Pneumosinus dilatans of the sphenoid and visual loss: when should the optic nerve be decompressed?
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CASE REPORT
Pneumosinus dilatans of the sphenoid and visual loss: when should the optic nerve be decompressed? Gilles Danassegarane 1 & Maxime Bretonnier 1 & Julien Tinois 1 & Maïa Proisy 2 & Laurent Riffaud 1,3 Received: 6 July 2020 / Accepted: 1 October 2020 # Springer-Verlag GmbH Germany, part of Springer Nature 2020
Abstract Pneumosinus dilatans of the sphenoid sinus is a rare disorder which can be responsible for visual impairment and blindness. We present the case of an adolescent female who experienced progressive decrease in right-eye vision over 2 years. CT scan of the head showed an extensive pneumatization of the sphenoid bone extending to the lesser wing of the sphenoid and to the anterior clinoid process on the right side. MRI revealed right nerve atrophy in the optic canal and in the posterior part of the orbit. A surgical decompression of the right optic canal was performed via an intradural fronto-pterional approach. Postoperatively, her vision worsened, and at 3 years the patient was able to count fingers at 2.5 m. Our case and literature review of symptomatic sphenoidal pneumosinus dilatans confirmed that visual prognosis in such cases depended on the preoperative visual status. Early surgical decompression should be proposed whenever possible, before signs of severe visual disorders and optic atrophy. Keywords Pneumosinus dilatans . Sphenoid sinus . Vision . Surgery . Optic nerve . Optic canal
Introduction
Case report
Pneumosinus dilatans (PSD) is a rare condition of unknown origin first so-called in 1918 [1], in which dilated paranasal sinuses are lined with normal mucosa and filled with air. The frontal sinus is the most commonly affected, with presenting complaints usually aesthetic in nature (“Cro-Magnon-like” brow), and the sphenoid sinus is the most important for visual damage because of its intimate relation with the optic nerve in the optic canal [2, 3]. We report the case of an adolescent who suffered a progressive unilateral loss of vision resulting from PSD of the sphenoid sinus despite optic nerve surgical decompression, and review the literature on this rare disorder in order to determine the visual prognosis in such a situation and define the optimal strategy for surgery in terms of timing and procedure.
History and examination
* Laurent Riffaud [email protected] 1
Department of Pediatric Neurosurgery, Rennes University Hospital, Rennes, France
2
Department of Pediatric Radiology, Rennes University Hospital, Rennes, France
3
INSERM MediCIS, unit U1099 LTSI, Rennes 1 University, Rennes, France
This 14-year-old girl, with no significant medical history, complained about a gradual painless decrease of vision in her right eye (RE) over several months, before she was referred to the Ophthalmological Department. Her birth and developmental history were normal, with no symptoms suggesting a neurological, ophthalmological, or endocrinal disorder. At first admission, visual acuity was 20/50 P5 in the RE and 20/12.5 P1.5 in the left eye (LE). Fundus examination showed
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