SCIENTIFIC
Segmental Absence of Muscularis Propria with Repeated Episodes of Spontaneous Bowel Perforation
AUTHORS : Edward C . Tobin , MD
Nicolas Nolan , MD Nadia Naumova , MD Michael Elmore , MD
Department of Surgery , West Virginia University School of Medicine , Charleston Division , Charleston Area Medical Center
CASE PRESENTATION
A 69-year-old woman with a history of osteogenesis imperfecta ( OI ) of unknown type , anxiety , depression , and gastroesophageal reflux disease presented with a one-day history of sudden onset , severe abdominal pain . She had blue sclerae but demonstrated no other manifestation of OI , such as pathologic fractures or constipation . Her surgical history was remarkable for a left colectomy for spontaneous colon perforation . Notably , her daughter had OI and suffered multiple pathologic fractures as well as spontaneous bowel perforation of undetermined etiology . The daughter ’ s records were not available for review .
ABSTRACT
On examination , the patient was ill appearing and noted to have blue sclerae . The patient ’ s abdomen was distended and demonstrated diffuse rebound tenderness , consistent with peritonitis . Computed tomography ( CT ) of the abdomen and pelvis from an outlying facility demonstrated pneumatosis and markedly thickened bowel wall in the area of the rectosigmoid junction . She underwent an emergent exploratory laparotomy . Intraoperatively , she was immediately found to have gross spillage of stool throughout the abdomen , extensive adhesive disease , a normal caliber colon , and anatomy otherwise consistent with her prior left colectomy . After significant lysis of adhesions , she was noted to have a focal perforation of the distal sigmoid colon with no mass noted near the area of rupture . As there was hard stool noted within the abdomen , the perforation was thought to be secondary to a stercoral ulcer . The patient had previously undergone a left colectomy , and therefore , resection of the diseased portion of colon required segmental resection of the distal sigmoid and distal transverse colon . An end transverse colostomy was then created . At the conclusion of the operation , the patient remained hemodynamically stable and her ostomy viable .
Immediately postoperatively , the patient was tachycardic with a heart rate of approximately 120 beats per minute ( bpm ) and had developed a lactic acidosis ( lactic acid level of 6.7mmol / L ). Over the next 36 hours , she received five units of packed red blood cells , two units of fresh frozen plasma , and 8.5L of crystalloid . On postoperative day two , her tachycardia had improved with a heart rate of approximately 100 bpm and her lactic acidosis had resolved . Over this time , her leukocytosis had improved from 36 x10 3 mcL preoperatively to 12 x10 3 mcL . On postoperative day four , the patient had persistent tachycardia of approximately 100 bpm , increasing abdominal tenderness , and increasing leukocytosis to 21 x10 3 mcL . A CT scan of the abdomen / pelvis demonstrated a large intra-abdominal abscess , which was not amenable to percutaneous drainage . Therefore , the patient underwent repeat exploratory laparotomy . She was found to have necrosis of the distal transverse colon just proximal to the end colostomy , which was thought to be secondary to ischemia , given its proximity to the watershed area between the superior mesenteric and inferior mesenteric artery distributions . There was also
Segmental absence of the muscularis propria is an extremely rare phenomenon with the vast majority of such cases presenting in infancy . The patient discussed in our report originally presented five years prior with an episode of spontaneous bowel perforation . On re-presentation , the patient was again found to have a spontaneous bowel perforation . On each occasion , the defect was associated with a segmental absence of muscularis propria . To the authors ’ knowledge , this is the first described case of a patient presenting with multiple episodes of spontaneous bowel perforation associated with a segmental absence of muscularis propria .
an area of necrosis and perforation in the mid-jejunum , thought to be secondary to a missed enterotomy . A complete colectomy and small bowel resection were performed . Intraoperatively , the patient became hypotensive and coagulopathic and thus was left with an open abdomen and in discontinuity . Two days later , she underwent abdominal closure with end ileostomy creation . Approximately one week later , the patient developed worsening leukocytosis and ischemic changes of the ileostomy , necessitating revision . After this revision , the ostomy became productive , and the patient tolerated enteral feeding . The patient developed pneumonia and acute respiratory distress syndrome requiring prolonged mechanical ventilatory support and the use of paralytic agents to maintain adequate oxygenation . The patient ’ s decline in respiratory function was accompanied by a severe acute kidney injury requiring continuous renal replacement therapy . The patient ’ s multi-system organ failure ultimately led to her death .
The final gross pathologic examination demonstrated a colonic segment 13 cm in length with a circumference of 4.7cm . A transmural wall defect of 4.5cm x 4.1cm was noted 3.4cm from the nearest stapled margin . Of note , there were 10-15 mucosal excrescences ranging in size from 0.2 cm to 0.4 cm , along with four pericolonic , sub-centimeter lymph nodes . The final microscopic examination demonstrated transmural perforation with surrounding acute inflammation , and an associated phlegmon of the pericolonic fat . The surgical margins were viable and free of lesions .
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