Comments on: Surgical Management of Idiopathic Intracranial Hypertension in Pregnancy

Letters to the Editor Arif O. Khan, MD Eye Institute, Cleveland Clinic Abu Dhabi, Abu Dhabi, UAE; and Department of Ophthalmology, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Cleveland, Ohio. The author reports no conflicts of interest. REFERENCES 1. Berry JC, Rangu N, Harvey BJ, O'Brien JC. Case of an inadvertent filtering bleb in Marfan syndrome resulting in clinical hypotony. J Neuroophthalmol. 2023. Epub ahead of print. Comments on: Surgical Management of Idiopathic Intracranial Hypertension in Pregnancy I n their excellent article, Karimaghaei et al1 provide a table of patients reported in the literature (up to August 2021) who had undergone surgery for idiopathic intracranial hypertension (IIH) during pregnancy. Since the time of that review, we have reported the case of a patient with fulminant IIH in pregnancy who was treated surgically with good outcome.2 As suggested by Karimaghaei et al,1 our patient required multidisciplinary coordination of her care, and we wish to draw readers' attention to the added challenge posed by the fulminant presentation during pregnancy. Surgical Management of Idiopathic Intracranial Hypertension in Pregnancy: Response T yndel et al1 discuss their successful management of a 13-week pregnant 28-year-old woman presenting with fulminant idiopathic intracranial hypertension (IIH) through multidisciplinary coordination with neurology, high-risk obstetrics, and neurosurgery. We agree that aggressive treatment is warranted for acute and severe (i.e., fulminant) IIH, and the most important (but often underrecognized) temporizing approach is the placement of a lumbar drain until definitive surgery can be performed.2 The choice of surgical procedure in IIH, including fulminant disease, is often dictated by the availability of local surgical expertise for a specific procedure (e.g., optic nerve sheath fenestration, venous sinus stenting, and cerebrospinal fluid shunt).1,2 Another point that merits discussion is the decision to use acetazolamide during pregnancy.1 Many providers prefer to avoid acetazolamide especially during the first 20 weeks of gestation because of the category C classification of the drug e284 2. Shawaf S, Noureddin B, Khouri A, Traboulsi EI. A family with a syndrome of ectopia lentis, spontaneous filtering blebs, and craniofacial dysmorphism. Ophthalmic Genet. 1995;16:163–169. 3. Patel N, Khan AO, Mansour A, et al. Mutations in ASPH cause facial dysmorphism, lens dislocation, anterior-segment abnormalities, and spontaneous filtering blebs, or Traboulsi syndrome. Am J Hum Genet. 2014;94:755–759. 4. Jones G, Johnson K, Eason J, et al. Traboulsi syndrome caused by mutations in ASPH: an autosomal recessive disorder with overlapping features of Marfan syndrome. Eur J Med Genet. 2022;65:104572. 5. Kulkarni N, Lloyd IC, Ashworth J, Biswas S, Black GCM, ClaytonSmith J; NIHR BioResource Consortium. Traboulsi syndrome due to ASPH mutation: an under-recognised cause of ectopia lentis. Clin Dysmorphol. 2019;28:184–189. Felix Tyndel, MD, MA, FRCPC, Richard Wennberg, MD, PhD, FRCPC, Ivan Radovanovic, MD, PhD, FRCSC Division of Neurology (FT), University of Toronto, Toronto Western Hospital, Toronto, Canada; Division of Neurology (RW), University of Toronto, Toronto, Canada; and Division of Neurosurgery (IR), University of Toronto, Toronto, Canada. The authors report no conflicts of interest. REFERENCES 1. Karimaghaei S, Raviskanthan S, Karimaghaei C, et al. Surgical management of idiopathic intracranial hypertension in pregnancy. J Neuroophthalmol. 2022;42:419–427. 2. Tyndel F, Steriade C, Gallo A, Wennberg R, Radovanovic I. Fulminant idiopathic intracranial hypertension in pregnancy. Case Rep Neurol. 2022;14:251–255. as a possible teratogen.2 We acknowledge, however, that there are limited data on possible teratogenicity in humans for acetazolamide, and we agree with Tyndel et al that a multidisciplinary approach, including individual risk-benefit analysis, is appropriate for fulminant disease in general but especially in pregnancy. Sam Karimaghaei, MD, Subahari Raviskanthan, MBBS, Cina Karimaghaei, MD, Peter W. Mortensen, MD, Amina I. Malik, MD, Yi J. Zhang, MD, Andrew G. Lee, MD Department of Ophthalmology (SK), Harvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas; Department of Ophthalmology (SR, PWM, AIM, AGL), Blanton Eye Institute, Houston Methodist Hospital, Houston, Texas; Department of Neuro-Ophthalmology (SR), Royal Victorian Eye and Ear Hospital, Melbourne, Australia; Department of Neurology (SR), Alfred Health, Melbourne, Australia; Department of Neurology (SR), Royal Melbourne Hospital, Melbourne, Australia; Department of Ophthalmology (CK, AGL), University of Texas Medical Branch, Galveston, Texas; Department of Neurosurgery (YJZ), Houston Methodist Hospital, Houston, Texas; Departments of Ophthalmology, Neurology, and Neurosurgery (AGL), Weill Cornell Medicine, New York, New York; University of Texas MD Anderson Cancer Center (AGL), Houston, Texas; Texas A and M College of Medicine (AGL), Bryan, Texas; and Department of Ophthalmology (AGL), The University of Iowa Hospitals and Clinics, Iowa City, Iowa. The authors report no conflicts of interest. Letters to the Editor: J Neuro-Ophthalmol 2024; 44: e282-286 Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited.