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Basal encephalocele and morningglory · PDF fileBritishJournalofOphthalmology, 1983,67, 349-351 Basal encephalocele andmorningglory syndrome JOSEPH CAPRIOLI AND ROBERT L. LESSER Fromthe

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  • British Journal of Ophthalmology, 1983, 67, 349-351

    Basal encephalocele and morning glory syndromeJOSEPH CAPRIOLI AND ROBERT L. LESSER

    From the Department of Ophthalmology and Visual Sciences, Yale University School of Medicine,New Haven, Connecticut, USA

    SUMMARY Basal encephaloceles are often associated with other midline anomalies such as hyper-telorism, broad nasal root, cleft lip, and cleft palate. Optic disc anomalies such as pallor, dysplasia,optic pit, coloboma, and megalopapilla have been reported to occur in patients with basalencephalocele We report a case of a child with a sphenoethmoidal encephalocele and morning glorysyndrome of the optic nerve. The presence of such optic nerve anomalies with facial midlineanomalies should alert the clinician to the possible presence of a basal encephalocele.

    Basal encephaloceles are classified in accordance withtheir location as trans-sphenoidal, spheno-orbital,sphenoethmoidal, transethmoidal or, spheno-maxillary. ' Although the transethmoidal is the mostfrequent, these are all rare anomalies, accounting forabout l 5% of all encephaloceles.2

    Basal encephaloceles are often associated withother midline anomalies such as hypertelorism, broadnasal root, cleft lip, and cleft palate.' ` Optic nerveanomalies such as disc pallor, pit of the optic nervehead, coloboma, optic nerve dysplasia, and megalo-papilla have been reported in patients with basalencephalocele.'3 8 The finding of such abnormali-ties of the optic nerve along with hypertelorism andcleft lip or palate should lead to the suspicion of thepresence of basal encephalocele.We report here a case of a child with a sphenoeth-

    moidal encephalocele associated with morning glorysyndrome of the optic nerve head and macularhypoplasia.

    Case report

    A 17-month-old white male was referred to theNeuro-Ophthalmology Service in November 1976 forinvestigation of 'papilloedema' of the left eye. Hewas born with a cleft lip and palate, was normalneurologically, and had normal milestones for growthand development. The child had chronic otitis mediasince the age of 2 months, for which he had beentreated with oral antibiotics. At the age of 6 monthshe underwent successful repair of the cleft lip. At 16Correspondence to Robert L. Lesser, MD, Department ofOphthalmology and Visual Sciences, Yale University School ofMedicine, 333 Cedar Street, New Haven, Connecticut 06510. USA.

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    months of age he was admitted for repair of the cleftpalate and was found to have a soft pulsatile mass ofthe posterior pharynx extending through the palataldefect. Plain skull films and laminography of the baseof the skull revealed a midline bony defect extendingfrom the anterior wall of the sella turcica through theanterior sphenoid and ethmoid sinuses (Figs. 1, 2, 3,4).On 14 November 1976 the patient underwent bi-

    frontal craniotomy with repair of the encephaloceleby elevation of the herniated chiasmatic cistern andplacement of a wire mesh dural flap.

    Fig. 1 Lateral view ofskull showing encephaloceleprotruding into nasopharynx.

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  • Joseph Caprioli and Robert L. Lesser

    Fig. 3 Left optic nerve head ofpatient at age 17 months,with morning glory syndrome.

    Recently examination showed a 5-year-old boy ofnormal intelligence. The visual acuity was 6/7 5 in theright eye and finger counting at 4 feet (I2 m) in theleft eye. Refraction under cyclopentolate revealed a

    \c hyperopia of 2-25 dioptres and 0 75 dioptre in the; right and left eyes respectively. The motility was

    normal and the patient was orthophoric. The pupilsmeasured 4 mm in diameter in the right eye and 5 mmin the left eye; light reaction was 4+ on the right and

    Fig. 2 Sagittal diagrammatic representation ofsphenoethmoidal encephalocele with herniation ofmeninges and their contents into nasopharynx.

    The patient fixed and followed light with each eye.A moderate degree of hypertelorism was present.Refraction under cyclopentolate revealed a hyperopiaof 2-00 dioptres in each eye. The pupils were equalbut the left eye showed a more sluggish reaction tolight; a left afferent pupillary defect was present. Thecorneal diameters were 12 mm in each eye. Slit-lampexamination gave normal results. Applanationtonometry revealed pressures of 15 mm Hg in theright eye and 13 mm Hg in the left. Fundus examina-tion of the right eye was entirely normal. In the lefteye a large funnel-shaped optic disc was noted with acentral glial tuft and a raised circumpapillary ridgewhich was lightly pigmented. The vessels radiated outfrom the margin of the disc in a spoke-like fashion(Fig. 5). No foveal reflex was present and no macular Fig. 4 Laminography in coronal plane revealing midlinearchitecture could be identified. encephalocele.

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  • Basal encephalocele and morning glory syndrome

    Fig. 5 Coronal diagrammatic representation ofsphenoethmoidal encephalocele.

    1+ on the left, and there was an afferent pupillarvdefect on the left. External examination showedmoderate hypertelorism and a 2 mm ptosis on the left;there was 10 mm of levator function of both upperlids. Slit-lamp examination was unremarkable. Theright fundus was normal. The ophthalmoscopicappearance of the left optic disc was unchanged fromthe previous examination. No well differentiatedmacula could be identified.

    Discussion

    Basal encephalocele is a rare midline bony defect ofthe base of the skull which may allow protrusion ofthe meninges and their contents. Herniated meningesin such cases may be found in the nasal cavity. naso-pharynx, the sphenoid and ethmoid sinuses, the orbit.and the pterygopalatine fossa, the site depending onthe location and size of the bony defect.' Fig. 5 is adiagrammatic representation of a sphenoethmoidalencephalocele. These encephaloceles may present asa mass in the nasopharynx, masquerading as nasalpolyps or enlarged adenoids, and may be associatedwith cleft palate. In cases in which the true -identity ofsuch masses was unknown, unfortunate surgicalattempts at biopsy or repair of cleft palate have resul-ted in cerebrospinal fluid rhinorrhea, meningitis, andeven death.Two physical findings which were uniformly present

    in a series of 8 cases reported by Pollock et al.' were abroad nasal root and a congenital cystic mass locatedin the midline of the nasal or epipharyngeal space.Hypertelorism is also usually present but may be mildand inconspicuous. Other less constant associated

    findings include cleft lip, cleft palate, agenesis of thecorpus callosum, and spina bifida. "'

    Optic nerve anomalies which have been associatedwith basal encephaloceles have been reviewed byGoldhammer and Smith.6 They include aplasia.hypoplasia, pit of the optic nerve head, dysplasia.megalopapilla, and coloboma. An additional opticdisc anomaly called the morning glory syndrome hadbeen described by several authors, but to our know-ledge had never been reported in association withbasal encephalocele. Pollock et al.' reported a caseof basal encephalocele and optic disc anomaly des-cribed as a coloboma which has some of the charac-teristics of the morning glory syndrome.

    In 1970 Kindler reported 10 cases of the morningglory syndrome.9 In each case the nerve head wasfunnel shaped, contained a central white dot of glialtissue, and was 'surrounded by an elevated annulus ofchorioretinal pigment disturbance'. The retinalvessels radiate out from the edge of the disc asmultiple narrow branches. The visual acuity wasseverely diminished in all cases. Krause emphasisedthe typical features of morning glory syndrome asbeing an excavated optic disc and an elevated cir-cumpapillary ring of chorioretinal pigmentarvdisturbances. 'The presence of midline facial anomalies such as

    broad nasal root, hypertelorism. cleft lip, and cleftpalate in association with anomalies of the optic nerveshould alert the physician to the possible presence ofan otherwise unrecognised basal encephalocele.

    References

    I Pollock JA, Newton TH, Hoyt WF. Transsphenoidal andtransethmoidal encephaloceles. Radiology 1968; 90: 442-53.

    2 Ingraham FD, Swan H. Spina bifida and cranium bididum. NEngl J Med 1943; 228: 559-63.

    3 Van Nouhuys JM, Bruyn GW. Nasopharyngeal transsphenoidalencephalocele, craterlike hole in the optic disc, and agenesis ofthe corpus callosum; pneumoencephalographic visualization of acase. Psvchiatr Neurol Neurochir (Amst) 1964; 67: 243-258.

    4 Blumenfeld R, Skolnik EM. Intranasal encephaloceles. ArchOtolarvngol 1965; 82: 527-31.

    5 Davis CH Jr, Alexander E Jr. Congenital nasofrontalencephalomeningoceles and teratomas; review of seven cases. JNeurosurg 1959; 16: 365-77.

    6 Goldhammer Y, Smith JL. Optic nerve anomalies in basalencephalocele. Arch Ophthalmol 1975; 93:115-8.

    7 Lewin ML, Schuster MM. Transpalatal correction of basilarmeningocele with cleft palate. Arch Surg 1965; 90: 687-93.

    8 Walsh FB, Hoyt WF. Clinical Neuro-Ophthalmology. Baltimore:Williams and Wilkins, 1969: 673.

    9 Kindler P. Morning glory syndrome: unusual congenital opticdisc anomaly. Am J Ophthalmol 197t); 69: 376-84.

    10 Krause U. Three cases of the morning glory syndrome. ActaOphthalmol (Kbh) 1972; 50: 188-98.

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