MULTIPLE INFANTILE HEPATIC HAEMANGIOMA WITH KASABACH-MERRITT SYNDROME PRESENTING WITH RAISED INTRABDOMINAL PRESSURE...

Infantile hepatic haemangioma is a common benign vascular malformation in the liver, however, its association with Kasabach-Merritt syndrome, raised intrabdominal pressure and intestinal obstruction is rare. We report this case of syndromic infantile hepatic haemangioma to raise awareness for its suspicion in this unusual presentation and the role of propranolol in the management.

We report a case of a 3-month-old female who presented with abdominal swelling, difficulty in breathing, constipation, bilateral inguinal swellings and protrusion of the rectal mucosa. There were multiple cutaneous vascular lesions on the palms, chest and abdomen. Examination under mild sedation revealed multiple kernel-like nodules in the enlarged liver. She had thrombocytopenia and a computerised tomographic scan of the abdomen showed multiple rim enhancing hyperdense vascular lesions involving the entire liver. She was placed on oral propranolol with regression of the lesion and resolution of symptoms.

Multiple Hepatic haemangiomas with Kasabach-Merritt syndrome can present with raised intrabdominal pressure and features of intestinal obstruction. This possibility should be considered particularly in infants also presenting with cutaneous vascular conditions. Conservative care with propranolol is an effective treatment option.