The clinical and imaging presentations of the pontine tegmental cap dysplasia
10.3760/cma.j.cn115330-20250213-00099
- VernacularTitle:脑桥被盖“帽状”发育不良的临床和影像学表现
- Author:
Linsheng WANG
1
;
Lihong ZHANG
;
Lixin SUN
;
Xiaoqin LI
;
Jinye LI
;
Xiaoqian GE
;
Bing ZHAO
Author Information
1. 山东省第二人民医院影像中心，济南 250021
- Publication Type:Journal Article
- Keywords:
Congenital abnormalities;
Pontine tegmental cap dysplasia;
Ear, inner;
Tomography, X-ray computed;
Magnetic resonance imaging
- From:
Chinese Journal of Otorhinolaryngology Head and Neck Surgery
2025;60(11):1426-1430
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To summarize the clinical and imaging presentations of the pontine tegmental cap dysplasia (PTCD).Methods:The clinical, high resolution CT(HRCT) and MRI materials of 4 patients with PTCD between August 2007 to December 2024 were retrospectively analyzed. Among these, there were 2 males and 2 females, their ages ranged from 10 months to 16 years.Results:Of 4 PTCD patients, severe or profound severe hearing loss ( n=8 ears), developmental delay, hypotonia and severe facioplegia ( n=3 cases) were found. On HRCT, all of 4 cases were associated with temporal anomalies [including a narrow bony cochlear nerve canal ( n=8 ears), duplicated (each n=4 ears) or narrow ( n=1 ear) internal auditory canal, enlarged vestibular aqueduct ( n=2 ears), enlarged vestibules and dysplastic lateral semicircular canals ( n=3 ears), ossicular deformation( n=2 ears). The stenosis of the labyrinthine segments of the facial nerve canal ( n=3 ears) and facial nerve canal ectopia(n=6 ears)], atrial or ventricular septal defect (each n=1 case), thoracic or lumbar vertebral anomalies and ribs fusion ( n=3 cases). On the brain MRI, the variable flattening of the ventral pons and dysmorphism of the dorsal upper pons cap-like bulging and protruding in the fourth ventricle were shown in all cases, the vermian and cerebellar peduncles hypoplasia gave rise to a molar tooth appearance. The dysplastic ( n=3 ears), aplastic( n=5 ears) cochlear nerves and dysplastic facial nerves ( n=3 ears) were found. Conclusion:The PTCD patients usually present severe hearing loss, developmental delay, hypotonia, and facioplegia. The flattening of the ventral pons and the dorsal upper pons cap-like bulging usually with duplicated internal auditory canal and severe facial and auditory nerves dysplasia are its imaging features.
