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Hypoplastic trochlear nerve

MedGen UID:
1841585
•Concept ID:
C5826803
•
Congenital Abnormality
Synonym: Hypoplastia of cranial nerve IV
 
HPO: HP:0034965

Definition

Underdevelopment of the trochlear nerve, which is the fourth cranial nerve and one of the ocular motor nerves that controls eye movement. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVHypoplastic trochlear nerve

Recent clinical studies

Etiology

Possible association of congenital Brown syndrome with congenital cranial dysinnervation disorders.
Ellis FJ, Jeffery AR, Seidman DJ, Sprague JB, Coussens T, Schuller J
J AAPOS 2012 Dec;16(6):558-64. doi: 10.1016/j.jaapos.2012.09.005. PMID: 23237754
Congenital superior oblique palsy and trochlear nerve absence: a clinical and radiological study.
Yang HK, Kim JH, Hwang JM
Ophthalmology 2012 Jan;119(1):170-7. Epub 2011 Sep 15 doi: 10.1016/j.ophtha.2011.06.038. PMID: 21924501
Absence of the trochlear nerve in patients with superior oblique hypoplasia.
Kim JH, Hwang JM
Ophthalmology 2010 Nov;117(11):2208-13.e1-2. Epub 2010 Jun 8 doi: 10.1016/j.ophtha.2010.02.017. PMID: 20570358
High-resolution magnetic resonance imaging demonstrates abnormalities of motor nerves and extraocular muscles in patients with neuropathic strabismus.
Demer JL, Ortube MC, Engle EC, Thacker N
J AAPOS 2006 Apr;10(2):135-42. doi: 10.1016/j.jaapos.2005.12.006. PMID: 16678748Free PMC Article
Optic nerve hypoplasia with good visual acuity and visual field defects: a study of children of diabetic mothers.
Petersen RA, Walton DS
Arch Ophthalmol 1977 Feb;95(2):254-8. doi: 10.1001/archopht.1977.04450020055011. PMID: 836211

Diagnosis

Combined Brown syndrome and superior oblique palsy without a trochlear nerve: case report.
Yang HK, Kim JH, Kim JS, Hwang JM
BMC Ophthalmol 2017 Aug 25;17(1):159. doi: 10.1186/s12886-017-0553-9. PMID: 28841851Free PMC Article
Possible association of congenital Brown syndrome with congenital cranial dysinnervation disorders.
Ellis FJ, Jeffery AR, Seidman DJ, Sprague JB, Coussens T, Schuller J
J AAPOS 2012 Dec;16(6):558-64. doi: 10.1016/j.jaapos.2012.09.005. PMID: 23237754
Congenital superior oblique palsy and trochlear nerve absence: a clinical and radiological study.
Yang HK, Kim JH, Hwang JM
Ophthalmology 2012 Jan;119(1):170-7. Epub 2011 Sep 15 doi: 10.1016/j.ophtha.2011.06.038. PMID: 21924501
Absence of the trochlear nerve in patients with superior oblique hypoplasia.
Kim JH, Hwang JM
Ophthalmology 2010 Nov;117(11):2208-13.e1-2. Epub 2010 Jun 8 doi: 10.1016/j.ophtha.2010.02.017. PMID: 20570358
High-resolution magnetic resonance imaging demonstrates abnormalities of motor nerves and extraocular muscles in patients with neuropathic strabismus.
Demer JL, Ortube MC, Engle EC, Thacker N
J AAPOS 2006 Apr;10(2):135-42. doi: 10.1016/j.jaapos.2005.12.006. PMID: 16678748Free PMC Article

Clinical prediction guides

Congenital superior oblique palsy and trochlear nerve absence: a clinical and radiological study.
Yang HK, Kim JH, Hwang JM
Ophthalmology 2012 Jan;119(1):170-7. Epub 2011 Sep 15 doi: 10.1016/j.ophtha.2011.06.038. PMID: 21924501
Absence of the trochlear nerve in patients with superior oblique hypoplasia.
Kim JH, Hwang JM
Ophthalmology 2010 Nov;117(11):2208-13.e1-2. Epub 2010 Jun 8 doi: 10.1016/j.ophtha.2010.02.017. PMID: 20570358

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