Mesenteric neurofibromatosis in childhood

Abstract

A child is reported in whom severe malabsorptive symptoms were present from a short time after birth together with increasing pseudo‐obstructive episodes. Total neurofibromatous involvement of the smallbowel mesentery was found at laparotomy at the age of 4 1/2 years. The child survived a further 2 1/4 years. The relevant literature is discussed.

Intestinal obstruction in von Recklinghausen's disease

“…From the first published article in 1972–2013 we identified 25 articles reporting 25 patients with von Recklinghausen's disease who underwent laparotomy for acute intestinal obstruction , and with the addition of three patients from our institution (Table ) the mean age was 44.2 (0.5–73) years. The male/female ratio was approximately 1/1.…”

Mesenteric plexiform neurofibroma in an 11-year-old boy with von Recklinghausen disease

“…Intestinal plexiform neurofibromas present with ulceration, bleeding, intestinal obstruction, and rarely, intussusception, volvulus, and perforation. Intestinal vascular and lymphatic obstruction may result in bowel edema, loss of protein-rich chyle into the bowel lumen, causing hypoproteinemia and malabsorption [4,5,10]. However, most small mesenteric plexiform neurofibromas are asymptomatic.…”

Gastric lesions in generalized neurofibromatosis

“…The patient's abdomen demonstrating cafe-au-lait spots and a Subcutaneous nodule. Latimer, 1971 ; Wilkerson, 1971), anaemia (Burton and Fitzgerald, 1970), dyspepsia or abdominal pain (Perea and Gregory, 1962), or less commonly as perforation (Warshauer and Nelson, 1953), volvulus (Collins, 1944), jaundice (Curry and Gray, 1972), intussusception (Marshak et al, 1963) or malabsorption(Cywes and Mayell, 1972) The neurofibromas are usually found in the duodenum and small bowel(Lawson and Latimer, 1971 ;Wilkerson, 1971), but tumours affecting the colon(Ternberg and Winters, 1965), omenturn (Thomas and Jelliffe, I953), mesentery(Cywes and Mayell, 1972) and appendix(Mazare et al, 1968) have been reported. One patient with multiple ganglioneuromas affecting the oesophagus was mentioned byRussell and Rubinstein (1971).…”