Abdominal migraine in children with neurofibromatosis type 1: A case series and review of gastrointestinal involvement in NF1

Background: Symptomatic involvement of the gastrointestinal tract in childr en with neurofibromatosis type 1 (NF1) is rare. Most reported complications in adults are caused by the presence of neurofibromas in the stomach, smal l bowel, or mesentery. In contrast, abdominal pain in children with NF1 may be the result of nonanatomic causes, such as migraine. There are no previo us reports of an association between abdominal migraine and NF1. Methods: Children with abdominal migraine were identified from a group of c hildren with NF1, all of whom had been followed up for a minimum of 3 years . Medical records of cases were reviewed independently by two authors. MEDL INE was searched via PubMed for all reports of children with NF1 and any as sociated gastrointestinal involvement. Results: Six children with NF1 and intermittent, episodic, severe abdominal pain are reported. Investigations for obstructive or inflammatory causes o f abdominal pain were negative. All patients had previously been diagnosed with migraine headaches by a neurologist. In five of the six patients, prop ranolol (10-15 mg three times daily) resulted in relief of their abdominal pain within days of starting therapy. Our review identified 24 children in the medical literature with gastrointestinal complications of NF1, mostly s econdary to visceral neurofibromas. In almost all of these cases, clinical examination and simple radiologic investigations led to the definitive diag nosis. There were no reports of abdominal migraine complicating NF1. Conclusions: Abdominal pain secondary to migraine is an unrecognized cause of abdominal pain in children with NF1 and may be more common than anatomic causes of abdominal pain in children with NF1. In children with NF1 and se vere recurrent abdominal pain in whom an evaluation for anatomic lesions is negative, a trial of migraine therapy may be indicated.