Surgical Emergencies in Intestinal Venous Malformations
19 April 2017
14 August 2017
25 September 2017 (eFirst)
Background Venous malformations (VMs) can occur in any part of the body; however, the gastrointestinal tract is a frequent location. These are usually asymptomatic, thus, representing a challenge to diagnosis. Intestinal location of VMs can be associated with severe complications that ultimately require an emergency surgery. Our aim was to analyze all patients with an intestinal VM with special focus on those who required emergency surgery.
Materials and Methods A retrospective study of patients presenting complication caused by intestinal VM was performed. Clinical records, associated anomalies, physical findings, and treatment were assessed.
Results Twenty-one patients had a diagnosis of intestinal VM, 16 (76%) were associated to blue rubber bleb nevus syndrome (BRBNS) and 5 (24%) were isolated. Only four (19%) of the total cases presented an episode of acute abdomen with hemodynamic instability that required an emergency surgery. Findings included two gastrointestinal bleedings, one volvulus, and one intussusception of small bowel. All patients underwent an uneventful recovery and are presently doing well.
Conclusion Intestinal VM can be challenging to diagnose in emergency situations, such as gastrointestinal situation or acute abdomen. The complications associated with it must be kept in mind, regardless of its low incidence.
- 1 Gordon FH, Watkinson A, Hodgson H. Vascular malformations of the gastrointestinal tract. Best Pract Res ClinGastroenterol 2001; 15 (01) 41-58
- 2 Dasgupta R, Fishman SJ. Management of visceral vascular anomalies. SeminPediatrSurg 2014; 23 (04) 216-220
- 3 de la Torre L, Carrasco D, Mora MA, Ramírez J, López S. Vascular malformations of the colon in children. J Pediatr Surg 2002; 37 (12) 1754-1757
- 4 Montero Reyes I, Martinez Cardet LF, Gonzalez Fernández S. , et al. Malformación venosa de íleon terminal. Rev CubanaPediatr 2006 ;4(78). Available at http://scielo.sld.cu/scielo.php?script=sci_arttext&pid=S0034-75312006000400009&lng=en
- 5 Emil S, Milanchi S, Goldstein A. Cecal vascular malformation mimicking appendicitis in a child. PediatrSurgInt 2005; 21 (09) 723-724
- 6 Sugito K, Kusafuka T, Hoshino M. , et al. Usefulness of color doppler sonography and 99m Tc-RBC scintigraphy for preoperative diagnosis of a venous malformation of the small intestine in a 2-year-old child. J Clin Ultrasound 2008; 36 (01) 56-58
- 7 Lal H, Neyaz Z, Singh RK, Mohindra S. Vascular malformation of the jejunum presenting as obscure gastrointestinal haemorrhage: detection with multidetector CT angiography. Singapore Med J 2010; 51 (06) e103-e106
- 8 Ninomiya IS, Steimberg C, Udaquiola J. , et al. Intestinal venous vascular malformation: unusual etiology of gastrointestinal bleeding in pediatrics. Case report [Article in Spanish]. Arch Argent Pediatr 2016; 114 (03) e159-e162
- 9 Schwalb G, Cocca A, Attie M, Basack N, Aversa L. Malformaciones vasculares en pediatría. Hematologia 2013; 17 (01) 55-59
- 10 Wang Y, Zhao X, You X. Blue rubber bleb nevus syndrome coexisted with intestinal intussusception: a case report. Pan Afr Med J 2014; 17: 212
- 11 Ramírez M, López Gutiérrez JC, Diaz M. , et al. Bean or blue rubber blue nevus syndrome. Presentation of 6 patients [Article in Spanish]. Cir Pediatr 2010; 23 (04) 241-244
- 12 Dubois J, Rypens F, Garel L, Yazbeck S, Therasse E, Soulez G. Pediatric gastrointestinal vascular anomalies: imaging and therapeutic issues. PediatrRadiol 2007; 37 (06) 566-574
- 13 Li Z, Yuan H, Yu D. , et al. The surgery for blue rubber bleb nevus syndrome. Ann Med Surg (Lond) 2015; 5: 93-96
- 14 Hasosah MY, Abdul-Wahab AA, Bin-Yahab SA. , et al. Blue rubber bleb nevus syndrome: extensive small bowel vascular lesions responsible for gastrointestinal bleeding. J Paediatr Child Health 2010; 46 (1–2): 63-65