Case Reports
Saturday, October 12, 2024
11:30 AM–01:00 PM
Abstract
Introduction: Hennekam Syndrome (HS) is a rare genetic disorder characterized by maldevelopment of the lymphatic system, leading to intestinal lymphangiectasia (IL). Symptom management involves supportive care, but is often insufficient. Octreotide is a somatostatin analogue known to decrease splanchnic blood flow in cases of severe gastrointestinal bleeds. It is postulated subcutaneous octreotide may decrease IL-related ascites, preventing hospitalization. There are no published case reports regarding the use of octreotide in pediatric patients for this indication.
Case: A 3-year-old girl with HS and monthly hospitalizations for past 4 months, presented with dyspnea, abdominal girth of 103cm (baseline: mid-60’s) and 14.3kg weight gain. Peritoneal drain output was 4,200mL within 12 hours. Drain output from Day 2-6 was consistently 1,000ml/24 hours, replaced with albumin at 0.5mL per 1mL output. Subcutaneous octreotide was initiated on Day 7 at 70mcg/kg/day. On Day 8, drain output decreased to 85ml, but the patient complained of stomach pain. Octreotide was decreased to 18mcg/kg/day with a goal titration to 45mcg/kg/day. On Day 10, the patient’s drain output remained negligible and the drain was clamped. There was no reaccumulation of peritoneal fluid and the patient was discharged on Day 14 on subcutaneous octreotide. The patient did not require readmission for peritoneal ascites management for the next 10 months.
Discussion: Symptom management is key for reducing life-threatening edema in patients with HS. Although the exact mechanism of how octreotide reduces lymphatic output is unknown, octreotide inhibits gastrointestinal vasoactive proteins, reduces intestinal absorption of fat and stimulates the autonomic nervous system. In our case, octreotide significantly reduced peritoneal drain output after administration of only two doses. Adverse effects reported were minimal and resolved with daily dose reduction.
Conclusion: Subcutaneous octreotide administration at doses between 20-70mcg/kg/day may reduce the need for hospitalization for peritoneal fluid drainage in patients with HS who fail traditional supportive care measures.
Presenting Author
Courtney Kain Pharm.D., BCPPSNemours Children's Health
Authors
Michael Cellucci MD
Nemours Children's Health