Ehlers-Danlos Syndrome Type IV and a Novel Mutation of the Type III Procollagen Gene as a Cause of Abdominal Apoplexy

Hassan, Iffat; Rasmussen, Todd E.; Schwarze, Ulrike; Rose, Peter S.; Whiteman, David A.H.; Gloviczki, Peter

doi:10.4065/77.8.861

Cited by 15 publications

(4 citation statements)

References 12 publications

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“…3 No bleeding source was identified in three of the remaining six cases including our patient, and all three survived. 4,9 All six survivors were discharged from the hospital and were in good health at follow-up. Although abdominal apoplexy remains a rare and curious diagnosis, modern surgical management has likely improved the prognosis considerably.…”

Section: Discussionmentioning

confidence: 98%

“…Although the pathophysiology behind abdominal apoplexy has remained somewhat of a mystery since Barber's report, modern gene sequencing has previously implicated a mutation in the procollagen gene as a potential cause. 4 Most often, the bleeding source is identified within the splanchnic vasculature with the middle colic, pancreaticoduodenal, and superior mesenteric arteries usually involved. 5 The role of anticoagulation in the pathophysiology of abdominal apoplexy has not been studied.…”

Section: Discussionmentioning

confidence: 99%

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Spontaneous Colonic Mesenteric Hemorrhage: Report of an Unusual Case of Abdominal Apoplexy

Reilly

Blansfield

Zebley

2005

Diseases of the Colon &Amp; Rectum

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We describe a case of idiopathic abdominal apoplexy secondary to a spontaneous mesocolic bleed. Preoperative computed tomography of the abdomen suggested a ruptured colonic mass at the splenic flexure. The diagnosis was made when exploratory laparotomy revealed hemoperitoneum and an extensive hematoma in the transverse mesocolon, with no apparent source. Idiopathic abdominal apoplexy is rare, the reported mortality has historically been high, and a short hospital stay and rapid, complete recovery are uncommon, although outcomes may be improving in recent years. We present such a case and examine the clinical presentation, management, and prognosis through literature review.

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Section: Discussionmentioning

confidence: 98%

Section: Discussionmentioning

confidence: 99%

Spontaneous Colonic Mesenteric Hemorrhage: Report of an Unusual Case of Abdominal Apoplexy

Reilly

Blansfield

Zebley

2005

Diseases of the Colon &Amp; Rectum

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“…Los de índole no traumática pueden vincularse a conectivopatías, neoplasias, arteriopatías, coagulopatías, terapia anticoagulante, pancreatitis aguda, enfermedad de Crohn y hernia inguinal encarcelada 2,6,7,8 . Actualmente no existen protocolos claros de tratamiento 2,8,9 . La aparición de un HME puede ser una complicación infrecuente del tratamiento prolongado con anticoagulantes orales, dado que aumentan el riesgo de desarrollar complicaciones hemorrágicas multiorgánicas graves, incluyendo la hemorragia intramural del intestino delgado, hematoma de la pared, hemorragias abdominales y retroperitoneales 2,10 .…”

Section: Discussionunclassified

Hematoma mesentérico espontáneo asociado a warfarina con resolución quirúrgica

Montero

Petersen

Cafaro

et al. 2019

Rev Fac Cien Med Univ Nac Cordoba

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Introducción: El hematoma mesentérico espontaneo es poco frecuente. Se ha informado como complicación de anticoagulación prolongada, asociado con mayor frecuencia a la warfarina. El estudio diagnostico estándar es la tomografía computada. La terapéutica de esta patología, está en discusión. Se sabe que en pacientes hemodinámicamente estables con hematoma mesentérico espontaneo se lleva a cabo una conducta conservadora, dejando el tratamiento quirúrgico como alternativa para pacientes en shock. Nuestro objetivo es reportar el caso de una paciente adulta con hematoma mesentérico espontáneo en relación a warfarina, tratado de manera quirúrgica con buena evolución. Caso clínico: Paciente de 67 años, sexo femenino, con hematoma mesentérico espontáneo en relación a warfarina, con antecedente de reemplazo de válvula biológica mitral con plástica tricúspidea de más de 3 meses, en controles diarios por mal manejo de la coagulación con anticoagulante oral; consulto por dolor abdominal súbito, en región epigástrica, acompañado de vómitos gástricos. Al examen físico se objetivo defensa y reacción peritoneal. Se solicitó tomografía computada abdomen y pelvis donde se informó hematoma mesentérico. Debido a que la paciente presentaba signos de irritación peritoneal se realizó cirugía de urgencia con resección segmentaria de intestino delgado. Con buena evolución postquirúrgica, siendo dada de alta al quinto día. Anatomía patológica informa hemorragia masiva submucosa. Conclusión: Ante un paciente que presenta dolor abdominal con signos de irritación peritoneal, alteración de la Relación Internacional Normatizada por consumo de warfarina, sin historia traumática, debe sospecharse un hematoma mesentérico espontáneo y considerar el tratamiento quirúrgico.

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“…In younger patients congenital defects or systemic disease might account for the hemorrhage such as polyarteritis nodosa and Ehlers-Danlos syndrome. [ 1 , 12 , 13 ]…”

Section: Discussionmentioning

confidence: 99%

Abdominal apoplexy because of the rupture of gastroduodenal artery and inferior pancreaticoduodenal artery

Wang

Xiu

2017

Medicine

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Rationale:Abdominal apoplexy is a rare and fatal emergency event, which is coined as a comparison to the cerebrovascular apoplexy. The exact mechanism of abdominal apoplexy was unclear, but arteriosclerosis, hypertension, abdominal aneurysm, and other predisposing angiopathy were considered to be the main reasons of abdominal apoplexy. The development of the imaging technology gave us more opportunities to confirm the diagnosis of abdominal apoplexy. However, the diagnosis and identification of the bleeding sites still continued to be a challenge.Patient concerns:A 55-year-old man presented to the emergency department with chief complains of sudden severe abdominal pain.Diagnosis:The patient was diagnosed as abdominal apoplexy with 2 synchronous bleeding sites.Interventions:Angiography confirmed diagnosis of abdominal apoplexy and revealed 2 synchronous bleeding sites in gastroduodenal artery (GDA) and inferior pancreaticoduodenal artery (IPDA). Transcatheter embolization was performed immediately.Outcomes:The patient recovered and was discharged very soon. Two months later, the patient totally recovered and the hematoma disappeared in the CT imaging.Lessons:The reported case is rare, given the very low incidence of abdominal apoplexy with 2 synchronous bleeding sites in GDA and IPDA. The awareness of abdominal apoplexy was still the key point in the management of this disease. Quick diagnosis by the imaging and immediate embolization were very important for the treatment.

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Ehlers-Danlos Syndrome Type IV and a Novel Mutation of the Type III Procollagen Gene as a Cause of Abdominal Apoplexy

Cited by 15 publications

References 12 publications

Spontaneous Colonic Mesenteric Hemorrhage: Report of an Unusual Case of Abdominal Apoplexy

Spontaneous Colonic Mesenteric Hemorrhage: Report of an Unusual Case of Abdominal Apoplexy

Hematoma mesentérico espontáneo asociado a warfarina con resolución quirúrgica

Abdominal apoplexy because of the rupture of gastroduodenal artery and inferior pancreaticoduodenal artery

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