Laparoscopic management of the congenital chylous ascites in a newborn: Case report

Abstract

Congenital chylous ascites (CCA) is a rare condition seen in the neonatal period. The pathogenesis is primarily related to congenital intestinal lymphangiectasis. Conservative treatment of chylous ascites involves paracentesis, total parenteral nutrition (TPN), medium-chain triglyceride (MCT)-based milk formula, use of somatostatin analogue and octreotide. Surgical treatment is considered when conservative treatment fails. We describe a laparoscopic treatment of CCA using the fibrin glue technique. A male infant, in whom foetal ascites was detected at 19 weeks of gestation, was born by caesarean section at 35 weeks of gestation weighing 3760 g. There was evidence of hydrops in the foetal scan. A diagnosis of chylous ascites was made by abdominal paracentesis. A magnetic resonance scan was suggestive of gross ascites, and no lymphatic malformation was identified. TPN and octreotide infusion was started and continued for 4 weeks, but the ascites persisted. The failure of conservative treatment led us to perform laparoscopic exploration. Intraoperatively, chylous ascites and multiple prominent lymphatic vessels around the root of the mesentery were noted. The fibrin glue was applied over the leaking mesenteric lymphatic vessels in the duodenopancreatic region. Oral feeding was started from post-operative day 7. After 2 weeks of the MCT formula, ascites progressed. Thus, laparoscopic exploration was necessary. We introduced an endoscopic applicator for fibrin glue and applied it into the place of leakage. The patient was doing well with no appearance of ascites reaccumulating and was discharged on the 45^th post-operative day. Follow-up ultrasonography (1^st, 3^rd and 9^th months after discharge) showed a small amount of ascitic fluid but with no clinical significance. Laparoscopic localisation and ligation of leakage sites could be difficult, especially in newborns and young infants due to the small size of lymphatic vessels. The use of fibrin glue to seal the lymphatic vessels is quite promising.

INTRODUCTION

Congenital chylous ascites (CCA) is a rare condition defined as the accumulation of chyle into the peritoneal cavity in infants younger than 3 months.[1] It is mostly caused by malformation of the intra-abdominal lymphatic system. Many other pathological conditions can result in this disease, including malignant neoplasms, infections, liver cirrhosis, blunt abdominal trauma and surgery injury (iatrogenic injury).[2] Management usually consists of medium-chain triglyceride (MCT)-based diet or total parenteral nutrition (TPN), and the addition of octreotide, which is a somatostatin analogue and has been reported to be immensely useful. Octreotide is known to decrease gastric, pancreatic and intestinal secretions and to reduce the splanchnic blood flow, which may contribute to decrease lymph flow. Surgical exploration is reserved for those cases, in whom conservative management has failed.[3] Here, we describe a case of a newborn with CCA successfully treated laparoscopically with the use of fibrin glue.

CASE REPORT

A male infant, in whom foetal ascites was detected at 19 weeks of gestation, was born by caesarean section at 35 weeks of gestation weighing 3760 g. There was evidence of hydrops in foetal scan. His Apgar scores were 9 at 1 min and 9 at 10 min. Around 30 min after birth, the neonate was noted to have tachypnoea with groan and cyanosis. On examination, he presented with intensive ascites that progressed to severe respiratory distress. Oxygen therapy and empirical antibiotics were administered. On the day of life (DOL) 1, the infant presented with significantly increased work of breathing and ascites. A biochemical examination revealed the following: total protein, 47.5 g/L; albumin, 21.6 g/L and white cell count, 4.06 × 109 cells/L with 18.5% lymphocytes. Serum electrolytes and liver and renal functions were normal. The abdominal US depicted normal findings other than the presence of massive ascites. Abdominal paracentesis was done, which suggested chylous ascites. The milky fluid had triglyceride levels of 2.05 mmol/L, sugar 4.4 mmol/L, protein 26.3 g/l and albumin 14.7 g/L, peritoneal fluid cultures were positive for congenital CMV infection. An MCT-based diet along with octreotide infusion at 5–20 μg/kg/h was started and continued for 4 weeks, but the ascites persisted. A magnetic resonance (MR) scan was suggestive of gross ascites, and no lymphatic malformation/cyst was identified [Figure 1]. Repeated paracenteses were mandatory, and a considerable amount of fresh-frozen plasma was given. The failure of conservative treatment led us to perform laparoscopic exploration on the 29^nd DOL. On diagnostic laparoscopy, ascitic fluid was seen [Figure 2]. Multiple prominent lymphatic vessels [Figure 3] were noted at the base of the mesentery in the ileocaecal region over which fibrin glue was applied [Figure 4]. Oral feeding was started from post-operative day 7. The patient presented after 2 weeks of surgery with re-accumulation of the ascitic fluid again. Thus, laparoscopic re-exploration was done, whereas the lymph leakage appeared under the mesocolon transversum, in the region of the third part of the duodenum. We introduced an endoscopic applicator for fibrin glue and applied it into the place of leakage. The patient was doing well with no appearance of ascites reaccumulating and was discharged on the 45^th post-operative day. Follow-up US (1^st, 3^rd and 9^th months after discharge) showed a small amount of ascitic fluid, but with no clinical significance.

Figure 1.

MR scan was suggestive of gross ascites, and no lymphatic malformation/cyst was identified. MR: Magnetic resonance

Figure 2.

Chylous ascites during diagnostic laparoscopy accumulated in bursa omentalis

Figure 3.

Multiple prominent lymphatic vessels around the root of the mesentery

Figure 4.

Fibrin glue application over the leaking mesenteric lymphatic vessel

DISCUSSION

CCA is a relatively rare entity, accounting for only 4% of cases of neonatal ascites, and it is primarily related to congenital abnormalities of the lymphatics. The reported incidence of CCA is reported to be 1:20 000–1:187 000 live births.[2] Although the incidence of chylous ascites has increased in recent years, the treatment remains unsatisfactory in some cases because of the prolonged duration of disease.[4] The majority of cases occur due to lymphatic malformation, which can be atresia or stenosis of lymphatics, lymphangiomatosis and chylous cysts.[3] About 30% of cases are idiopathic, in which ‘leaky or seeping lymphatics’ due to delayed maturation of lacteals lead to CCA.[4] Secondary chylous ascites may be caused by extensive abdominal surgery, neoplasia, inflammation, trauma or iatrogenic injuries.[5] Apart from iatrogenic injuries that are usually a consequence of surgeries with the opening of the abdominal cavity, there are also non-accidental injuries (battered-child syndrome), with newborns and young children being at particular risk.[6] The lymphatics obstruction could be also caused by external compression, as in incarcerated hernia, malrotation, intussusception, inflammatory enlargement of the lymph nodes and malignancy may also lead to chylous ascites.[7] Neonates with CCA usually present with abdominal distension with or without respiratory distress. Due to the constant loss of proteins and lymphocytes, chylous ascites has other dreadful complications too, which are related to mechanical, nutritional and immunological factors.[3] US of the abdomen is the initial step that confirms the presence of ascites. Analysis of the fluid obtained by abdominal paracentesis is the most useful diagnostic method. The ascitic fluid known as chyle is usually color free or milky white.[8] US, computed tomography or MR imaging are the diagnostic modalities for the determination of the underlying cause of chylous ascites. Lymphangiography still remains the ‘golden standard’ in visualisation of the lymphatic system, although it is technically difficult to carry out and connected with a high risk in newborns and infants.[9] The treatment of CCA is primarily conservative and is intended to decrease the lymph flow, allowing the gut to rest and decreasing the intestinal secretions. Conservative management includes TPN and enteral feeding with a formula high in MCT, low in long-chain triglycerides and enhanced in protein content. In refractory cases, the administration of somatostatin or its analogue octreotide is needed. However, these treatment modalities usually take many weeks to be effective.[10] Surgery is only recommended when conservative treatment fails. Kuroiwa et al. demonstrated that 58% of children with intractable chylous ascites have a lesion amenable to surgical intervention.[11] Surgical approach, consisting of resection of the macroscopic localised anomaly or ligation of the leaking lymphatic vessels, is considered if 1–2 months of conservative treatment fails.[2] Management strategy should be based on clinical judgment of the amount and consequences of lymph loss. Mitsunaga et al. suggested that the aim of surgical exploration is to locate and close the lymph fistula. To facilitate the challenging and uncertain task of identifying the source of leakage, administration of fatty meal and pre-operative or intraoperative injection of lipophilic dye.[12] Zeidan et al. described an experience with fibrin glue application in six children who underwent surgical treatment for primary or secondary chylous ascites refractory to conservative measures. Fibrin glue application during surgical exploration has been proposed to control lymph leakage.[5] Saxena et al. demonstrated a case report of an 8-year-old male with refractory chylous ascites successfully treated by fibrin glue, which was applied at the base of the mesentery in the ileocaecal region.[3] Recently, localisation of leaking point and lymphatic duct ligation could be performed under laparoscopic guidance with minimal invasiveness. Laparoscopy may have the advantage of exploring the peritoneal cavity in its natural state, in addition, to have a magnified image of the abdominal cavity.[12]

CONCLUSION

CCA is an uncommon pathology. It can be suspected early with antenatal US, and the diagnosis is confirmed postnatally by analysis of the ascitic fluid. Although most of the cases are managed conservatively, surgery may be required for refractory cases. The localisation of leakage sites is difficult, especially in newborns. Laparoscopic exploration and, subsequently, the use of fibrin glue to seal the lymphatics seems promising, especially in cases where one cannot identify any specific site of leakage.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.