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Magnetic Resonance Imaging in 14 Patients with Congenital Oculomotor Nerve Palsy

  • Hee Kyung Yang
  • Jae Hyoung Kim
  • Jeong-Min Hwang
Original Article
  • 93 Downloads

Abstract

Purpose

To elucidate the imaging findings of congenital oculomotor nerve palsy by evaluating the oculomotor nerve and extraocular muscles on magnetic resonance (MR) imaging in a relatively large series of 14 patients with congenital oculomotor nerve palsy.

Methods

Ophthalmologic examination of 14 consecutive patients diagnosed with congenital oculomotor nerve palsy, and MR imaging of the orbit and oculomotor nerves were performed.

Results

Of the 14 patients with congenital oculomotor nerve palsy, 13 patients (93%) were unilateral and 12 patients (86%) had incomplete palsy. On MR imaging, 5 patients (36%) had hypoplasia or aplasia of the oculomotor nerve with variable degrees of extraocular muscle atrophy on the affected side. Of the 14 patients, nine (64%) had normal-sized oculomotor nerves with or without muscle atrophy. The most frequently involved muscles were the inferior rectus (64%) and medial rectus (50%), followed by the superior rectus (29%) and inferior oblique muscles (14%). None of the 14 patients showed an abnormal abducens nerve.

Conclusion

Congenital oculomotor nerve palsy was mostly incomplete and hypoplasia or aplasia of the oculomotor nerve was apparent in one third of patients showing variable degrees of extraocular muscle atrophy, mostly of the medial rectus and inferior rectus muscles.

Keywords

Congenital hypoplasia Aplasia Congenital cranial dysinnervation disorder Third nerve palsy Congenital strabismus 

Notes

Funding

This work was supported by the National Research Foundation of Korea (NRF) grant funded by the Korea Government (MSIP) (No. 2017R1A2B4011450) (JMH) and by the SNUBH research fund (No. 02-2016-051).

Conflict of interest

H.K. Yang, J.H. Kim and J.-M. Hwang declare that they have no competing interests.

References

  1. 1.
    Gutowski NJ, Bosley TM, Engle EC. 110th ENMC International Workshop: the congenital cranial dysinnervation disorders (CCDDs). Naarden, The Netherlands, 25–27 October, 2002. Neuromuscul Disord. 2003;13:573–8.CrossRefGoogle Scholar
  2. 2.
    Kim JH, Hwang JM. Imaging of cranial nerves III, IV, VI in congenital cranial dysinnervation disorders. Korean J Ophthalmol. 2017;31:183–93.CrossRefGoogle Scholar
  3. 3.
    Demer JL, Ortube MC, Engle EC, Thacker N. High-resolution magnetic resonance imaging demonstrates abnormalities of motor nerves and extraocular muscles in patients with neuropathic strabismus. J AAPOS. 2006;10:135–42.CrossRefGoogle Scholar
  4. 4.
    Wu TE, Isenberg SJ, Demer JL. Magnetic resonance imaging demonstrates neuropathology in congenital inferior division oculomotor palsy. J AAPOS. 2006;10:473–5.CrossRefGoogle Scholar
  5. 5.
    Lim KH, Engle EC, Demer JL. Abnormalities of the oculomotor nerve in congenital fibrosis of the extraocular muscles and congenital oculomotor palsy. Invest Ophthalmol Vis Sci. 2007;48:1601–6.CrossRefGoogle Scholar
  6. 6.
    Kim JH, Hwang JM. Magnetic resonance imaging in three patients with congenital oculomotor nerve palsy. Br J Ophthalmol. 2009;93:1266–7.CrossRefGoogle Scholar
  7. 7.
    Yang HK, Choi BY, Kim JH, Koo JW, Chang MY, Hwang JM. CHARGE syndrome with oculomotor nerve palsy. J AAPOS. 2015;19:555–7.CrossRefGoogle Scholar
  8. 8.
    Yang HK, Kim JH, Hwang JM. Congenital superior oblique palsy and trochlear nerve absence: a clinical and radiological study. Ophthalmology. 2012;119:170–7.CrossRefGoogle Scholar
  9. 9.
    Kim JH, Hwang JM. Presence of the abducens nerve according to the type of Duane’s retraction syndrome. Ophthalmology. 2005;112:109–13.CrossRefGoogle Scholar
  10. 10.
    Yang HK, Kim JH, Hwang JM. Abducens nerve in patients with type 3 Duane’s retraction syndrome. PLoS ONE. 2016;11:e150670.CrossRefGoogle Scholar
  11. 11.
    Kim JH, Hwang JM. Hypoplastic oculomotor nerve and absent abducens nerve in congenital fibrosis syndrome and synergistic divergence with magnetic resonance imaging. Ophthalmology. 2005;112:728–32.CrossRefGoogle Scholar
  12. 12.
    Engle EC, Goumnerov BC, McKeown CA, Schatz M, Johns DR, Porter JD, Beggs AH. Oculomotor nerve and muscle abnormalities in congenital fibrosis of the extraocular muscles. Ann Neurol. 1997;41:314–25.CrossRefGoogle Scholar
  13. 13.
    Brodsky MC, Pollock SC, Buckley EG. Neural misdirection in congenital ocular fibrosis syndrome: implications and pathogenesis. J Pediatr Ophthalmol Strabismus. 1989;26:159–61.Google Scholar
  14. 14.
    Ing EB, Sullivan TJ, Clarke MP, Buncic JR. Oculomotor nerve palsies in children. J Pediatr Ophthalmol Strabismus. 1992;29:331–6.Google Scholar
  15. 15.
    Norman AA, Farris BK, Siatkowski RM. Neuroma as a cause of oculomotor palsy in infancy and early childhood. J AAPOS. 2001;5:9–12.CrossRefGoogle Scholar

Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Hee Kyung Yang
    • 1
  • Jae Hyoung Kim
    • 2
  • Jeong-Min Hwang
    • 1
  1. 1.Department of Ophthalmology, Seoul National University College of MedicineSeoul National University Bundang HospitalSeongnamKorea (Republic of)
  2. 2.Department of Radiology, Seoul National University College of MedicineSeoul National University Bundang HospitalSeongnamKorea (Republic of)

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