Table 2.

Disorders reported in patients with basal cell nevus syndrome.

Clinical manifestations
Skin	Central nervous system
Cutaneous dyskeratosis:	Ectopic calcification:
∘ erythematous-squamous spots degenerating in NBCC	∘ falx cerebri
	∘ tentorium cerebelli
∘ nodular or patch lesions	∘ spotted meningeal calcification
∘ palmo-plantar pits	∘ complete or partial bony bridging of the sella turcica
Multiple basal cell carcinomas
Benign dermal cysts	Meningioma
∘ Multiple nevi	Medulloblastoma
	Multiform glioblastoma
	Moderate mental retardation
	Grand mal
	Congenital hydrocephalus
	Huntington's chorea
Stomatologic system	Ocular system
Odontogenic keratocysts	Cataract, coloboma, microphthalmia
Dental ectopy, heterotopy	Chalazions
Impacted teeth	Internal strabismus
Dental agenesis	Rotatory nistagmus
Malocclusion	Exophthalmus
Maxillary fibrosarcoma	Hypertelorism
Ameloblastoma	Congenital blindness
Odontogenic myxoma
Spindle cell carcinoma
Cleft palate and lip
Mandibular prognathism
High-arched palate
Squamous cell carcinoma
Skeletal open bite
Idiopathic pseudocyst
Hyperplasia of the mandibular coronoid processes
Musculo-skeletal system	Cardio-vascular system
Congenital skeletal anomalies:	Cardiac fibroma (interventricular septum)
∘ bifid, fused, splayed, or missing ribs	Absent internal carotid artery
∘ bifid wedges fused vertebra
∘ scoliosis
∘ frontal, temporal and parietal bossing
∘ polydactyly
∘ sindactyly
∘ short fourth metarcapal
∘ sprengel shoulder
Polyostotic bone cysts
Genito-urinary system	Auditory system
Males:	Middle ear anomalies
∘ hypogonadism	∘ otosclerosis
∘ cryptorchidism	∘ conductive hearing loss
Females:	Posteriorly angulated ears
∘ ovarian calcifications
∘ ovarian cysts
∘ ovarian fibroma
hypogonadism
Respiratory system	Biochemical findings
Bronchogenic cysts	High levels of CAMP
Hyaline membrane disease	High levels of AP
Gastro-enteric system
Linfomesenteryc cysts
Gastric polyps