy, our technique of laparoscopic, splenectomy, and our results. The aim of this study was to show if an anterior approach with elective vessel endoligature in a patient in decubitus position was a better and safer approach than the other techniques previously published. PATIENTS AND METHODS: Between January 1996 and December 2002, 54 children underwent laparoscopic splenectomy. We use the anterior pedicle approach and elective vessel endoligature in order to prevent persistence of any exogenous material. Sixteen children also had a concomitant cholecystectomy. Their age ranged between 1 and 18 years (mean 7.5 years). Twenty children had hereditary spherocytosis, 15 were affected by sickle cell disease, six had an idiopathic thrombocytopenia, four had beta-thalassemia, and nine another hemolytic disease. RESULTS: Mean operative time was 150 min (range, 115-230 min). Hospital stays ranged from 3 to 15 days (mean: 5.4). Seven patients had complications (four pneumonia and three deep abscess with successful antibiotic treatment). Median follow-up was 42 months (2 months-6 years) without problems regarding procedure. DISCUSSION: With this technique consisting in first elective arterial and secondary venal ligature, we preserved blood splenic sequestration and pancreatic tail lesion. The procedure is safer. For us, considering the low complication rate for this type of patient, and the advantages of a small abdominal trauma in the postoperative period, laparoscopy with anterior approach for elective splenectomy and eventual cholecystectomy in hematologic disorders is the best technique.
Assuntos
Doenças Hematológicas/cirurgia , Laparoscopia/métodos , Esplenectomia/métodos , Abscesso Abdominal/epidemiologia , Abscesso Abdominal/etiologia , Adolescente , Anemia Falciforme/cirurgia , Doenças Autoimunes/cirurgia , Criança , Pré-Escolar , Colecistectomia Laparoscópica/estatística & dados numéricos , Feminino , Doenças Hematológicas/genética , Doenças Hematológicas/imunologia , Humanos , Lactente , Laparoscopia/estatística & dados numéricos , Tempo de Internação/estatística & dados numéricos , Ligadura , Masculino , Paris , Derrame Pleural/epidemiologia , Derrame Pleural/etiologia , Complicações Pós-Operatórias/epidemiologia , Estudos Retrospectivos , Segurança , Esferocitose Hereditária/cirurgia , Esplenectomia/estatística & dados numéricos , Talassemia/cirurgia , Trombocitopenia/cirurgia , Resultado do TratamentoRESUMO
An 11-month-old boy presented with an intrahepatic foreign body after ingestion of a sewing needle. Surgical management using an exclusive laparoscopic extraction was successful.