title: Pontine tegmental cap dysplasia: report of two new cases from Kuwait.
abstract: We describe two patients (a Filipino boy aged 2.7 years and a Kuwaiti girl aged 4.8 Years) with clinical and MRI findings consistent with the diagnosis of pontine tegmental cap dysplasia (PTCD) and compare them with 23 other cases reported in the literature. Both presented with feeding problems (VII nerve), sensori-neural deafness (VIII nerve) and hypotonia from birth and later developed corneal opacities due to loss of corneal sensation (V nerve). They have severe psychomotor developmental delay. The MRI of their brain showed a flattened ventral pons, vaulted "cap"- like structure protruding into 4th ventricle and a "molar tooth" sign. One of our patients also had Tetralogy of Fallot (TOF) successfully corrected. The other had no extracranial manifestations. The findings in our patients are similar to those reported except for the occurrence of TOF which has not been reported before in association with PTCD.
authors.affiliation: Al Adan Hospital, Pediatric Department, Neurology Unit, Kuwait. Electronic address: jov_maja@yahoo.com.
authors.forename: Marina
authors.initials: M
authors.lastname: Jovanovic
authors.affiliation: Al Adan Hospital, Pediatric Department, Neurology Unit, Kuwait. Electronic address: mzakkariah72@gmail.com.
authors.forename: Mohammed Zakkhariah
authors.initials: MZ
authors.lastname: Chinnathambi
authors.affiliation: Clinical Center Nis, Institute of Radiology, Serbia. Electronic address: ivanadidi@yahoo.com.
authors.forename: Ivana
authors.initials: I
authors.lastname: Markovic
authors.affiliation: Al Adan Hospital, Pediatric Department, Kuwait. Electronic address: magedeilewa@yahoo.com.
authors.forename: Maged Elshahat
authors.initials: ME
authors.lastname: Abdrabon Eilewa
authors.affiliation: Al Adan Hospital, Pediatric Department, Kuwait. Electronic address: drsasadeq@hotmail.com.
authors.forename: Sameera Abdulla
authors.initials: SA
authors.lastname: Sadeq
authors.affiliation: Al Adan Hospital, Pediatric Department, Neurology Unit, Kuwait. Electronic address: alliemoosa@gmail.com.
authors.forename: Allie
authors.initials: A
authors.lastname: Moosa
mesh_terms: D017668:Age of Onset; D001933:Brain Stem / Q000473:pathology; D002675:Child, Preschool; D003638:Deafness / Q000151:congenital; D005155:Facial Nerve Diseases / Q000151:congenital / Q000473:pathology; D005260:Female; D020546:Fourth Ventricle / Q000473:pathology; D006801:Humans; D007231:Infant, Newborn; D007730:Kuwait; D008279:Magnetic Resonance Imaging; D008297:Male; D011149:Pons / Q000002:abnormalities* / Q000473:pathology*; D013681:Tegmentum Mesencephali / Q000002:abnormalities* / Q000473:pathology*; D013771:Tetralogy of Fallot / Q000150:complications; D000160:Vestibulocochlear Nerve Diseases / Q000151:congenital / Q000473:pathology
keywords: Cranial nerves; Deafness; Feeding difficulty; PTCD; Pontine tegmental cap dysplasia