Unveiling a rare aetiology of secondary acquired nasolacrimal duct obstruction: A case of morning glory syndrome with contralateral naso-ethmoidal encephalocele

Muthukrishnan Vallinayagam; Shravya C Balla; Anujeet Paul; Srikanth Krishnagopal

doi:10.4103/ijo.IJO_2378_20

CASE REPORT

Year : 2021 | Volume : 1 | Issue : 2 | Page : 373-375

Unveiling a rare aetiology of secondary acquired nasolacrimal duct obstruction: A case of morning glory syndrome with contralateral naso-ethmoidal encephalocele

Muthukrishnan Vallinayagam, Shravya C Balla, Anujeet Paul, Srikanth Krishnagopal
Department of Ophthalmology, Mahatma Gandhi Medical College and Research Institute, Sri Balaji Vidyapeeth, Pondicherry, India

Date of Submission	22-Jul-2020
Date of Acceptance	18-Sep-2020
Date of Web Publication	01-Apr-2021

Correspondence Address:
Dr. Muthukrishnan Vallinayagam
Department of Ophthalmology, Mahatma Gandhi Medical College and Research Institute, Pondicherry, Sri Balaji Vidyapeeth, Pondicherry - 607 402
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_2378_20

Abstract

Morning Glory Syndrome (MGS) is a well-established association of basal encephalocele and usually presents with midline cranio-facial anomalies. 45-year-old female presented with Secondary Acquired Nasolacrimal Duct Obstruction (SANDO) in left eye. She had a broad nasal bridge, hypertelorism and a swelling inferomedial to medial canthus. Fundus examination in right eye showed Morning Glory disc anomaly. CT of the orbit and brain disclosed left naso-ethmoidal encephalocele. CT Dacryocystography showed partial dehiscence of nasolacrimal duct bilaterally. The patient underwent surgical repair of encephalocele followed by resolution of epiphora. The association of MGS with contralateral naso-ethmoidal encephalocele and SANDO is presented for its rarity.

Keywords: Morning glory syndrome, naso-ethmoidal encephalocele, Secondary Acquired Nasolacrimal Duct Obstruction

How to cite this article:
Vallinayagam M, Balla SC, Paul A, Krishnagopal S. Unveiling a rare aetiology of secondary acquired nasolacrimal duct obstruction: A case of morning glory syndrome with contralateral naso-ethmoidal encephalocele. Indian J Ophthalmol Case Rep 2021;1:373-5

How to cite this URL:
Vallinayagam M, Balla SC, Paul A, Krishnagopal S. Unveiling a rare aetiology of secondary acquired nasolacrimal duct obstruction: A case of morning glory syndrome with contralateral naso-ethmoidal encephalocele. Indian J Ophthalmol Case Rep [serial online] 2021 [cited 2023 Mar 29];1:373-5. Available from: https://www.ijoreports.in/text.asp?2021/1/2/373/312376

Morning Glory Syndrome (MGS) is a congenital optic disc anomaly with a funnel-shaped excavation at the optic disc with central glial tuft, surrounded by a grey-white annulus of chorioretinal pigmentation and abnormal vasculature arising from optic disc.^[1],[2],[3] The characteristic midline craniofacial anomalies such as corpus callosal agenesis, panhypopituitarism and basal encephalocele are the classical conglomeration of features in MGS.^[3] Encephaloceles are congenital anomalies where a portion of the central nervous system herniates through a calvarial defect.^[1] They are occasionally associated with MGS.^[4] We report an unusual presentation of MGS with contralateral naso-ethmoidal encephalocele and Secondary Acquired Nasolacrimal Duct Obstruction (SANDO).

Case Report

A 45-year-old female presented with watery discharge in the left eye for twenty years. There was history suggestive of lacrimal abscess 5 years back, for which incision and drainage was performed elsewhere. She had poor vision in right eye since childhood. The visual acuity was perception of light and 20/20 in OD and OS, respectively. She had orbital asymmetry, hypertelorism, broad nasal bridge and a compressive soft tissue swelling inferomedial to medial canthus on the left side [Figure 1]a. Regurgitation test was positive [Figure 1]b. Lacrimal syringing confirmed blockage of the left nasolacrimal duct, whereas the right was patent. Fundus examination of the right eye revealed a pale funnel-shaped excavation of the optic disc with central gliosis and arteriolar attenuation. Vessels resembling spokes of a wheel with sheathing and a concentric ring of pigment surrounding the optic disc were observed [Figure 2].

Figure 1: (a) Facial features depicting hypertelorism, broad nasal bridge and soft tissue swelling inferomedial to medial canthus on the left side. (b) Positive regurgitation test

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Figure 2: Colour fundus photo of the right eye showing Morning Glory disc anomaly with funnel shaped excavation, central gliosis, peripapillary pigmentation and “spokes of wheel” vasculature with sheathing

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CT of brain and orbit revealed a large bony defect in the floor of the anterior cranial fossa and cribriform plate. A portion of the frontal lobe was noted to herniate through the defect into anterior ethmoidal sinus and medial canthal region [Figure 3]a. CT Dacryocystography showed that the left lacrimal sac was displaced superiorly and there was bilateral dehiscence of proximal part of nasolacrimal duct [Figure 3]b. The patient underwent bifrontal craniotomy for excision of encephalocele and repair of the defect with tensor fascia lata graft [Figure 4]a, following which there was a notable resolution of epiphora [Figure 4]b. An observant approach was chosen for the lacrimal sac pathology due to spontaneous remission of epiphora and to avoid inadvertent neurological sequelae of surgical intervention in an asymptomatic patient.

Figure 3: (a) CT of brain and orbit (coronal view) showing bony defect with herniation of frontal lobe and meninges into the anterior ethmoidal sinus and medial canthal region. (b) CT Dacryocystography showed superior displacement of left lacrimal sac and bilateral dehiscence of proximal part of nasolacrimal duct (Dotted arrows)

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Figure 4: (a) Postoperative CT of Brain and orbit (coronal view) showing repaired bony defect with tensor fascia graft. (b) Postoperative picture showing resolution of symptoms with absence of epiphora

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Discussion

Morning Glory Syndrome (MGS) is characterised by a symmetrical excavation of optic disc with a central mass of glial tissue, surrounded by ring of chorioretinal pigment with vessels radiating from the margin of the disc in a spoke-like fashion.^[3] An unilateral presentation with poor vision and a female preponderance is characteristic.^[5] Macular involvement or anisometric amblyopia may explain visual loss in the majority.^[3] An analogous galaxy of features typified MGS in this case.

MGS has been associated with persistent hyperplastic primary vitreous, pupillary membrane remnants, aniridia, microphthalmos, cataract, foveal hypoplasia, lid hemangiomas, ciliary body cysts, Duane's syndrome and preretinal gliosis.^[1],[2],[5] Serous and rhegmatogenous retinal detachments have been reported in one-third of patients.^[5] SANDO forms a conspicuous association in this patient with MGS and this conjunction has not been reported to the best of our knowledge.

Encephaloceles are caused by a defective development of calvarial bone and herniation of intracranial contents.^[1],[6] Encephaloceles are classified as sincipital and basal encephaloceles.^[6] MGS has been customarily associated with basal encephalocoele.^{[1],[3],[4],[5],[7]} The consistently reported features of encephalocele are hypertelorism, broad nasal root and a compressible cystic mass in the nasal or epipharyngeal space.^[7] Naso-ethmoidal encephalocele is consistent with the afore-mentioned features. MGS coupled with a contralateral naso-ethmoidal encephalocele is an unusual entity. The herniated intracranial tissue in the ethmoid region and the displaced lacrimal sac accounted for the palpable mass.

SANDO is an unprecedented accompaniment of MGS and Naso-ethmoidal encephalocele. We speculate this to have resulted from the mass effect of the encephalocele on the lacrimal drainage pathway or due to the dehiscence of proximal part of nasolacrimal duct. Bilateral dehiscence of the bony nasolacrimal duct was noted in this patient, but this is quite a common pre-operative finding even in normal individuals.^[8] The resolution of epiphora after repair of encephalocele offers corroborative evidence.

The various reports in the literature have advocated surgical management of encephalocele to prevent infection and mass effect, opting for an endoscopic and trans-sphenoidal approach.^[4] A bi-frontal craniotomy with excision of herniated mass and anterior cranial fossa repair resulted in a gratifying outcome in this case.

Conclusion

MGS may be associated with naso-ethmoidal encephalocele, which can be contralateral. SANDO may be a rare synchronous manifestation, possibly due to the pressure effect on the lacrimal sac. Neuroimaging may reveal the intricate relation of the herniated mass to the lacrimal sac, precluding surgical intervention for SANDO.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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