Idiopathic Chylothorax in Infants-Is Octreotide Alone Enough ?

Address for correspondence: Dr. Mahesh Maralihalli E-mail: drmaheshmaralihalli@gmail.com 1Dr. Mahesh B Maralihalli, MBBS, MD, Assistant Professor, 2Dr. Mahantesh R Matti, MBBS, MD, 3Dr. Suhas N Joshi, MBBS, MD. All from Department of Paediatrics, SDM College of Medical Sciences and Hospital Sattur, Dharwar Karnataka. 4Dr. Venkatesh M Annigeri MBBS, MS, MCh. Department of Paediatric Surgery, SDM College of Medical Sciences and Hospital Sattur, Dharwar Karnataka, India. Abstract


Introduction
C hylothorax occurs due to accumula on of lympha c fl uid in the pleural cavity.It can occur spontaneously in the neonates or due to developmental anomalies of lympha c, or following injury to thoracic duct or lympha c vessels.In most cases its diffi cult to establish e ology, par cularly with congenital lesions.Loss of lympha c fl uid into pleural cavity can cause nutri onal, electrolyte, and immunologic complica ons.Diagnosis is typically established by its clinical and biochemical characteris cs including triglycerides content of 1.1 mmol/L or more and a total cell count of 1000 cells/ microlitre or more with 80% or more lymphocytes 1 .There are no specifi c guidelines about the treatment for idiopathic congenital chylothorax.Most of the children respond to conserva ve measures in non-iatrogenic cases.Most advice, adequate drainage of the pleural fl uid, along with changes in enteral feeds.Recently Octreo de has been used successfully in treatment of congenital chylothorax in few cases.Here we present a fi ve month baby who responded to only three doses of Octreo de alone.

The Case
Five month old female baby was referred to us with a chest x-ray showing right sided pleural eff usion, that was taken during an episode of acute illness.Baby was delivered by cesarean sec on, cried a er birth.Infant was gaining weight adequately.On admission, baby had stable vitals.Her weight and height were normal for age.There were no pallor, edema, hepatosplenomegaly, icterus, lymphadenopathy.

General physical examina on was normal.
There was decreased air entry in right lung fi eld.Other systems were normal.There were no dysmorphic features.Her blood counts, ESR, C-reac ve protein, electrolytes, urea and crea nine were normal.CT chest (Figure 1) showed gross right pleural eff usion with par al collapse of right lung, medias nal shi towards le side.
Pleural tap fl uid was milky, opalescent on appearance.Pleural fl uid analysis showed predominant lymphocytes with occasional mesothelial cells, Triglycerides 5154 mg%, proteins 5.6 gm%, sugar 212 mg% sugges ve of chylous eff usion, ZN stain was nega ve for Acid fast bacilli, pleural fl uid culture was sterile.Octreo de was given as intravenous infusion at dosage of 100 μg/kg over 10 hours (10μg/kg/ hour) once a day.Baby's vitals, blood sugar, cardiac monitoring was done.A er three days of treatment chest X ray (Figure 2 ) was taken, showing complete clearance pleural eff usion.A chest X ray was taken a er 15 days of follow up, was normal.

Discussion
Congenital or primary chylothorax is the most common type of pleural eff usion seen in fetuses and newborns, 50% of chylothoraces are present at birth or appear during the fi rst week of life.It may be associated with Down syndrome, Noonan or Turner syndrome.Other e ologies are classifi ed as trauma c and non trauma c 2 .Trauma c causes include Noniatrogenic (childbirth, blunt trauma to chest) and iatrogenic causes (thoracic surgery, head and neck surgery).Non-trauma c causes include malignancy, diseases (Tuberculosis, Benign tumor) and Idiopathic.Most cases of neonatal chylothorax occur without clear e ology, termed as idiopathic congenital chylothorax.
This infant was incidentally found to have pleural eff usion.Diagnos c pleural tap showed chylous fl uid.CT chest showed gross right side pleural eff usion.There was no lymphadenopathy, le lung was normal.Inves ga ons showed no evidence to the possible e ology.Lymphangiography could not be done due to non availability at our centre.
Op mal treatment guidelines remain debatable.Conserva ve treatment includes use of a low-fat diet supplemented with medium chain triglycerides or total parenteral nutri on (TPN).This op on requires placement of pleural drain.Surgical interven on is warranted if pleural drainage persists beyond 2 to 5 weeks of TPN.Surgical op ons are thoracic duct liga on, pleuroperitonial shun ng, pleurectomy, or pleurodesis 1 .Few case reports have been published about the successful use of Octreo de, a somatosta n anologue in congenital or postopera ve chylothorax in children 3,4 .Octreo de may be given subcutaneously (20-70 μg/kg/day, TDS), or as an intravenous infusion (1-10μg/kg/hr).The dura on of therapy depends on the reduc on in pleural drain volume 5 .Octreo de therapy dura ons were longer while trea ng congenital chylothoraces than in postopera ve cases, ranging from 3 to 29 days 3 .Octreo de is believed to act by decreasing lympha c fl ow through a reduc on in gastric, intes nal, and pancrea c secre ons or by a decrease in hepa c venous pressure and splanchnic blood fl ow.Octreo de is used for the treatment of growth hormone producing tumors, diarrhea and fl ushing episodes associated with carcinoid syndrome and diarrhea associated with vasoac ve intes nal pep de secre ng tumors.Adverse eff ects are headache, cardiac conduc on changes, vomi ng, hyper or hypoglycemia.This infant had no respiratory distress and was on exclusive breast feeding.Pleural drain was not placed.There was complete clearance of pleural eff usion a er three doses of Octreo de.We believe this is one of the few cases where in only Octreo de has been used without pleural drain to treat idiopathic asymp toma c chylothorax and complete clearance of eff usion as early as with three doses only.But more studies and evidences are required before selec on of cases for Octreo de alone therapy.

Conclusion
There are no op mal guidelines available for treatment of idiopathic chylothorax in infants.Few cases have been treated with Octreo de a er surgical drainage.But in our case, only Octreo de was used to treat and complete clearance of the pleural eff usion was documented as early as 3days only.We believe that this is one of the very few cases wherein only Octreo de has been used to treat idiopathic chylothorax and to document complete clearance of chylothorax within three days only.

Fig 2 :
Fig 2: Chest X-ray taken a er three doses of Octreo de therapy showing resolu on of eff usion.

Fig 1 :
Fig 1: CT Chest of the baby showing right sided pleural eff usion