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Table 2 Disorders reported in patients with basal cell nevus syndrome.

From: Nevoid basal cell carcinoma syndrome (Gorlin 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