title: An adult case of midgut volvulus in familial visceral myopathy.
abstract: We report an adult case of midgut volvulus in familial visceral myopathy (FVM) that had affected family members over three generations. The patient was a Japanese woman in her fifties, who had chronic intestinal pseudo-obstruction (CIPO) since the age of about 40 years and had been treated chronically with conservative therapies. Her abdominal symptoms suddenly worsened and surgery became necessary. Surgery revealed a midgut volvulus secondary to intestinal malrotation and the twisted intestine was resected. Histology revealed diffuse damage of myocytes confined to the muscularis propria throughout the resected intestine. The myocytes were irregulary arranged, contained cytoplasmic inclusions, and had mild and focal vacuolar changes. The mucsularis propria showed hypertrophy with delicate interstitial fibrosis. A diagnosis of FVM was made on the basis of this characteristic myopathy. Intestinal malrotation is known to be a complication of CIPO in children, but is rare in adults. Although midgut volvulus appears to be extremely rare, it can occur after a relatively stable chronic phase in adult CIPO patients, who should be monitored carefully to assess the risk of such complications.
authors: Department of Diagnostic Pathology, NTT Medical Center Tokyo, Shinagawa-ku, Tokyo, Japan. majikana26@hotmail.com
authors: Takahiko
authors: T
authors: Nakajima
authors: Nobuyuki
authors: N
authors: Matsuhashi
authors: Satoshi
authors: S
authors: Nara
authors: Atsushi
authors: A
authors: Nakajima
authors: Johji
authors: J
authors: Imura
authors: Atsushi
authors: A
authors: Kihara
authors: Kenichiro
authors: K
authors: Murata
authors: Junichi
authors: J
authors: Fukushima
authors: Hajime
authors: H
authors: Horiuchi
mesh_terms: D005192:Family Health; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D007418:Intestinal Pseudo-Obstruction / Q000150:complications / Q000235:genetics / Q000473:pathology*; D045822:Intestinal Volvulus / Q000175:diagnosis* / Q000209:etiology / Q000235:genetics; D007422:Intestines / Q000473:pathology / Q000601:surgery; D008875:Middle Aged; D032389:Myocytes, Smooth Muscle / Q000473:pathology; D016896:Treatment Outcome