Acute Abdomen and Perforated Bowel with a Rare Pathology: Nonfamilial Visceral Myopathy

Abstract

Visceral myopathy is a rare chronic disease affecting the peristalsis of the bowel causing intermittent pseudoobstruction. We report an atypical case of an eighty-nine-year-old woman with no prior history of abdominal illness who was admitted to our hospital with 2 days of increasing nausea, abdominal distension, and abdominal pain. On arrival at the hospital, she was critically ill. Abdominal X-ray showed distended loops of the colon and liquid levels resembling colonic obstruction. A subsequent abdominal CT scan confirmed the colonic obstruction. A suspicion of sigmoid volvulus was raised, that is why a barium enema was performed but no lower colonic obstruction could be confirmed. Acute laparotomy showed perforated cecum without intestinal obstruction. Postoperatively, the patient became septic which was fatal for the patient. Pathology gave the diagnosis visceral myopathy. It is very difficult to make the diagnosis clinically and radiologically since visceral myopathy mimics other more common gastrointestinal diseases. It is important to consider visceral myopathy as a possible diagnosis in cases with recurrent episodes of abdominal pain, vomiting, and abdominal distension, but without actual intestinal obstruction.

Misato underlies visceral myopathy in Drosophila

“…Particularly, degeneration of the visceral muscle along with fibrosis is the hallmark of visceral myopathy (VM) accompanying intestinal dilation and obstruction, deficient bowel movement, abdominal pain, and malnutrition 7 . Although VM is a rare disease, pathogenic symptoms are severe and often familial 8 , 9 . A member of actins specific to smooth muscles was previously suggested as a causative factor for VM through genetic studies and genome-wide sequencing 10 – 12 , however, whether intervention of a specific gene in vivo is linked to VM has remained elusive.…”

Familial visceral myopathy diagnosed by exome sequencing of a patient with chronic intestinal pseudo-obstruction

“…Chronic intestinal pseudo-obstruction (CIP) is a heterogeneous group of severe gut motility disorders characterized by impaired gastrointestinal propulsion without mechanical obstruction. Onset is from infancy [1] to 89 years of age [2]. The adult prevalence rate is estimated to be 0.9/ 100 000 [3].…”

Hollow Visceral Myopathy, a Rare Gastrointestinal Disorder: A Case Report and Short Review

“…Additionally, a rare case of intestinal perforation secondary to VM has also been reported. 6 As per current literature, the predominant symptoms include nausea, vomiting, chronic constipation, abdominal pain, abdominal distension, and features of malnutrition. A few cases were also reported to present with diarrhea and steatorrhea secondary to small intestinal bacterial overgrowth due to intestinal stasis.…”

“…5 In the literature, patients with HVM usually have a chronic duration of symptoms coupled with nausea, vomiting, abdominal pain, and distention, such as that seen in our patient. 6 Furthermore, to establish a diagnosis, mechanical obstruction should be ruled out via radiological investigations, and these patients often require histological examination of the bowel mucosa, both of which were performed for our patient. 7 Our case report is unique as it not only describes a rare clinical condition but also discusses the diagnostic challenges associated with the disease entity due to varied clinical symptoms and its similarity with other more common GI disorders.…”