Segmental Absence of Intestinal Musculature: An Enticing Predicament.

Abstract

Segmental absence of intestinal musculature (SAIM) was first identified as a cause of intestinal perforation and remains difficult to diagnose clinically. Diagnosing SAIM preoperatively can be difficult; however, detection during the surgical procedure may be feasible if gross observations reveal bowel alterations such as a thinned-out wall, signs of gangrenous changes, or adjacent bowel dilatation. A final diagnosis can be made with the help of histopathology, making it a gold standard. Even though this condition is more prevalent among newborns, only a handful of cases have been published in the literature, mentioning involvement of the older patients. Maintaining a high index of suspicion for this disease when dealing with cases of intestinal obstruction or perforation in children and adults is instrumental in facilitating swift diagnosis and prompt treatment, thus improving the patient's outcome. We, hereby, would like to add one more case report of a 4-year-old girl child presenting with this rare entity to the literature.