Bilateral morning glory syndrome associated with sphenoid encephalocele

Case report

Restricted access

✓ Morning glory syndrome is a congenital anomaly of the optic disc in which the disc is enlarged and excavated, with white glial tissue in the center. A case is presented of morning glory syndrome associated with sphenoid encephalocele, median cleft lip, and agenesis of the corpus callosum. A 22-day-old boy was referred to the Wakayama Medical College Hospital for management of dyspnea due to a soft-tissue mass in the oral cavity. Magnetic resonance imaging revealed a mass extending through a bone defect in the sphenoid region and into the oral cavity. Surgical repair was attempted through a bifrontal craniotomy. A bone defect was identified in the sphenoid plate, through which the arachnoid membrane was connected to the oral cavity. Both optic nerves were elongated and adhered to the encephalocele. The wall of the meningocele was compressed digitally through the oral cavity and sutured to the dura mater of the bone defect. The operative findings suggest that a basal encephalocele protruding from a bone defect in the sphenoid plate may disturb the normal development of the optic nerve.

Article Information

Address reprint requests to: Toru Itakura, M.D., Department of Neurological Surgery, Wakayama Medical College, 7–27 Wakayama City, Japan.

© AANS, except where prohibited by US copyright law.

Headings

Figures

  • View in gallery

    Ophthalmoscopic view of the left eye showing an enlarged disc surrounded by glial tissue.

  • View in gallery

    Left: Preoperative magnetic resonance (MR) T1-weighted image demonstrating a mass with low intensity extending into the retropharyngeal region and posterior oral cavity (arrow) and agenesis of the corpus callosum. Right: Postoperative MR image showing that the meningocele protruding into the oral cavity had decreased in size.

References

  • 1.

    CaprioJi JLesser RL: Basal encephalocele and morning glory syndrome. Br J Ophthalmol 67:3493511983CaprioJi J Lesser RL: Basal encephalocele and morning glory syndrome. Br J Ophthalmol 67:349–351 1983

    • Search Google Scholar
    • Export Citation
  • 2.

    Giuffrè G: Morning glory syndrome: clinical and electro-functional study of three cases. Br J Ophthalmol 70:2292361986Giuffrè G: Morning glory syndrome: clinical and electro-functional study of three cases. Br J Ophthalmol 70:229–236 1986

    • Search Google Scholar
    • Export Citation
  • 3.

    Ingraham FDSwan H: Spina bifida and cranium bifidum: a survey of 546 cases. N Engl J Med 228:5595631943Ingraham FD Swan H: Spina bifida and cranium bifidum: a survey of 546 cases. N Engl J Med 228:559–563 1943

    • Search Google Scholar
    • Export Citation
  • 4.

    Kindler P: Morning glory syndrome: unusual congenital optic disk anomaly. Am J Ophthalmol 69:3763841970Kindler P: Morning glory syndrome: unusual congenital optic disk anomaly. Am J Ophthalmol 69:376–384 1970

    • Search Google Scholar
    • Export Citation
  • 5.

    Nawratzki ISchwartzenberg TZaubermann Het al: Bilateral morning glory syndrome with midline brain lesion in an autistic child. Metab Pediatr Syst Ophthalmol 8:35361985Nawratzki I Schwartzenberg T Zaubermann H et al: Bilateral morning glory syndrome with midline brain lesion in an autistic child. Metab Pediatr Syst Ophthalmol 8:35–36 1985

    • Search Google Scholar
    • Export Citation
  • 6.

    Pollock JANewton THHoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 90:4424531968Pollock JA Newton TH Hoyt WF: Transsphenoidal and transethmoidal encephaloceles. A review of clinical and roentgen features in 8 cases. Radiology 90:442–453 1968

    • Search Google Scholar
    • Export Citation
  • 7.

    Steinkuller PG: The morning glory disk anomaly: case report and literature review. J Pediatr Ophthalmol Strabismus 17:81871980Steinkuller PG: The morning glory disk anomaly: case report and literature review. J Pediatr Ophthalmol Strabismus 17:81–87 1980

    • Search Google Scholar
    • Export Citation
  • 8.

    Takezawa NSato MYanagisawa Tet al: [Pituitary dwarfism associated with morning glory syndrome and transsphenoidal encephalocele: a case report.] No To Hattatsu 19:4924961987 (Jpn)Takezawa N Sato M Yanagisawa T et al: [Pituitary dwarfism associated with morning glory syndrome and transsphenoidal encephalocele: a case report.] No To Hattatsu 19:492–496 1987 (Jpn)

    • Search Google Scholar
    • Export Citation
  • 9.

    Tuchmann-Duplessis HAuroux MHaegel P: Illustrated Human Embryology. New York: Springer-Verlag1982Tuchmann-Duplessis H Auroux M Haegel P: Illustrated Human Embryology. New York: Springer-Verlag 1982

    • Search Google Scholar
    • Export Citation

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 145 145 14
Full Text Views 232 232 1
PDF Downloads 69 69 1
EPUB Downloads 0 0 0

PubMed

Google Scholar