P0815 - Multidisciplinary Management of Rectal Bleeding Due to Extensive Cavernous Hemangiomas in a Pediatric Patient With Klippel-Trenaunay Syndrome

Ghanapati V. Jambula, MD¹, Omer Usman, MD¹, Muhammad Waqas Khan, MBBS², Muhammad Usman Shahbaz, MBBS³, Muhammad Ali Hassan, MBBS⁴, Muhammad Shaheer Mannan, MD⁵, Maryam Abbas Malik, MBBS^2
1Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX; ²Services Institute of Medical Sciences, Lahore, Punjab, Pakistan; ³Jinnah Hospital, Lahore, Punjab, Pakistan; ⁴Shifa College of Medicine, Islamabad, Islamabad, Pakistan; ⁵Marshfield Medical Center, Marshfield, WI

Introduction: Klippel-Trenaunay Syndrome (KTS) is a congenital disorder with an incidence of about 1:100,000, characterized by the triad of cutaneous hemangiomas, soft tissue or bone hypertrophy, capillary and venous malformations. Gastrointestinal involvement in KTS includes bleeding(25%), varices(20%), portal hypertension(5-10%), malabsorption, and intestinal obstruction(exceedingly rare) in decreasing order of incidence. Our case highlights the strategies in management of a patient with profuse GI bleeding due to KTS.

Case Description/Methods: A 5-year-old child presented with profuse rectal bleeding since birth, accompanied by diffuse swelling and bluish discoloration of his left flank, and multiple varicose veins in the left leg. Examination revealed small hemangiomatous lesions scattered along the torso and trunk. Barium enema showed diffuse mucosal irregularity with polypoidal filling defects in the sigmoid colon and rectum. Doppler ultrasound revealed slow-flow vascular lesions in the left flank and leg without arteriovenous communication. CT scan abdomen and pelvis showed diffuse cavernous hemangiomas in the descending colon, sigmoid colon, and rectum. Histopathology demonstrated numerous dilated, irregular vascular channels in the submucosa, muscularis propria, and serosa, with calcification and hemosiderin-laden macrophages. A laparotomy was performed, revealing a grossly thickened rectum, sigmoid, and part of the descending colon with dilated vessels. A Low Anterior Resection (LAR) with partial proctocolectomy and colorectal anastomosis was undertaken. Additionally, a soft, fluctuant retroperitoneal lymphangiomatous lesion was aspirated, confirming its nature. Postop, recovery was uneventful with discharge on the fifth day. Follow-up over several months showed no recurrence of rectal bleeding, with plans for ongoing surveillance and potential endoscopic ablation if needed.

Discussion: Cavernous hemangiomas from KTS often cause life-threatening GI complications, requiring careful evaluation and treatment. A multidisciplinary approach includes gastroenterology for initial evaluation, endoscopic assessment, and therapeutic interventions like cauterization, banding, or clipping of bleeding vessels. Surgical interventions, include excision of vascular malformations, debulking surgeries, and partial bowel resection. Interventional radiologists often perform minimally invasive procedures like sclerotherapy or embolization. Long-term follow-up is essential to monitor for recurrence or residual lesions.



Disclosures:


Ghanapati V. Jambula, MD¹, Omer Usman, MD¹, Muhammad Waqas Khan, MBBS², Muhammad Usman Shahbaz, MBBS³, Muhammad Ali Hassan, MBBS⁴, Muhammad Shaheer Mannan, MD⁵, Maryam Abbas Malik, MBBS². P0815 - Multidisciplinary Management of Rectal Bleeding Due to Extensive Cavernous Hemangiomas in a Pediatric Patient With Klippel-Trenaunay Syndrome, ACG 2024 Annual Scientific Meeting Abstracts. Philadelphia, PA: American College of Gastroenterology.