Trigeminal neurinomas in infants: report of two cases

Abstract

Two cases of trigeminal neurinoma in two infant girls aged 3 and 6 months are reported. Both presented with temporal cranial vault bulging at birth. The 6-month-old patient suffered onset of focal fits 1 month before admission and her neurological examination revealed no abnormalities. The 3-month-old patient had right exophthalmus and a subcutaneous fronto-orbital plexiform neurofibroma at birth. Neurological examination disclosed a sensory deficit of the first trigeminal nerve division. She also had a family medical history of von Reckling-hausen's disease. The incidence of trigeminal neurinomas in children is reviewed. The patients in these two cases are the youngest recorded; the cases are the only ones reported in infants. Clinical, radiological, and therapeutic aspects are discussed.

Perinatal Neurofibromatosis: Two Case Reports and Review of the Literature

“…3 Perinatal NF-1 may present clinically in extraordinary ways: congenital stridor resulting from a laryngeal tumor 11,36 ; airway obstruction from tracheal compression 14,22 ; as part of a ''congenital neurocristopathy'' occurring together with neuroblastoma, Schwannoma, and plexiform neurofibroma 6,10,12,13,15,22,28 (Fig. 2); congenital buphthalmos, proptosis, and glaucoma secondary to orbital NFA 17,18,20,22,26,27,30,33,35 ; progressive respiratory compromise resulting from rapidly growing NFAs in the tongue, oral cavity, neck 14,19,22,26 ; a vascular malformation 34 ; fetal hydrops following compression of neck vessels or vena cava by tumor. 5,6,28,37 Over a third of patients in the review had the generalized form of the disease, ''congenital disseminated NF'' (Table 1).…”

“…NF-1 is one of the hereditary neurocutaneous syndromes that often affects the globe and ocular adenexa. 17,18,20,27,30,33,35 Almost half of these neonates with this disease have physical signs consisting of multiple café au lait spots, PNFAs, and lesions affecting the ocular structures. 41 Forty percent (34/85) of the neonatal presenting findings here were related to the eye.…”

“…Sphenoid bone dysplasias are found with orbital and intracranial involvement. 18,20,30,33 Patients with a sphenoid dysplasia may present with a pulsating, proptotic eye as the result of herniation of the meninges or temporal lobe into the orbit along with a palpable mass. 1,33 Sphenoid bone defects were found here in 6 of 36 or 17%.…”

Giant Multi-Compartmental Trigeminal Neurinoma in a Child: A Case Report

“…However a solitary neurinoma at this site in a child without neurofibromatosis is a rare occurrence [2][3][4]. Only a few case reports of small sized solitary neurinomas in children without neurofibromatosis are reported in the literature [2][3][4][5]. We report a rare case of a giant multicompartmental trigeminal neurinoma without neurofibromatosis in a child that was completely excised by single stage petrosal approach, with good neurological recovery.…”

Single stage complete removal of dumbbell trigeminal schwannoma in a child by skull base approach

“…[ 5 ] Frequently seen in the fourth–fifth decades of life, TS in childhood is extremely rare, even rarer is their occurrence in childhood, in the absence of neurofibromatosis (NF), only five such cases have been described in literature. [ 1 2 3 4 5 6 7 ] All literature that describes surgical approaches for these tumors is mostly confined to adults. [ 3 4 5 ] The rarity of these tumors in childhood and their multi-compartmental extensions pose a unique challenge that needs to be understood.…”