Refractory congenital chylous ascites: First report of fibrin glue and mesh application by laparoscopy.

Chylous ascites (CA) is a form of ascites having leakage of lipid-rich lymph into the peritoneal cavity, due to damage or obstruction in the lymphatic system. Aetiology of CA could be congenital or acquired. Primary lymphatic hypoplasia is seen commonly in children and presents with lymphoedema, chylothorax or CA. CA is initially treated conservatively with the aim to provide gut rest and decrease intestinal secretions. Surgical treatment is recommended if 1-2 months of conservative approach fails. The success of the operation depends on identifying the site of leakage of the lymphatic duct. Surgical options are ligation of leaking lymphatics, peritoneo-venous shunt, laparotomy and fibrin glue. Laparoscopy has been used for diagnosis but not for glue and mesh application in congenital CA where the lymphatic leak is unidentified. We present here the first experience of laparoscopic fibrin glue and mesh application in congenital CA with successful outcomes.

INTRODUCTION

Chylous ascites (CA) is a form of ascites having leakage of lipid-rich lymph into the peritoneal cavity, due to damage or obstruction in the lymphatic system. Aetiology of CA could be congenital or acquired due to cirrhosis, tuberculosis, filiariasis or postsurgical. Idiopathic causes having leaky lymphatics account for 30% of cases of congenital CA. Ninety percent of cases respond to medical treatment by strict gut rest and somatostatin analogue, but refractory cases need surgical intervention. Cases of congenital CA where no definite leak is identified are difficult to treat. Case reports of using laparoscopy for exploration and diagnosis followed by laparotomy for fibrin glue/mesh application have been reported,[12] but the total laparoscopic approach for glue and mesh application in congenital CA has not been reported. We present our first experience with complete laparoscopic approach for fibrin glue and mesh application with successful outcomes for refractory congenital CA.

CASE REPORT

A 7-month-old, full-term, female child presented to us with massive abdominal distension, inguinal hernia and oozing of fluid from tense protruding umbilical hernia [Figure 1a]. At birth, she had mild distension which increased after feeding. Ascitic tap showed milky-white fluid with the presence of chylomicrons, triglycerides and cholesterol. Child was on medium-chain triglycerides (Monogen) diet since then and had undergone medical treatment with six times paracentesis, twice strict gut rest for 4 weeks with total parenteral nutrition (TPN) and octreotide infusion. Massive ascites developed on the resumption of feeds. The child presented to us at 7 months of age when laparoscopy was planned. Magnetic resonance (MR) lymphangiography could not identify the definite lymphatic leak and cisterna chyle. Six hours before surgery fat-rich milk with Sudan dye was given but no definite lymphatic leak was identified during surgery. On laparoscopy, retroperitoneum was exposed [Figure 1b]. Kocherisation of the duodenum was done to dissect at inferior vena cava (IVC), aorta and renal artery for lumbar lymphatics. Few thin lymphatics were identified around IVC and aorta, but no leak was appreciated [Figure 1c]. Lesser sac was opened to dissect at left gastric artery, splenic artery, hepatic artery and right crus of diaphragm and aorta, to apply glue and mesh at the location of cisterna chyle and celiac axis [Figure 2a]. Fibrin glue was applied and strips of vicryl mesh were put over the entire exposed retroperitoneum [Figure 2b and c]. Vicryl was also fixed by sutures to retroperitoneal tissues. After surgery child was kept on gut rest and octreotide infusion at 3ug/kg/h for 2 weeks and then normal feeds were resumed. In follow-up of 3 years, the child is growing well on normal diet with no ascites on ultrasonography.

Figure 1

(a) Child with congenital chylous ascites, massive distension and tense umblical hernia. (b) Complete retroperitoneum exposed. Right kidney, inferior vena cava and aorta visualised. (c) Suction tip at inferior vena cava, fine lymphatics (white arrow), Aorta (black arrow)

Figure 2

(a) Hepatic artery (arrow 1), left gastric artery/vein (arrow 2), splenic artery (arrow 3), right crus of diaphragm (arrow 4). (b) Glue instillation. (c) Mesh application to cover retroperitoneum

DISCUSSION

Primary lymphatic hypoplasia is seen commonly in children and presents with lymphoedema, chylothorax or CA. Primary treatment is medical management which requires to keep patient strictly nil by mouth, somatostatin/octreotide infusion and TPN for 4–6 weeks.

If CA persists for more than 10 weeks of bowel rest, surgery is the option. MR lymphangiography may help in identifying lymphatic leaks that can be ligated or macroscopic localised anomaly which can be resected.[3] In cases where no lesion or leak is identified peritoneo-venous shunt or glue/mesh application are the options. Peritoneo-venous shunt has complications like perforation and shunt block.[3] Successful outcome has been reported with glue and mesh application in cases of unidentified leaks. Laparoscopy has been used successfully for ligation of lymphatics in cases of the identifiable leak.[4] but where no leaks are identified glue and mesh application has been done by converting to laparotomy.[12]

Cisterna chyle is located at the T11-L2 vertebra anteriorly, between the aorta and right crus of the diaphragm. Intestinal trunk ascends on left of descending aorta, superior to the left renal artery, crosses second lumbar vertebra anteriorly and joins left or right lumbar trunk to form common trunk, which extends to cisterna chili or thoracic duct.[5] Wide variation in formation of trunks and location of cisterna chyli is known. Sites of the expected leak are the root of mesentery, celiac trunk, right and left lumbar lymphatics and cisternal chyli. Hence, glue and mesh application in retroperitoneum is recommended in these areas in case of unidentified leaks for successful outcomes.

Retroperitoneal dissection in these areas is feasible with laparoscopic magnification and spacious distended abdomen. Thorough instillation of glue and mesh application in these recommended areas is important to achieve good seals and successful outcomes in cases with unidentified leaks.

Declaration of patient consent

The authors certify that they have obtained allappropriate patient consent forms. In the form the patient (s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in thejournal. The patients understand that their names and initial s will not be published and due efforts will bemade to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.