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Review
. 2020 Jul 10;99(28):e21199.
doi: 10.1097/MD.0000000000021199.

Blue rubber bleb nevus syndrome with the complication of intussusception: A case report and literature review

Zeming Hu  1   2 , Xuan Lin  1 , Jianing Zhong  2 , Qingfang He  1 , Qin Peng  3 , Jianbo Xiao  1   2 , Bin Chen  1 , Jianhong Zhang  1
Affiliations
Review

Blue rubber bleb nevus syndrome with the complication of intussusception: A case report and literature review

Zeming Hu et al. Medicine (Baltimore). .

Abstract

Rationale: Blue rubber bleb nevus syndrome (BRBNS) is an extremely rare disorder characterized by multifocal venous malformations involving various organs such as the skin and gastrointestinal tract. Severe complications of BRBNS, such as intussusception, volvulus, and intestinal infarction are rarer and require surgery. This report describes a 33-year-old male of BRBNS complicated with intussusception that was successfully diagnosed and treated with surgery.

Patient concerns: A 33-year-old Chinese man presented with persistent, colicky pain accompanied by nausea, abdominal distension, and dizziness. The patient presented with sporadic bluish nodules on his skin involving his head, neck, thorax, abdomen, and planta pedis.

Diagnoses: BRBNS with the complication of intussusception.

Interventions: An emergency laparotomy was performed, and postoperative management included blood transfusions and oral iron supplementation for 2 weeks.

Outcomes: The patient's postoperative course of hospitalization was uneventful. During the 4-month follow-up, the patient showed no signs of intussusception recurrence.

Lessons: Patients diagnosed with BRBNS who present with acute abdominal pain and distension should raise suspicion for the presence of intussusception, which requires emergent surgical intervention.

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Conflict of interest statement

The authors have no conflicts of interest to declare.

Figures

Figure 1
Figure 1
Representative images of skin lesions. Red arrows indicate multiple bluish nodules involving in the head and neck (A), thorax (B), abdomen (C,), and planta pedis (D).
Figure 2
Figure 2
Images of abdominal and pelvic contrast-enhanced computed tomography (CT). (A) Probable hemangiomas or vascular malformations involving in the liver. (B–D) Typical signs of sleeve sheath and concentric circles revealed the presence of intussusception.
Figure 3
Figure 3
Histologic examination of intestinal lesions showed multiple thrombosed cavernous hemangiomas, which further confirmed the diagnosis of blue rubber bleb nevus syndrome.
See this image and copyright information in PMC

References

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    1. Martinez CA, Rodrigues MR, Sato DT, et al. Blue rubber bleb nevus syndrome as a cause of lower digestive bleeding. Case Rep Surg 2014;2014:683684. - PMC - PubMed
    1. El Bakkaly A, Ettayebi F, Oubeja H, et al. Bean's syndrome in children: about two cases. Pan Afr Med J 2017;28:102. - PMC - PubMed
    1. Tang X, Gao J, Yang X, et al. A 10-year delayed diagnosis of blue rubber bleb nevus syndrome characterized by refractory iron-deficiency anemia: a case report and literature review. Medicine (Baltimore) 2018;97:e10873. - PMC - PubMed
    1. Terata M, Kikuchi A, Kanasugi T, et al. Association of blue rubber bleb nevus syndrome and placenta previa: report of a case. J Clin Ultrasound 2013;41:517–20. - PubMed

Supplementary concepts