Jae Hyoung Kim1, Jeong-Min Hwang. 1. Department of Radiology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Seongnam, Korea.
Abstract
OBJECTIVE: Congenital cranial dysinnervation disorders (CCDDs) are developmental abnormalities involving the hypoplasia or aplasia of the cranial nerves with muscle dysinnervation. This study aimed to determine if congenital superior oblique palsy with superior oblique hypoplasia also can be classified as a CCDD. DESIGN: Observational case series. PARTICIPANTS: Ten patients with superior oblique hypoplasia. METHODS: Ophthalmologic examination and thin-section magnetic resonance imaging (MRI) at the brainstem level as well as across the orbit were performed in 10 patients with superior oblique hypoplasia. To confirm the accuracy of the procedure, the results obtained were compared with those of a control group comprising 12 children by using the same technique. MAIN OUTCOME MEASURES: Examination of ductions and versions, 3-step test, angle of deviation, and presence of the trochlear nerve and superior oblique hypoplasia on MRI. RESULTS: Trochlear nerve was absent on the affected side in 10 patients with confirmed hypoplastic superior oblique muscles. The trochlear nerves as well as normal-sized superior oblique muscles were observed in 24 (100%) of 24 eyes screened as controls. CONCLUSIONS: Congenital superior oblique palsy with superior oblique hypoplasia also can be classified as a CCDD by the MRI documentation of congenital aplasia of the trochlear nerve. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.
OBJECTIVE:Congenital cranial dysinnervation disorders (CCDDs) are developmental abnormalities involving the hypoplasia or aplasia of the cranial nerves with muscle dysinnervation. This study aimed to determine if congenital superior oblique palsy with superior oblique hypoplasia also can be classified as a CCDD. DESIGN: Observational case series. PARTICIPANTS: Ten patients with superior oblique hypoplasia. METHODS: Ophthalmologic examination and thin-section magnetic resonance imaging (MRI) at the brainstem level as well as across the orbit were performed in 10 patients with superior oblique hypoplasia. To confirm the accuracy of the procedure, the results obtained were compared with those of a control group comprising 12 children by using the same technique. MAIN OUTCOME MEASURES: Examination of ductions and versions, 3-step test, angle of deviation, and presence of the trochlear nerve and superior oblique hypoplasia on MRI. RESULTS: Trochlear nerve was absent on the affected side in 10 patients with confirmed hypoplastic superior oblique muscles. The trochlear nerves as well as normal-sized superior oblique muscles were observed in 24 (100%) of 24 eyes screened as controls. CONCLUSIONS:Congenital superior oblique palsy with superior oblique hypoplasia also can be classified as a CCDD by the MRI documentation of congenital aplasia of the trochlear nerve. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.