Familial spontaneous rupture of the colon

Schofield, Philip F.; Macdonald, N.; Clegg, J.

doi:10.1007/bf02616763

Cited by 4 publications

(3 citation statements)

References 8 publications

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“…A total of 43 case reports describing 102 operations in 48 patients (Fig. ) were identified by electronic literature searching and cross‐referencing. An additional three patients undergoing seven surgical procedures were identified from the case records of the two national specialist intestinal failure units at Salford and St Mark's Hospitals, UK.…”

Section: Resultsmentioning

confidence: 99%

“…A number of strategies to manage patients with suspected or confirmed EDS and colonic perforation have been described in the literature. These include primary repair [9][10][11], primary repair and defunctioning stoma [5,6,9,[11][12][13][14][15], Hartmann's procedure [9,[14][15][16][17][18][19][20][21][22][23][24][25][26][27][28][29][30][31] and total abdominal colectomy (TAC) with either an endileostomy or ileorectal anastomosis (IRA) [8,[32][33][34][35][36]. While total colectomy and IRA may prevent further colonic perforation, the leak rate of IRA in such cases has been reported to be as high as 50% [35].…”

Section: Introductionmentioning

confidence: 99%

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Management of colonic complications of type IV Ehlers–Danlos syndrome: a systematic review and evidence‐based management strategy

et al. 2019

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Aim Type IV Ehlers Danlos Syndrome (EDS) is a connective tissue disorder affecting approximately 1 per 100,000-200,000 people. Life expectancy is reduced secondary to spontaneous vascular rupture or colonic perforation. Surgery carries significant morbidity and mortality. While strategies to manage colonic perforation include primary repair with or without a defunctioning stoma, Hartmann's procedure, total abdominal colectomy with end ileostomy and ileorectal anastomosis, evidence is contradictory and has not previously been evaluated in order to form a treatment strategy. We aim to review the published literature and identify outcome data relating to operative management of colonic perforation in type IV EDS.Methods Pubmed, EM-BASE, Cochrane library and Google Scholar were searched with the following details: Ehlers Danlos Syndrome AND colonic surgery. The main outcome measure was re-perforation rates following colonic surgery on patients with type IV EDS. If the nature of surgery and follow up were reported, data were recorded in a SPSS database according to PRISMA guidelines.Results One hundred and nine operations have been described in 51 patients in 44 case series. There were 26 visceral re-perforations, 2 affecting the small intestine and 24 colonic. Survival analysis favoured total abdominal colectomy compared with operations where the colon was left in situ.Conclusions Total abdominal colectomy with end ileostomy or ileorectal anastomosis are the safest strategies after colonic perforation in type IV EDS. Anastomotic leak rates are high. End colostomy is high risk for colonic re-perforation and anastomotic leak rates are extremely high. Restoration of colonic continuity should be avoided.

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Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Management of colonic complications of type IV Ehlers–Danlos syndrome: a systematic review and evidence‐based management strategy

et al. 2019

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“…Eleven patients died after the initial perforation, 4 patients died from vascular complications, 4 patients died from multiorgan failure or subsequent infection, 4 patients died from the anastomotic leak, 2 died from recurrent perforation, 1 patient died from evisceration, 1 patient died from splenic rupture, and 8 patients died from unspecified causes. Among the reported cases of “spontaneous” colonic perforation, 12 patients were documented as presenting with constipation [11,13,14,21,26,38,43,44].…”

Section: Discussionmentioning

confidence: 99%

Intestinal Perforation in Children as an Important Differential Diagnosis of Vascular Ehlers-Danlos Syndrome

2019

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Patient: Male, 6 Final Diagnosis: Colonic perforation secondary to vascular Ehlers Danlos Syndrome Symptoms: Abdominal pain • constipation Medication: — Clinical Procedure: Loop colostomy followed by total colectomy and ileostomy Specialty: Surgery Objective: Unusual clinical course Background: Ehlers-Danlos Syndrome (EDS) is a group of connective tissue disorders with heterogeneous clinical features associated with varying genetic mutations. EDS type IV, also known as vascular EDS (vEDS), is the rarest type but has fatal complications, including rupture of major vasculature and intestinal and uterine perforation. Intestinal perforation can be spontaneous or a consequence of long-standing constipation, a common symptom among patients with EDS. Case Report: We present a case of a 6-year-old boy with the previous diagnosis of vEDS who presented with colonic perforation from a stercoral ulcer. He underwent diagnostic laparoscopy and loop colostomy, with an uneventful postoperative course. Unfortunately, he developed a second colonic perforation 14 months after the initial episode and underwent total abdominal colectomy with end ileostomy. Conclusions: Intestinal perforation is a well-documented and devastating complication of vEDS. However, spontaneous intestinal perforation is extremely rare in a young child. Therefore, the diagnosis of vEDS should be included in the differential diagnosis if a child presents with intestinal perforation. There is no clear guideline available for surgical management of colonic perforation in patients with vEDS, but total abdominal colectomy appears to provide the best chance of preventing recurrent perforation.

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Colon perforation in Ehlers-Danlos syndrome

Sykes

1984

The American Journal of Surgery

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Familial spontaneous rupture of the colon

Cited by 4 publications

References 8 publications

Management of colonic complications of type IV Ehlers–Danlos syndrome: a systematic review and evidence‐based management strategy

Management of colonic complications of type IV Ehlers–Danlos syndrome: a systematic review and evidence‐based management strategy

Intestinal Perforation in Children as an Important Differential Diagnosis of Vascular Ehlers-Danlos Syndrome

Colon perforation in Ehlers-Danlos syndrome

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