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Idiopathic congenital chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space and is a relatively rare condition. It is a cause of progressive respiratory distress with nutritional and immunological consequences. Treatment of congenital chylothorax has been conservative management and cases unresponsive usually require surgery. We report a case of idiopathic congenital chylothorax treated with octreotide (a somatostatin analogue), avoiding surgery after failed conservative medical treatment. The patient promptly improved after initiation of endovenous octreotide treatment (10 µg/kg/h) with no observed side effects.
Chylothorax is defined as an abnormal accumulation of lymphatic fluid within the pleural space due the disruption of the thoracic duct or its lymphatic tributaries. Chylothoraces may be either acquired or congenital.1,2 Congenital chylothoraces may be associated with abnormalities of the lymphatic system such as lymphangiomatosis and lymphangiectasia, abnormal karyotpe or additional congenital anomalies.3 Independent of the aetiology, chylothorax leads to impairment of respiratory, nutritional and immunological functions.4 An overall mortality rate from 20–60% has been reported depending upon associated findings, gestational age and the duration and severity of the pleural effusion.4
The diagnosis of chylothorax is established via fluid analysis and includes a triglyceride content of 100 mg/dl or more and a total cell count of 1000 cells/µl or more with 80% or more lymphocytes.2 Upon recognition of chylothorax, conservative treatment is indicated,5 although the timing of spontaneous resolution is variable and the prolonged chyle loss leads to lymphocytes, protein and immune globulins loss, placing the patient at risk for infection, malnutrition and anasarca.6 Surgical intervention is recommended if medical treatment has failed, although surgical interventions are not entirely risk free and are not always successful.5,7–9
Recent literature has suggested that octreotide (a long-acting synthetic analogue of endogenous somatostatin) might provide an alternative medical treatment to infants with persistent chylothorax.10–14 We report a neonate with congenital chylothorax successfully treated with continuous administration of octreotide after unresponsive conservative management.
A 381 kg male neonate delivered at 38 2/7 weeks gestation via a scheduled caesarean section for suspected macrosomia and previous caesarean. There was a previous history of a caesarean delivery that resulted in a stillborn with lymphoedema with no other significant maternal/parental medical history. Apgar scores were 10 at 1 min and 10 at 5 min without hydropic or dysmorphic features at clinical examination. On day 2, the neonate developed acute respiratory distress requiring supplemental oxygen.
Blood samples for cultures, group B Streptococcus and serological evaluation for TORCH (toxoplasmosis, other, rubella, cytomegalovirus, herpes simplex virus) group were carried out and antibiotics were started. Chest x-ray (figure 1) and the ultrasound revealed a right basilar pleural effusion without any cardiac or abdominal structural abnormalities. Diagnostic thoracentesis was performed on the right side. Approximately 37 ml of cloudy yellowish fluid was recovered. Fluid analysis had characteristic findings of chylothorax with 225 mg/dl of triglycerides, with negative bacterial cultures and no malignant cells. Rapid re-accumulation of pleural effusion mandated placement of a chest tube. Blood cultures and serological evaluation were negative.
Enteral feeding with breast milk was replaced by total parenteral nutrition. Daily pleural effusion ranged from 50–200 ml. After 14 days of conservative management, empirical intravenous infusion of octreotide 3.5 µg/kg/h daily was started. Seventy-two hours after starting octreotide, the neonate had acute respiratory distress due to blocked chest drain, which was corrected. Cautiously, we increased the daily dose to a maximum dose 10 µg/kg/h daily. As recommended, we monitored closely for the evidence of glucose tolerance, liver and renal impairment, and evidence of cholelithiasis. No side effects were noted. Chest drainage markedly decreased coincidently with total cessation of chest tube output on day 24. The chest tube was removed on day 28 and octreotide infusion was discontinued 2 days later (figure 2).
After an additional observation period with progressive enteral feeding nearing total oral intake with a medium-chain triglyceride formula, the baby began to pass liquid and semi-liquid stools in a context of rotavirus gastroenteritis. The infant was discharged on day 37 with resolution of the chylothorax.
Idiopathic congenital chylothorax is a rare condition2 and is usually a transient condition that resolves by cessation of lymphatic flow in the thorax. Management is predominantly conservative and, in resistant cases, pleurodesis, ligation of the thoracic duct or placement of pleuroperitoneal shunts may be considered. However, the timing of surgery is not uniformly defined and is not risk-free of complications.5,7,8
Some case reports have been described regarding the use of octreotide as an alternative treatment to infants with persistent chylothorax.10–14 Octreotide is an octopeptide analogue of somatostatin with a longer half-life.6 Although the exact mechanism of action of octreotide is not understood, it may act on somatostatin receptors in the splanchnic circulation, and decrease lymph fluid production through a reduction in gastric, intestinal and pancreatic secretions or by a decrease in hepatic venous pressure and splanchnic blood flow.14 Octreotide is prescribed as an anti-secretory agent and has been used in the treatment of postoperative chylothorax in paediatric patients requiring cardiac surgery10 with no reports of recurrent chyle accumulation or significant adverse effects of treatment.12
The chylothorax in our case resolved completely, with prompt resolution of the effusion coincidental to the use of octreotide, with no adverse effect encountered. These findings are similar to other case reports of octreotide use in congenital chylothorax management10–14 and highlights that the treatment can be stopped soon after resolution of chylothorax with achievement of full enteral feeds. Besides the apparent safety of octreotide treatment, it is important to insure the adequate monitoring of its side-effects.15
In conclusion, octreotide appears to offer a very attractive and effective alternative for congenital chylothorax. As case numbers are small, multicentric controlled trials are required in order to substantiate these anecdotal results and to ascertain the optimal therapeutic schema and further guidelines in congenital chylothorax.
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.