Infantile myofibromatosis: a most unusual cause of gastric outlet obstruction

Abstract

Non-bilious vomiting in the newborn is common. Etiologies include both surgical and medical conditions. Gastroesophageal reflux, soy or milk protein allergy, and prostaglandin-induced foveolar hyperplasia are among the medical causes. Surgical entities include gastric antral webs, pre-ampullary duodenal and pyloric atresia, and hypertrophic pyloric stenosis. We report the unique case of an 8-day-old girl who presented with gastric outlet obstruction secondary to infantile myofibromatosis.

Gastric hamartoma of the pylorus in an infant

“…Indeed, before the laparotomy, a myofibroma of the pylorus was our presumptive diagnosis, the ultrasound images in our case being so very similar to those reported by Rohrer et al An infantile myofibroma can present as a solitary lesion or as a part of diffuse myofibromatosis with muscle, bone, soft tissue, skin, and visceral involvement [14].…”

“…A myofibroma of the pylorus is a rare cause of gastric outlet obstruction with similar ultrasound features to a gastric hamartoma, which make preoperative differentiation difficult [14]. Indeed, before the laparotomy, a myofibroma of the pylorus was our presumptive diagnosis, the ultrasound images in our case being so very similar to those reported by Rohrer et al An infantile myofibroma can present as a solitary lesion or as a part of diffuse myofibromatosis with muscle, bone, soft tissue, skin, and visceral involvement [14].…”

Multiple Bone Lesions in an 8-Month-Old Child Presenting with Pathologic Fracture

“…In cases of solitary or multicentric IM involving the viscera in which surgical resection is not advisable or the lesions are life-threatening, chemotherapy regimens have been described that successfully treated the lesions. These regimens include vinblastine with methotrexate, vincristine alone, vincristine with methotrexate, vincristine with actinomycin-D, and 2-chlorodeoxyadenosine [25][26][27][28][29] . Caution must be taken to balance the early and late side effects of these agents with their benefits.…”

Multicentric non‐visceral infantile myofibromatosis causing hip contracture

“…The prognosis for solitary and non‐visceral multicentric forms is excellent, whereas more than half of the infants with visceral involvement die from the disease and its complications 1,3,6–8 . Many of the lesions without visceral involvement stabilize and may undergo spontaneous regression within 1 or 2 years 1,3,7 . Here we report on a full‐term newborn girl who presented with the benign form of multicentric non‐visceral infantile myofibromatosis that caused significant contracture of the left hip, a complication not reported hitherto.…”

“…In the multicentric subgroups several soft‐tissue and bone sites may be involved alone, 1,3,5,6 or concomitantly with lung, cardiac, gastrointestinal or even central nervous system involvement (multicentric with visceral involvement) 7,8 . The prognosis for solitary and non‐visceral multicentric forms is excellent, whereas more than half of the infants with visceral involvement die from the disease and its complications 1,3,6–8 . Many of the lesions without visceral involvement stabilize and may undergo spontaneous regression within 1 or 2 years 1,3,7 .…”