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FURTHER HISTORY

To rule out generalized lymphangiectasia, our patient had an extensive gastro-enterology and cardiac work-up. Endoscopy and biopsy were normal. Our patient also had a normal barium swallow. Cardiac echo showed a normal anatomy, large vessel connections, left ventricular function, no pericardial effusion, and right ventricular pressures of 40-50 mm Hg. This right sided hypertension was felt to be secondary to the patient's chronic lung disease.

LYMPHANGIECTASIA
The characteristic radiographic findings of lymphangiectasia are: hyperinflation and interstitial infiltration. The disease course includes frequent upper airway infections. The patient's symptoms tend to improve over time. Long term cases of survival are sporadic. The treatment for lymphangiectasia is similar to that of chylothorax. Initially, a conservative approach of a low fat, high protein diet with MCT's and vitamin supplementation is implemented.

FURTHER HISTORY

Unfortunately, our patient suffered a significant number of complications. These were attributed to both the prolonged chylothorax, as well as the multiple invasive treatments. The patient was re-admitted to the PICU with respiratory decompensation. A diagnosis of chylothorax tamponade was made. Our patient had a secondary ischemic gut and a perforated transverse colon (Clostridoium difficile).

One month later, our patient was extubated. Feeding studies at that time showed significant aspiration and our patient was discharged with a colostomy and G-tube feeds.

Lung Transplantation was considered for our patient. Post lung transplantation, lymphatic drainage can be re-established in the transplanted lung in approximately 2 - 4 weeks. However, no cases of transplantation for lymphangiectasia have been completed. Our patient was denied transplantaqtion as an option secondary to his multiple associated problems in addition to small size (6.6 Kg). At discharge, our patient was taking furosemide, ranitidine, cisapride, vitamins ADEK, Aldactazide, Pamidronate, Portagen + NaCl + KCl.

Since discharge, our patient has had no significant respiratory problems or admissions. Follow-up chest CT scan (6 months post discharge) Should I have an image for here, I don't have a CT scan? showed: chronic pleural thickening, bilateral lower lobe bronchiectasis, thickening of interlobular septa and clearing of previous interstitial infiltrate

Future Therapy: Pleuroperitoneal Shunt (12) (13)
Pleuroperitoneal shunts have been used for 15 patients (19 chylous effusions). The patients age ranged from 1 month to 11.5 years. The most common cause of chylothorax was post operative congenital heart disease (7/15). The duration of the chylothorax pre-shunt ranged from 5 to 810 days (Mean 76days, Median 14 days). Of the 17 pleural chylothoraces treated with shunts, 14 resolved in an average of 95 days. Of the failures, all were attributed to elevated right side cardiac pressures. Within this group, there were 6 shunt failures and 2 shunt infections.

Future Therapies: Prednisone in Chylothorax (14)
There have been some reports of prednisone use for the treatment of chylothorax. The biological rationale is as follows: With protein loss in the chylothorax,, there is a decreased quantity of protein in tissue. Therefore, prednisone increased protein synthesis in the liver. This increases plasma oncotic pressure and fluid shifts from the interstitium and lymph into blood. In a case report, an eighteen-month patient with Noonan syndrome (without lymphangiectasia) developed a chylothorax. Despite previous treatment with a thoracic duct ligation, tetracycline pleurodesis and pleurectomy, the patient's chylothorax persisted. Prednisone was started at 1g/kg BID for 1 month and weaned over the subsequent 2 months. The patient had complete resolution and no recurrence at 8 months follow-up.

Two other cases of prednisone have been reported -- both patients were post-operative Fontan patients.

Future Therapies: Intrapartum Drainage of Pleural Effusions (15)
Within the literature, 74 total cases of intrapartum pleural drainage have been reported. Either Pleuro-Amniotic Shunt or intrapartum thoracentesis can accomplish this. With a pleuro-amniotic shunt, the fetus does not need repeated thoracentesis for repeated pleural effusions. However, for a shunt, the mother must be of an early gestational age and not at high risk for imminent delivery. Complications include shunt blockage or dislocation.

Intrapartum thoracentesis has been reported within 4 patients in a case series. All patients with intrapartum thoracentesis were eventually intubated, ventilated, and had chest tubes. The authors suggest that the risk for performing a thoracentesis increased the time to chest tube, allowing for time to stabilize the newborn. Within the case series, 2 deaths were reported (aspiration, fulminant sepsis).

COMMENT

For patient with chylothorax, rather than trying conservative management with diet (MCT's), it may be advantageous to go straight to nothing by mouth and TPN. The rationale for this is that diet (even MCT's) can stimulate chyle production and thus, delay the resolution of the chylothorax. Given the long-term complications of prolonged chylous leakages0 +.-3, it would be in the patients' best interest to stop the leak and then try dietary treatment to prevent recurrence.


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