Acta Pædiatrica ISSN 0803–5253

REVIEW ARTICLE

Therapeutic management of neonatal chylous ascites: report of a case and

review of the literature
Belma Saygili Karagol ([email protected])1, Aysegul Zenciroglu1, Selim Gokce2, Ahmet Afsin Kundak1, Mehmet Sah Ipek1
1.Sami Ulus Maternity, Childrens’ Education and Research Hospital, Division of Neonatology, Ankara, Turkey
2.Sami Ulus Maternity, Childrens’ Education and Research Hospital, Division of Pediatric Gastroenterology and Nutrition, Ankara, Turkey

Keywords ABSTRACT
Chylous ascites, Lymphatic leakage, Medium-chain
triglycerides, Neonate, Octreotide Congenital chylous ascites is a rare condition seen in the neonatal period and the data on
pathogenesis and treatment modalities are limited. In this article, we report a case of neo-
Correspondence
Belma Saygili Karagol, Mesa Koru sitesi Mimoza A nate with chylous ascites and review the therapeutic management procedures on chylous
blok No:14, 06830 Çayyolu ⁄ Ankara, Turkey. ascites in childhood. We present our experience in the diagnosis and treatment of this
Tel: +90 506 8935398 | condition.
Fax: +90 312 2402600 |
Email: belmakaragol@ yahoo.com Conclusion: Medium-chain triglycerides (MCT)-based diet can be tried as a first option in
Received chylous ascites treatment. In resistant or unresponsive cases, somatostatin along with TPN can have
27 January 2010; revised 10 March 2010; use in closing the lymphatic leakage or relieving the symptoms effectively and rapidly. Conventional
accepted 29 March 2010.
regimens including enteral feeding with MCT-based formula can then be re-administered as a
DOI:10.1111/j.1651-2227.2010.01818.x maintenance treatment after reduction of lymph flow with the use of total parenteral nutrition (TPN)
and somatostatin infusion combination. Patient-specific approach should be attempted for chylous
ascites caused by various disorders and started as soon as possible.

INTRODUCTION yielded the milky ascitic fluid with a raised WBC of

Chylous ascites is the extravasation of the chyle into the 18 300 ⁄ mm3 (98% lymphocytes) and high triglyceride
peritoneal cavity and a rare clinical entity in the neonatal 11 431(N:30–100) mg ⁄ dL confirming the diagnosis of a
period. It is predominantly idiopathic in aetiology and pre- chylous ascites. Further tests were performed. There was no
sents a therapeutic challenge. Congenital chylous ascites is evidence of congenital TORCH infections, obstruction or
primarily related to inadequate lymph drainage as a result of malrotation on an upper gastrointestinal study, and negative
maldevelopment of the intra-abdominal lymphatic duct (1– work up for metabolic disorders. Abdominal computerized
4). A case of neonate with chylous ascites is described and tomography (CT) and karyotype and chromosomal screen-
therapeutic management procedures on chylous ascites in ing also revealed no abnormality.
childhood are reviewed. A treatment algorithm is proposed. The infant was initially fed with 50% medium-chain tri-
glycerides (MCT)-based formula (Peptijunior; Nutricia
advanced medical nutrition, Cuijk, Holland) and octreotide
CASE REPORT (a somatostatin analogue) was started with a dose of
A 20-day-old male baby was referred to Neonatal Intensive 4 lg ⁄ kg ⁄ h. Every day, abdominal girth was measured by the
Care Unit (NICU) of our hospital because of abdominal dis- same physician and abdominal paracentesis was performed
tension. He was born full term with a birth weight 4000 g by once a week. However, on the 10th day after admission,
caesarean section and the immediate post-natal course was abdominal distension and girth did not decrease and the
unremarkable. No evident dysmorphic features were noted infant was put on nil per os and total parenteral nutrition
on physical examination. Auscultation of the heart and was started. Octreotide dose was increased over to
lungs was muted. The abdomen was massively distended 8 lg ⁄ kg ⁄ h. Abdominal girth decreased to 37.4 cm and tri-
and dull to percussion. The abdominal girth was 40 cm in glyceride level in ascitic fluid ceased to 3848 mg ⁄ dL on day
admission to NICU. Vital functions were stable. Ultrasono- 15. By day 25, there was a gradual but definite decrease in
gram showed massive ascites; the liver and spleen appeared the abdominal girth to 36 cm and triglyceride level of ascites
to be normal and no pleural effusion was found. Echocardi- was 1195 mg ⁄ dL. 99m Tc-dextran lymphoscintigraphy
ography revealed no cardiac abnormalities. Laboratory revealed no leakage of chyle into the peritoneal cavity and
investigation included a normal blood count as white blood on day 40, the octreotide treatment was stopped. Abdomi-
cells (WBC): 6400 ⁄ mm3 with 65% lymphocytes, PLT: nal girth measurement and triglyceride level of ascitic fluid
346 000 ⁄ mm3 and Hb: 12.1 g ⁄ dL. Serum electrolytes, total were 35 cm and 144 mg ⁄ dL respectively. Enteral feeding
protein and albumin levels, liver and renal functions and with 50% MCT-based formula was re-introduced after
coagulation studies were normal. Abdominal paracentesis fasting for 4 weeks and full enteral feeding was established

ª2010 The Author(s)/Journal Compilation ª2010 Foundation Acta Pædiatrica/Acta Pædiatrica 2010 99, pp. 1307–1310 1307
Therapeutic management of neonatal chylous ascites Karagol et al.

over the next 2 weeks. Repeated ultrasonogram revealed no within the intestinal cell and thus bypass the enteric lym-
ascites in the abdomen and the patient was discharged phatics and directly enter the portal system. It is believed
shortly thereafter. that the reduction in dietary long-chain fats (LCT) reduces
On day 15 of discharge, at the age of two and a half lymphatic flow and pressure within the lymphatic system
months old; the baby was rehospitalized with an abdominal and decreases the amount of lymph leakage. In a series of
girth of 42 cm as the ascitic fluid re-accumulated. He was 103 Japanese patients, 63.5% were cured by enteral feeding
exclusively breastfed at home. Vital functions were stable, with a formula high in MCT, low in LCT and enhanced in
however, the abdomen was again massively distended. After protein content and repeated abdominal paracentesis (32).
reconfirmation of the diagnosis chylous ascites, a 50% Although these treatment modalities are usually successful,
MCT-based formula plus fat-free formula (Basic F ) enteral feedings have been shown to increase thoracic duct
enriched with vitamins, minerals and trace elements were lymph flow (33). In addition, prolonged use of low-LCT
restarted. The ascites gradually resolved clinically and radio- infant formula has been associated with poor neurological
logically by 2 weeks. The patient did well and was development, possibly from fatty acid deficiency, therefore
discharged at 3 months of age. At the age of 6 months, it should be limited to 3–4 months (19,29,34). Liao et al.
complementary feeding was added to diet. Follow-up study reported one case of neonatal chylous ascites that was put
showed no recurrence of the ascites with an abdominal girth on a strict MCT formula. The abdominal girth continued to
of 41 cm and a weight of 8490 g. The patient continued to increase, however, and warranted re-admission at 8 months
grow and develop while on normal diet on 8 months of age. of age (30).
For severe or complicated chylous ascites or chylous asci-
tes that persists after a maximum of 10 weeks of diet, TPN
DISCUSSION has been a successful option as it reduces the rate of chyle
Congenital chylous ascites is a rare condition seen in the flow. Nevertheless, there are only a few reported paediatric
neonatal period and the data on pathogenesis and treatment cases of chylous ascites in which TPN was the primary ther-
modalities are limited. The treatment of the underlying apeutic regimen (35,36). Mechanism of resolution following
cause of chylous ascites is of pivotal importance in manag- TPN is not fully known but may involve a maturation of the
ing patients with chylous ascites. Chylous ascites may be the lymphatic system over the time course of the TPN. It is also
result of the intra-abdominal ‘leaky-lymphatics’ (5), congen- possible that a leak in the lymphatic system would be
ital atresia and maldevelopment of the lymphatic vessels repaired during the treatment period. Routine conservative
(6,7), intestinal malrotation (8,9), obstructive lesions treatment, using TPN only or combined with an MCT diet,
(10,11), trauma (12,13), nonspecific bacterial, parasitic and needed 2–6 weeks to cure 60–100% cases (18,37). How-
tuberculous peritoneal infection (14), liver cirrhosis (15), ever, its major drawbacks are the risks associated with the
malignancy (16) and surgical injury (14,15,17,18). In con- use of central venous lines and the risk of diffuse atrophy of
trast, abdominal malignancy is the major cause in adults, the gut.
while in children, congenital lymphatic abnormalities are Despite several definite complications, repeated para-
more common (19). The pathogenesis of the congenital chy- centesis is commonly included in the conservative treat-
lous ascites is poorly understood and genetic factors are ment regimens to relieve respiratory insufficiency and
assumed to be involved (20,21). Consanguinity is a common abdominal distention (38). Some studies suggest prolonged
feature and the male infants are more liable to this condi- nonoperative management, avoiding paracentesis if possi-
tion (22,23). Cases of chylous ascites involving twins have ble (14).
been also reported (24,25). Surgical intervention was recommended if 1–2 months of
Paracentesis is not only diagnostic but also therapeutic conservative approach failed. Successful surgical treatment
method in the management of chylous ascites (26,27). The of congenital chylous ascites by resecting the macroscopi-
chyle is usually colour free; however, its appearance and cally localized anomaly or by ligation of an identifiable lym-
composition are not constant and depend on multiple fac- phatic leak, has been described in approximately 80% of
tors such as the size of fat particles, cellular content and diet patients (26,39). The success of the operation depended on
(20,28). Although lymphangiography is the gold standard in identifying the site of leakage of the lymphatic duct (32,40).
defining the cause of the lymphatic obstruction, lympho- If no leak can be identified, the area around the root of the
scintigraphy is also usable to evaluate the patency of the mesenteric vessels should be closely inspected after mobili-
lymphatic vessels. Lymphoscintigraphy of our patient zation of the colon, duodenum and the head of the pan-
revealed no leakage of chyle in the peritoneal cavity. This creas, and haemostasis should be performed by multiple
might be because of late imagination time of the lymphatics. ligations (41). A peritoneovenous shunt, either the Leveen
As the contrast medium for lymphoscintigraphy screening or Denver type, has been also reported to be successful at
could not have been found on current time, the lymphatic least temporarily, in children in whom repeated attempts of
vessel of our patient could have been evaluated on day 40. medical or surgical approach have failed (41,42).
Dietary management is an important treatment modality Somatostatin analogues have been demonstrated to be
in chylous ascites. MCT-based diet is accepted as the first effective in reducing lymphorrhea and may be proposed
measure to implement for reducing the chyle production in prior to consider the surgical approach. There have been
the peritoneal fluid (19,29–31). MCTs are not re-esterified reports of successful use of somatostatin in neonatal

1308 ª2010 The Author(s)/Journal Compilation ª2010 Foundation Acta Pædiatrica/Acta Pædiatrica 2010 99, pp. 1307–1310
Karagol et al. Therapeutic management of neonatal chylous ascites

chylothorax (43,44), chylous ascites after liver transplanta-

MCT
tion (15), Kasai portoenterostomy (45) or chylous ascites
caused by various disorders (46,47). The exact mechanisms
Treatment failure
of somatostatin on drying lymphatic flow are not completely
understood. It has been previously shown to decrease the
Somatostatin+TPN
intestinal absorption of fats, lower triglyceride concentra-
tion in the thoracic duct and attenuate lymph flow in the Maintenance treatment Treatment failure
major lymphatic channels (48). Satisfactory results were
achieved by the administration of the somatostatin com- MCT Surgery
bined with TPN (15,39,48).
In the reported case, enteral 50% MCT-based formula Figure 3 Treatment algorithm for the neonatal chylous ascites.
and abdominal paracentesis did not have any significant
effect in the acute period of chylous ascites. As the abdomi-
nal distension and girth had not decreased and the clinical
condition of the patient became worse, 50% MCT-based became stable. No ascites was seen upon abdominal ultra-
formula was stopped on the 10th day after admission. Total sonogram on follow-up examination.
parenteral nutrition combined with intravenous infusion of In conclusion, MCT-based diet can be tried as a first
octreotide provided satisfactory relief soon after. A signifi- option. In resistant or unresponsive cases, somatostatin
cant reduction of chylous ascites was observed after the use along with TPN can have use in closing the lymphatic leak-
of TPN and octreotide infusion combination. Abdominal age or relieving the symptoms effectively and rapidly. Con-
girth returned to normal measure within 20 days and ventional regimens including enteral feeding with MCT-
decrease of triglyceride levels of the chylous ascites was based formula can then be re-administered as a mainte-
observed by weekly repeated paracentesis (Figs 1 and 2). nance treatment after reduction of lymph flow with the use
None of the reported side effects of somatostatin were noted of TPN and somatostatin infusion combination (Fig. 3).
in our patient. After the first discharge, as the patient had Patient-specific approach should be attempted for chylous
been breastfed at home or lymphatic leakage had continued ascites caused by various disorders and started as soon as
yet at that time, the chylous ascites re-accumulated. There- possible. Further studies involving more patients with chy-
after, conservative treatment with MCT-based formula plus lous ascites are needed for exact speculation of the treat-
fat-free formula had been the primary treatment to reduce ment algorithm.
lymph flow on this patient. Ascites was gradually resolved
throughout the entire treatment course and abdominal girth
CONFLICT OF INTEREST
The authors report no conflicts of interest. The authors
alone are responsible for the content and writing of the
article.
Abdominal girth (cm)

44
42
40
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