Spontaneous neonatal chylothorax treated with octreotide

Chylothorax is the most common cause of pleural effusion causing respiratory distress in the neonate. Cases unresponsive to conservative management usually require surgery. We report a case of spontaneous neonatal chylothorax where surgery was avoided with the compassionate trail of octreotide. The chylothorax resolved promptly with no observed side effects. After reviewing the literature reporting the use of octreotide in the neonatal period, we found that neonates with isolated spontaneous chylothorax might be viewed as a group different from those with major cardiac malformations. They are at a lower risk for adverse effect of octreotide and lower threshold for commencement of octreotide may benefit them most.     

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.     

Congenital chylothorax: lymphopenia and high risk of neonatal infections

AIM: To describe the clinical course of patients with congenital chylothorax focusing on infectious complications. Congenital chylothorax is a common manifestation of non-immune hydrops fetalis (NIHF). The drainage of chyle leads to loss of cellular and plasmatic factors that influence the patient's immune response and increase the risk of infections. METHODS: In a retrospective analysis of 24 preterm infants with NIHF treated between 1998 and 2002, congenital chylothorax was diagnosed in 7 patients. RESULTS: All 7 patients were treated conservatively with pleural drainage over a median period of 22 d (range 10-36 d). Lymphopenia was found in all patients (median of minimal lymphocyte counts 285/microl, range 80-770). The nadir was on day 5 (2-6 d). Lymphopenia lasted for 12 d median (range 4-39 d) and was significantly correlated with the duration of lymph drainage (p = 0.001). Cell-surface analysis of peripheral blood lymphocytes was performed in two patients. Both patients had a decreased number of total T cells. Four out of seven (57%) patients developed nosocomial infections. This incidence of nosocomial infections in patients with congenital chylothorax is about three times higher than that in other neonatal patients. None of the children suffered from fungal or viral infection. Although there was a very high incidence of infections, no correlation between lymphopenia and the occurrence of infections could be shown. CONCLUSION: Drainage of congenital chylothorax results in the loss of lymphocytes and bears a high risk of infections.     

Congenital chylothorax in siblings

We describe two cases of congenital chylothorax in siblings with important differences from previously described familial cases. Our findings support the likelihood of an autosomal recessive inheritance in some cases of this condition, rather than X-linked recessive inheritance, which has also been suggested. Autopsy findings from one of these cases and others previously described suggest that the pathophysiological mechanisms involved may be variable.     

Octreotide as therapeutic option for congenital idiopathic chylothorax: a case series

Background: Octreotide, a somatostatin analogue, is used for the management of patients with refractory chylothorax although its safety and efficacy in neonates have not been evaluated in controlled clinical trials. We present one of the largest case series about the use of octreotide in congenital idiopathic chylothorax. Methods: Six cases of congenital chylothorax (CC) were prospectively collected, who were managed with same unit protocol for octreotide. Mean (SD) gestation was 34.5 (±2.2) weeks, and birthweight was 3410 (±840.4) g. All infants required chest drains from day 1 of life, and the mean (SD) duration of insertion was 36.1 (±8.5) days. Octreotide was commenced at a median age of 13.5 days (range 8–22), given for a median duration of 20 days (range 12–27). The starting dose was 0.5–1 μg/kg/h with an increment of 1–2 μg/kg/day to a maximum of 10 μg/kg/day. Resolution of chylothorax was achieved in five patients, being resistant to treatment in the sixth patient. None had adverse effects from octreotide. Full enteral feeds were reached at a mean age of 44 days. Conclusion: Early commencement of octreotide is recommended although further reports to evaluate the safety and efficacy would add to the profile of this medication in the treatment of CC.     

Congenital chylothorax and pulmonary hypertension complicated with heart failure and hepatopathy

Respiratory difficulty resulting from congenital chylothorax is usually relieved by postnatal thoracentesis, closed chest drainage, and oxygen therapy. However, early occurrence of congenital chylothorax or accumulation of a large amount of chylous fluid sometimes leads to pulmonary hypoplasia or persistent pulmonary hypertension of the newborn, which requires further customized mechanical ventilatory support. In these cases, conventional mechanical ventilation is primarily used during initial treatment and is later replaced by high‐frequency ventilation, but the advantages of inhaled nitric oxide treatment have rarely been described. This case suggests the benefits of inhaled nitric oxide in patients with congenital chylothorax, even when mechanical ventilation cannot improve respiratory distress because of severe pulmonary hypertension of the newborn leading to right cardiac dysfunction and possibly cholestasis.     

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 in a late preterm infant and successful treatment with octreotide

Chylothorax is defined as abnormal accumulation of lymphatic fluid in the pleural space and is a rare condition in neonates. Chylothorax causes respiratory and nutritional problems and a significant mortality rate. Octreotide is a long-acting somatostatin analog that can reduce lymphatic fluid production and has been used as a new strategy in the treatment of chylothorax. Here, we report a premature baby with severe bilateral pleural effusion diagnosed by prenatal ultrasound and subsequently confirmed to be congenital chylothorax after birth. This newborn baby was initially treated with bilateral chest tube insertion to relieve severe respiratory distress. However, the chylothorax recurred after a medium-chain-triglyceride-enriched formula was initiated. The accumulation of chylothorax diminished after the administration of octreotide. Therefore, octreotide may allow the patient to avoid invasive procedures, such as reinsertion of chest tubes or surgery.     

Congenital hypothyroidism and neonatal withdrawal syndrome

We describe a neonate born of drug dependent parents. This observation documents the variability of expression in the neonatal abstinence syndrome and the interaction with an additional disease.     

A Case of Lipoid Congenital Adrenal Hyperplasia Presenting with Cholestasis

Background

Lipoid congenital adrenal hyperplasia, is the rarest and usually the most severe form of adrenal steroidogenic defect,which may presents as infantile cholestasis.

Case Presentation

Here we present a 45 days old infant who came to our attention with cholestasis and severe intractable vomiting and electrolyte disturbances. Evaluation resulted in diagnosis of congenital adrenal hyperplasia. Hydrocortisone and flodrocortisone improved the symptoms including jaundice and vomiting. Hyponatremia and hyperkalemia also resolved with above mentioned treatment.

Conclusion

Congenital adrenal hyperplasia as one of the causes of neonatal cholestasis should be kept in mind, whenever there are also electrolytes abnormalities.     

Congenital chylothorax

Congenital chylothorax (CC) results from multiple lymphatic vessel anomalies or thoracic cavity defects and may accompany other congenital anomalies. Fetal chylothorax may increase the risk of death and complications from pleural space lymphatic fluid accumulation, which compromises lung development, pulmonary, and cardiovascular function and from complications arising from the loss of drained lymphatic contents. Prenatal interventions might improve survival in severe cases of fetal chylothorax. The neonatal treatment strategy is generally supportive with interventions that include thoracostomy drainage and attempts to decrease chyle flow using a stepwise approach that begins with the least invasive means. Evidence-based treatment choices are lacking and are much needed. Most cases of CC resolve with time even without specific lymphatic system studies to identify the exact pathology. Expertise in performing lymphatic studies is not universally available. Data on both efficacy and safety of the various therapeutic options are needed to determine the best approach to the treatment of CC.     

Octreotide in the treatment of congenital chylothorax

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 paediatric 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. Treatment was associated with prompt respiratory improvement prior to cessation of pleural drainage. Further studies are required to ascertain its true value in congenital chylothorax.     

Noonan syndrome with spontaneous chylothorax at birth

Abstract A female Chinese infant having the features of Noonan syndrome presented with acute respiratory distress at birth, secondary to a large spontaneous left-sided chylothorax. This condition must be recognized early in such infants, as prompt thoracentesis is life-saving.     

应用高分子绷带与Pavlik挽具治疗新生儿先天性膝关节脱位

目的介绍利用高分子绷带与Pavlik挽具治疗新生儿先天性膝关节脱位的方法。方法2004年6月～2005年6月收治6例(8膝)新生儿先天性膝关节脱位,其中4例(5髋)并有发育性髋关节脱位,均采用闭合复位、阶段性高分子绷带固定与Pavlik挽具治疗。结果6例(8膝)膝关节过伸消失、屈曲功能完全恢复,平均屈曲120°。结论本法对新生儿先天性膝关节脱位,尤其是合并有发育性髋关节脱位的患儿,疗效满意。     

允许性高碳酸血症在先天性重症膈疝中的作用

目的 探讨允许性高碳酸血症这一通气策略在先天性重症膈疝治疗中的意义.方法 回顾本院1985年1月到2007年5月的33例重症膈疝患儿,按通气方法分两个阶段,第一阶段14例,平均气道峰压PIP≥30cm H2O,尽可能保证较低二氧化碳分压(PaCO2)及较高的pH值.第二阶段19例,术前患儿采用允许性高碳酸血症的通气策略:控制PIP≤22cm H2O,保证导管前SaO2≥80%,PaCO2≤60mmHg,对导管后氧饱和度放宽标准,如果患儿导管前SaO2<80%或PaCO2>60mmHg,则应用高频通气,以出院患儿生存情况作为评价指标.结果 两个阶段患儿胎龄、体重、疾病的严重程度差异无统计学意义(P>0.05),第一阶段患儿生存率50%,第二阶段为84.2%;气道峰压,第二阶段较第一阶段明显降低,两者差异有统计学意义(P<0.05);第一阶段术前有2例发生气胸,术后患儿死亡,第二阶段则无类似气道损伤发生.延期手术/急诊手术组生存率为77.2%/65.4%(P>0.05).11例患儿进行了高频通气.8例患儿吸入NO,仅短时提高SaO2,但肺动脉高压未缓解.结论 允许性高碳酸血症配合低压高频通气可以明显减少医源性气道损伤,提高重症膈疝存活率.     

Theraphy of neonatal chylothorax

A case of neonatal chylothorax following closure of a left-sided diaphragmatic defect and our therapeutic approach to this entity are presented. Conservative therapy with total parenteral nutrition and drainage is sufficient in most cases. Fluid and protein losses should be balanced exactly; immunoglobulin losses are compensated by early substitution. Early surgical revision, which carries an especially high risk in patients with a diaphragmatic defect or hernia, can thus be avoided in the immediate postoperative period. Offprint requests to: S. Kellnar