{
  "abstract": "Obscure gastrointestinal (GI) bleeding may cause refractory anaemia and require repeated endoscopic evaluations. In addition, this may require iron supplementation or blood transfusion if the fall is precipitous.GI bleeding in children with previous abdominal surgery can be associated with anastomotic ulcers and may require endoscopic intervention. We describe the case of a 10 -year-old boy, who had DBD (donation after brain death) liver transplant for cryptogenic liver disease at 2 years of age. He was subsequently diagnosed with Niemann-Pick type C at 6 years of age. He presented with multiple episodes of melena, over the course of a 7-year period, requiring numerous endoscopies to delineate the cause.Blue rubber bleb nevus syndrome (BRBNS) is a rare vascular disorder characterized by multiple venous malformations primarily affecting the skin and gastrointestinal (GI) tract. The lesions can affect the distal small bowel and present with either obvious melena or occult blood loss, resulting in refractory anaemia.1 Pharmacological therapy includes somatostatin analogues like Octreotide in significant GI bleeding. Sirolimus (angiogenesis inhibitor) has been used as a therapeutic option with variable success.2 The patient had multiple endoscopic assessments including upper GI endoscopy, ileocolonoscopy and wireless capsule endoscopy (WCE). The first endoscopic assessment was normal. A relook endoscopy identified an angioectatic lesion in the second part of duodenum to which argon plasma coagulation (APC) was applied. Double balloon enteroscopy (DBE) was planned during a precipitous episode, with a significant drop in haemoglobin of 3.5 g/dl. Multiple angioectatic lesions were identified close to the Roux-en-Y anastomosis; however, haemostasis could not be achieved using clips or thermal coagulation. The patient underwent laparotomy with resection and re-fashion of Roux-en-Y anastomosis. An on the table, lap-assisted enteroscopy did not identify any other lesions. The histology of the bleeding area of the resected segment showed multiple dilated vascular channels in the submucosa, appearing to be venous in nature. There has been no further bleeding since the operative intervention in a 2 year follow up.There are no guidelines for the management of BRBNS with GI involvement. The treatment is determined by the severity of the disease and the extent of intestinal involvement. Therapeutic options include interventional endoscopy, surgery, and angiogenesis inhibitors.Successful treatment is reported in 92% of cases. Surgical intervention is curative once the disease site is identified. Disease recurrence is known in 25% of treated patients.3 Sirolimus has proven to be effective as a second line of treatment, but there are possible limiting side effects (bone marrow suppression with increased risk of infections, toxicity, vascular complications, hyperlipidaemia, high blood pressure, abnormal renal function, impaired wound healing, mouth ulcers, rashes, arthralgia, diabetes, malignancy).Our case study suggests that the diagnosis of BRBNS lesions can be challenging, especially in children with previous abdominal surgery and without any cutaneous stigmata. DBE during an acute episode can be useful to localise the lesion and treat it using haemostatic clips, injections, diathermy probes. Surgical help maybe needed in more extensive lesions or when endoscopic intervention is difficult or not possible.References Isoldi S, Belsha D, Yeop I, et al. Diagnosis and management of children with blue rubber bleb nevus syndrome: a multi-center case series. Dig Liver Dis. 2019;51:1537–46.Rimondi A, Sorge A, Murino A, et al. Treatment options for gastrointestinal bleeding blue rubber bleb nevus syndrome: systematic review. Dig Endoscopy. 2024;36:162–171.O’Kelly F, Lim KT, Ravi N, et al. The value of double balloon enteroscopy in diagnosing blue rubber bleb naevus syndrome: a case report. Cases J. 2010;18(3):29.Duran Y, Celik SK, Aksoy B, et al. Double balloon enteroscopy experience in children from Turkey. J Pediatr Gastroenterol Nutr. 2018;66:276.Aravindan U, Ganesan R, Thamarai KM. Surgery for blue rubber bleb nevus syndrome: case report. The Indian Journal of Surgery 2018;80(3):272–274.Duong JT, Geddis A, Carlberg K, et al. Sirolimus for management of GI bleeding in blue rubber bleb nevus syndrome: a case series. Pediatr Blood Cancer. 2022;69:e29970.",
  "authors": [
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Rayna Alamurova"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Natalia Nedelkopoulou"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Priya Narula"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Prithviraj Rao"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Arun Urs"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Shishu Sharma"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Dominique Schluckebier"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Zuzana Londt"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Mike Thomson"
    },
    {
      "affiliations": [
        "Sheffield Children’s Hospital"
      ],
      "name": "Akshay Kapoor"
    }
  ],
  "title": "OC94 Obscure gastrointestinal bleeding in a child post liver transplant: a case report",
  "uid": "8d305ded-bced-508a-a522-a70395c71d11"
}