非创伤性乳糜胸的电视胸腔镜外科治疗(附4例报告及文献复习)

目的通过4例非创伤性乳糜胸的电视胸腔镜治疗，结合文献探讨非创伤性乳糜胸的病因、诊断和治疗方法。方法4例经临床、实验室、影像学及核医学检查确诊的非创伤性乳糜胸，经电视胸腔镜行胸导管结扎及胸膜固定术。结果3例1次治愈，1例复发，经2次手术治愈，随访1～5年未复发。结论电视胸腔镜外科为非创伤性乳糜胸提供了一种安全、微创及有效的方法。     

新生儿乳糜胸1例并文献复习

新生儿乳糜胸是新生儿期较罕见疾病,患儿出生早期即可出现呼吸衰竭,并因丢失大量淋巴液导致感染、营养不良、免疫低下等并发症而危及生命。本文通过一例病例报告及文献复习,就新生儿乳糜胸的临床表现、实验室检查、非增强MRI应用、以及保守治疗方法做一总结分析,以提高儿科临床医生在本病的诊断和治疗方面的认识。     

Congenital pulmonary lymphangiectasis with chylothorax: A heterogeneous lymphatic vessel abnormality

We report on 7 perinatal autopsy cases of primary congenital pulmonary lymphangiectasis (CPL) with bilateral chylothorax. This study demonstrates that primary CPL is often complicated by chylous pleural effusions with ensuing pulmonary hypoplasia. Conversely, CPL appears to be a constant pathological finding in spontaneous congenital chylothorax. These observations indicate a common pathogenesis for both disorders. The basic defects is not an intrinsic lung abnormality, but a developmental error of the lymphatic system resulting in a pulmonary lymphatic obstruction sequence. The cause of CPL is heterogeneous. Apparently, most cases are sporadic occurrences. We report the second instance of CPL in sibs. This indicates that some cases are genetically determined with autosomal recessive inheritance. CPL may also be part of a multiple congenital anomalies (MCA) syndrome such as Noonan, Ullrich-Turner, and Down syndrome. © 1993 Wiley-Liss, Inc.     

Congenital chylothorax in a patient with 21 trisomy syndrome

A female infant with 21 trisomy syndrome associated with congenital chylothorax was reported. She was born at a gestational age of 34 weeks by Cesarean section because of fetal hydrothorax and hydrops fetus, confirmed by ultrasonography at 32 weeks. Emergent resuscitation and immediate thoracentesis were performed soon after birth. After beginning breast feeding, the serous pleural fluid became opalescent and a diagnosis of congenital chylothorax was made. Feeding was changed to medium-chain triglyceride (MCT) feeding and the production of pleural effusion disappeared after thoracentesis was performed several times. Accumulating evidence suggested that MCT feeding and intermittent thoracentesis under echo guide were effective. Some reports on patients, including this one, suggest that there may be more patients with 21 trisomy associated with congenital hydrothorax. Therefore, congenital hydrothorax might be listed as a complication of 21 trisomy.     

Congenital chylothorax treated with octreotide

Congenital chylothorax is the accumulation of lymphatic fluid within the pleural space. Cases unresponsive to conservative management usually require surgery. Octreotide has been used successfully to treat post-traumatic chylothoraces in the pediatric and adult population. Its exact mode of action is uncertain but it is believed to reduce lymphatic drainage by a direct action on splanchnic lymph flow. We report a case of congenital chylothorax where surgery was avoided with the compassionate trial of the somatostatin analogue, octreotide. A 33 week gestation female infant, born with the presence of large bilateral pleural effusion, was unresponsive to conservative management. Octreotide was commenced on day 15, with 10 days of an octreotide infusion, initially 0.5 μg/kg per hour and increased daily by 1 ug/kg per hour to 10 μg/kg per hour. Treatment was associated with prompt respiratory improvement prior to cessation of pleural drainage over the 10 day. She remains well at 6 months of age. Further studies are required to ascertain its true value in congenital chylothorax.     

Disorders of the lymph circulation: their relevance to anaesthesia and intensive care

The lymphatic system is known to perform three major functionsin the body: drainage of excess interstitial fluid and proteinsback to the systemic circulation; regulation of immune responsesby both cellular and humoral mechanisms; and absorption of lipidsfrom the intestine. Lymphatic disorders are seen following malignancy,congenital malformations, thoracic and abdominal surgery, trauma,and infectious diseases. They can occasionally cause mortality,and frequently morbidity and cosmetic disfiguration. Many lymphaticdisorders are encountered in the operating theatre and criticalcare settings. Disorders of the lymphatic circulation relevantto anaesthesia and intensive care medicine are discussed inthis review. Br J Anaesth 2003; 91: 265–72     

Chylothorax occurring 23 years post-irradiation: literature review and management strategies

Chylothorax is an uncommon condition rarely seen outside the clinical scenario of trauma or malignancy, and management may be difficult. We report the case of a 52-year-old man with a right chylothorax occurring 23 years after mantle irradiation for Hodgkin's disease. In addition, a Medline literature search was performed. Chylothorax occurring post-irradiation is rare and the aetiology is uncertain. Recurrent malignancy must be excluded. There are a number of management options and our patient was successfully treated with a combination of thoracocentesis, medium chain triglyceride diet and thoracoscopy with talc pleurodesis. There was no evidence of recurrent chylothorax at 2 years follow up. Prompt assessment and treatment is required. Surgical measures need to be considered early if medical treatment is unsuccessful.     

Congenital chylothorax: from foetal life to adolescence

Aim: To analyse the main prenatal and postnatal features of congenital chylothorax (CC), and the outcome including mid‐term follow‐up. Methods: We searched our databases for CC diagnosed between 1990 and 2006. Data of 29 cases were retrieved and analysed. Follow‐up until 3 years of age was available for all patients. Results: Most patients were diagnosed prenatally (94%) and most cases were complicated by foetal hydrops (66.7%). The overall survival rate at 3 years was 56%. A significantly poorer outcome was observed when foetal hydrops, preterm birth < 34 weeks, large effusions and/or early‐onset pneumothorax were present. An important but not significant improvement in the survival rate was observed through the study period; while in 1990–1998, the survival rate was 41.7%, from 1999 to 2006 it was 66.7% (p = 0.19). In the mid‐term follow‐up, we did not observe any recurrence of CC and most infants remain asymptomatic. However, 27% of survivors were diagnosed as having asthma in early infancy. Conclusion: CC still carries a significant risk of perinatal mortality. However, continuous advances in foetal and neonatal medicine are improving the prognosis of these patients, and nowadays most of them are likely to survive. Beyond the neonatal period, most survivors have an uneventful outcome.     

乳糜胸的诊断和治疗(3例罕见病例报告)

自1952～1985年收治自发性乳糜胸2例,外伤性乳糜胸1例。文献报道自发性乳糜胸发病率约占同期乳糜胸总数的3.8％,外伤性乳糜胸占1.9％。自发性乳糜胸的治疗原则为首先采用保守疗法,经两周治疗无效者应改为手术治疗。外伤性乳糜胸的治疗原则为每日引流量1000ml以上观察5天或每日引流量500ml以下观察10天不见好转即行手术治疗。目前经胸结扎胸导管已被广泛接受,对顽固性乳糜胸可考虑用滑石粉促进胸膜粘连固定和胸腹腔转流术。