Laparoscopic management of massive spontaneous external haemorrhage from the umbilical varix due to recanalisation of the paraumbilical vein in a patient with 'Child's Class A' liver cirrhosis.

Spontaneous external haemorrhage from the umbilical varix is an extremely rare complication of portal hypertension. Bleeding is usually into the peritoneal cavity and the treatment involves urgent laparotomy and ligation of the bleeding varices. We describe a cirrhotic 38-year-old man who presented with spontaneous external haemorrhage from the umbilical varix which was successfully managed laparoscopically by in-situ distal clipping and proximal transcutaneous ligation of the recanalised paraumbilical veins. We therefore feel that laparoscopy can be safely and effectively employed to control external haemorrhage from the umbilical varix associated with liver cirrhosis. This novel technique can help avoid a laparotomy and also help preserve the umbilicus.

INTRODUCTION

Spontaneous external haemorrhage from the umbilical varix is an extremely rare complication of portal hypertension.[1] Bleeding from umbilical varices is usually into the peritoneal cavity and the treatment involves urgent laparotomy and ligation of the bleeding varices.[2] The first case of external haemorrhage from an umbilical varix was reported by Douglas.[3] To the best of our knowledge, only three cases of external haemorrhage from umbilical varices have been reported till date.[4] The first two cases were successfully treated with variceal ligation and exomphalectomy. We describe a 38-year-old man who presented with spontaneous external haemorrhage from the umbilical varix which was successfully managed laparoscopically by in-situ distal clipping and proximal transcutaneous ligation of the recanalised paraumbilical veins.

CASE REPORT

A 38-year-old man presented to the emergency department with spontaneous massive external haemorrhage from the umbilicus following trivial trauma after wiping the umbilicus with a bath towel. He was a known case of alcoholic liver disease with portal hypertension categorised as Child's class A. There was no visible caput medusae or audible periumbilical venous hum. His blood pressure was 100/60 mm of Hg. Investigations revealed haemoglobin of 10.5 mg/dl, total leukocyte count - 4 500 cells/cumm, platelet count - 90 000 cells/cumm, liver function tests revealed total bilirubin - 1.2 mg/dl, direct bilirubin - 0.2 mg/dl, SGOT (Serum Glutamic Oxaloacetic Transaminase) - 112 IU/l, SGPT (Serum Glutamic Pyruvate Transaminase) - 68 IU/l, ALP (Alkaline phosphatase)- 90 IU/l, total protein - 7.8 g/dl, albumin - 3.5 g/dl, globulin - 4.3 g/dl and the prothombin time was within 1.31 seconds of control. Serological tests were negative for HBsAg and anti-HBs.

Temporary control of bleeding was achieved by applying a pressure dressing. An upper gastrointestinal endoscopy showed no gastroesophageal varices. Ultrasonography of the abdomen revealed coarse hepatomegaly but no ascites. Doppler sonography showed a portal vein diameter of 12 mm and patent, recanalised paraumbilical veins reaching the umbilicus. The patient continued to rebleed on removing the dressing the next day. Hence, he was planned on for emergency surgery.

A transverse sub-umbilical incision about 1.5 cm long was made and the umbilical cicatrix was identified. A vertical incision was made over the umbilical cicatrix and the dilated umbilical vein was identified and isolated circumferentially. The incision was then carried down (taking care not to injure the umbilical vein) and the peritoneal cavity was entered. A blunt 10-mm laparoscopic trocar was introduced under direct vision and pneumoperitoneum was thus established by an open technique. A 30 degree, 10-mm telescope was introduced to inspect the peritoneal cavity. Laparoscopy revealed a macronodular liver and minimal ascites. The dilated paraumbilical vein could be easily seen running within the falciform ligament. The umbilical vein was about 1 cm in diameter. Two more ports, 10-mm and 5-mm, were introduced in the left hypochondrium and left iliac fossa, respectively. The umbilical vein was then clipped in situ, with multiple medium/large titanium clips (LT 300 Ligaclip Extra; Ethicon Endo-Surgery, Cincinnati, OH, USA) close to the umbilicus. A few tributaries from the paraumbilical veins were also seen which were doubly clipped [Figure 1].

Figure 1

(a and b) Endoclips applied to the dilated paraumbilical veins and its tributaries (white arrows) Cirrhotic liver (black arrow)

The anterior abdominal wall was then transilluminated with the laparoscope and the paraumbilical veins running along the midline were easily identified. A 1.5-cm incision was made 3 cm below the xiphisternum and a vicryl (polyglactin 910, Ethicon. Inc, Johnson and Johnson) suture 2-0 was used to ligate these vessels in continuity. This was done transcutaneously with the help of a suture passer [Figure 2].

Figure 2

(a) Transillumination of the dilated paraumbilical veins in the falciform ligament using the scope light prior to transcutaneous ligation (white arrow). (b) Three ports used for the surgery. Epigastric incision (white arrow) used for transcutaneous ligation of the paraumbilical veins using the suture passer

Following proximal and distal control, the vein within the umbilical cicatrix had collapsed. The pneumoperitoneum was discontinued and the umbilicus was anchored back in place after closure of the defect in the rectus sheet with 1-0 vicryl suture. The operating time was one hour. Blood loss was minimal. A Doppler ultrasonography repeated a week later showed complete absence of venous flow to the umbilicus. The postoperative period was uneventful. The patient was discharged on the fifth postoperative day. The patient is on regular follow-up.

DISCUSSION

The presence of caput medusa and a loud venous hum at the umbilicus may be found in patients with chronic liver disease and is popularly known as the Cruveilhier-Baumgarten syndrome. This is due to portosystemic collateralisation between the paraumbilical vein running in the fatty falciform ligament with the periumbilical veins of the anterior abdominal wall.[5] Bleeding from these veins then occurs due to trivial trauma or increased intra-abdominal pressure such as at coughing. Our patient had large paraumbilical veins which channelled portal blood toward the umbilicus, which was clearly seen at laparoscopy. The umbilical skin was thinned out and the bleeding could be compared to that of bleeding from blowouts in case of varicose veins of the lower limbs.

Laparoscopy is now considered to be safe in patients with Child's class A and B liver disease. Laparoscopic treatment for spontaneous external bleeding from umbilical varix was probably first reported by Basu et al., where initial open ligation of the umbilical varix was unsuccessful. In our patient, the laparoscopic access was done under direct vision using the open technique, thereby avoiding injury to the vessels at the umbilicus. Additional trocars also could be thus safely placed under direct laparoscopic vision. We also ensured distal and proximal ligation of the offending veins with endoclips and suture, respectively. Accurate mapping of anterior abdominal veins could be done by transillumination technique using the scope's light prior to transcutaneous in-continuity suture ligation. The success of this procedure was demonstrated on follow-up Doppler ultrasonography, which showed complete obliteration of the paraumbilical venous flow to the umbilicus. We therefore feel that laparoscopy can be safely and effectively employed to control external haemorrhage from the umbilical varix associated with liver cirrhosis. It can help avoid a laparotomy and also help preserve the umbilicus.