A case report of mesenteric involvement in neurofibromatosis type 1

Abstract

Mutations in the Neurofibromatosis Type 1 (NF-1) gene, which is located on chromosome 17q11.2, are the cause of NF-1, an autosomal dominant hereditary condition in which tumors of the nerve system develop. Neurological, skeletal, and cutaneous abnormalities are symptoms of the condition. Of all gastrointestinal tract lesions, mesentery lesions are the least common. There are many gastrointestinal problems that can accompany mesenteric neurofibromas, or they may not. We describe a case of a 5-year-old kid with mesenteric neurofibromatosis, which results in bowel obstruction and abdominal discomfort. On a CT scan, the mesenteric vasculature was completely encircled by homogenous soft tissue lesions without any vessel wall invasion. Diffuse mesentery lesions were discovered during surgery, although they could not be fully removed.

Diagnosis of Neonatal Neurofibromatosis Type 1: a case report and review of the literature

“…Usually congenitally benign, PNF grows in a reticular pattern to replace normal tissue (14). PNF can be limited, nodular, or diffuse and seen in the paravertebral region of the trunk (31%), head & neck (31%) and extremities (25%) (5). Previous ultrasound reports showed that PNF was an irregularly lobulated hypoechoic mass, which ran along the nerve axis on the longitudinal image diagram and displayed target ring sign on the transverse image, with high echo in the center and low echo around.…”

Diagnosis of neonatal neurofibromatosis type 1: a case report and review of the literature

“…This has no family history of NF1 and is a spontaneous mutation. CALMs are early clinical features of NF1 [ 5 ]. According to the uniform diagnostic criteria of National Institutes of Health (NIH) for NF1 in 1988, six or more CALMs are diagnostic [ 6 ].…”

“…Usually congenitally benign, PNF grows in a reticular pattern to replace normal tissue [ 15 ]. PNF can be limited, nodular, or diffuse and seen in the paravertebral region of the trunk (31%), head & neck (31%) and extremities (25%) [ 5 ]. Previous ultrasound reports showed that PNF was an irregularly lobulated hypoechoic mass, which ran along the nerve axis on the longitudinal image diagram and displayed target ring sign on the transverse image, with high echo in the center and low echo around.…”