Ehlers-Danlos tipo IV con complicaciones vasculares precoces. Descripción de un caso y revisión bibliográfica

Jiménez, Ricardo Pavón; Miñano-Pérez, J.A.; Bercial-Arias, J.; González-Gutiérrez, M.A.; Morant-Gimeno, F.; Seminario-Noguera, I.; Arcos, A. Moreno-De; Sansegundo-Romero, E.

doi:10.1016/s0003-3170(04)74903-2

Cited by 3 publications

(1 citation statement)

References 17 publications

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“…We submit this case as a response to Soonawalla et al [ 1 ] as our case suggests that in such patients, a trial of conservative management instead of emergent surgical intervention may represent a reasonable clinical approach on an ad hoc basis. The operative and post-operative mortality rate for surgical intervention is high in Type-IV Ehlers-Danlos Syndrome, [ 6 - 8 ] and invasive interventions should be considered as a last resort.…”

Section: Discussionmentioning

confidence: 99%

Type IV Ehlers-Danlos Syndrome: A Surgical Emergency? A Case of Massive Retroperitoneal Hemorrhage

Chun

Pedro

et al. 2011

TOCMJ

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Retroperitoneal hemorrhagic bleeding is a known manifestation of Type-IV Ehlers-Danlos Syndrome that is caused by loss-of-function mutations of the pro-alpha-1 chains of type III pro-collagen (COL3A1) resulting in vascular fragility. A number of previous reports describe futile surgical intervention for retroperitoneal bleeding in Type-IV Ehlers-Danlos Syndrome with high post-operative mortality, although the rarity of retroperitoneal bleeding associated with Type-IV Ehlers-Danlos Syndrome precludes an evidence-based approach to clinical management. We report a 23-year-old male with history of Type-IV Ehlers-Danlos Syndrome who presented with severe abdominal pain and tachycardia following an episode of vomiting. Further work-up of his abdominal pain revealed massive retroperitoneal bleeding by CT-scan of the abdomen. Given numerous cases of catastrophic injury caused by surgical intervention in Type-IV Ehlers-Danlos Syndrome, the patient was treated non-operatively, and the patient made a full recovery. This case suggests that even in cases of large retroperitoneal hemorrhages associated with Ehlers-Danlos Syndrome, it may not truly represent a surgical emergency.

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Section: Discussionmentioning

confidence: 99%