Asymptomatic idiopathic intracranial hypertension post female to male gender transition

2021 ◽  
Vol 14 (12) ◽  
pp. e246454
Author(s):  
Geoffrey Peter Ronan ◽  
Brian Sweeney

A 23- year-man post female to male (FTM) gender transition was found to have bilateral papilloedema at a routine optician visit. The patient was referred on for formal ophthalmological and neurological assessments. Optical coherence tomography (OCT) confirmed the presence of bilateral papilloedema. The patient was entirely asymptomatic and had no medical history. He took testosterone intramuscularly once per month. Neurological examination was otherwise normal. Investigations including routine blood panels, CT brain, MRI brain and cerebral MR venogram were all normal. Lumbar puncture yielded cerebrospinal fluid (CSF) normal in appearance but demonstrated raised intracranial pressure. In the absence of other causative aetiologies a diagnosis of idiopathic intracranial hypertension (IIH) was made. Treatment was commenced with acetazolamide and the patient was discharged with outpatient ophthalmological and neurological follow-up.

2021 ◽  
Vol 10 (8) ◽  
pp. 555-557
Author(s):  
Praveena Kiran Kher ◽  
Jigna Mukesh Motwani ◽  
Sachin Vishwanath Daigavane

Majority of idiopathic intracranial hypertension (IIH) cases present with bilateral papilloedema. Unilateral papilloedema is a rare condition in IIH. This is a report of one such unusual case. Papilloedema is clinically defined as swelling of the optic nerve head secondary to raised intracranial pressure.1 Disc oedema is a non-specific term that includes papilloedema but also refers to a disc swollen from other causes. Classically, presence of papilloedema is most commonly due to an intracranial space occupying lesion leading to raised intracranial pressure; however, it may be caused due to other aetiologies such as hydrocephalus, shunt failures, idiopathic intracranial hypertension and drugs.2 Idiopathic intracranial hypertension, also known as pseudotumour cerebri, is a disorder characterised by increased intracranial pressure of unclear pathogenesis in the absence of other structural and obstructive lesions. This is a case of acute established unilateral disc oedema secondary to idiopathic intracranial hypertension.


Author(s):  
Ayman G. Elnahry ◽  
Gehad A. Elnahry

Background: In the current coronavirus disease 2019 (COVID-19) pandemic, health systems are struggling to prioritize care for affected patients, however, physicians globally are also attempting to maintain care for other lessthreatening medical conditions that may lead to permanent disabilities if untreated. Idiopathic intracranial hypertension (IIH) is a relatively common condition affecting young females that could lead to permanent blindness if not properly treated. In this article, we provide some insight and recommendations regarding the management of IIH during the pandemic. Methods: The diagnosis, follow-up, and treatment methods of IIH during the COVID-19 pandemic period are reviewed. COVID-19 as a mimic of IIH is also discussed. Results: Diagnosis and follow-up of papilledema due to IIH during the COVID-19 pandemic can be facilitated by nonmydriatic fundus photography and optical coherence tomography. COVID-19 may mimic IIH by presenting as cerebral venous sinus thrombosis, papillophlebitis, or meningoencephalitis, so a high index of suspicious is required in these cases. When surgical treatment is indicated, optic nerve sheath fenestration may be the primary procedure of choice during the pandemic period. Conclusion: IIH is a serious vision threatening condition that could lead to permanent blindness and disability at a relatively young age if left untreated. It could be the first presentation of a COVID-19 infection. Certain precautions during the diagnosis and management of this condition could be taken that may allow appropriate care to be delivered to these patients while minimizing the risk of coronavirus infection.


2011 ◽  
Vol 115 (1) ◽  
pp. 165-170 ◽  
Author(s):  
Zhijun Yang ◽  
Bo Wang ◽  
Chungcheng Wang ◽  
Pinan Liu

Object The authors aim to identify the characteristics of primary spontaneous CSF rhinorrhea and propose a hypothesis for its pathogenesis. Methods Between 2003 and 2009, 21 patients diagnosed with primary spontaneous CSF rhinorrhea underwent surgery in the authors' hospital. The clinical aspects were retrospectively reviewed, and their characteristics were analyzed. Results There were 18 women and 3 men, whose ages ranged from 37 to 74 years (mean 53 years). Body mass index (BMI) ranged from 22 to 58.8 kg/m2 (mean 31.2 kg/m2). Eighteen patients (85.7%) were overweight, and 18 (85.7%) suffered from headache or tinnitus before rhinorrhea. Radiological images revealed fully or partially empty sellae in 14 patients (66.7%). The preoperative intracranial pressure (ICP) ranged from 11 to 28 cm H2O (mean 17.6 cm H2O), while the postoperative ICP ranged from 21 to 32 cm H2O (mean 25.5 cm H2O, p < 0.01). An endoscopeassisted transnasal approach was chosen for the repair. Postoperatively, in 95.2% of patients a cure was achieved. Rhinorrhea recurred in only 1 patient, and a leakage from a new defect occurred in another patient 4 years after the operation. Both patients underwent additional surgery, which was successful. The follow-up period varied from 5 to 75 months with a mean of 34 months. Conclusions All patients had direct or indirect evidence of elevated ICP, most patients presented with symptoms of idiopathic intracranial hypertension (IIH), and most patients were women and obese. Primary spontaneous CSF rhinorrhea may be due to IIH, and it is a rare symptom of IIH. When treating or monitoring these patients during follow-up, ICP should be controlled, and other symptoms of IIH should be noted.


2020 ◽  
pp. 6054-6059
Author(s):  
Alexandra Sinclair

Idiopathic intracranial hypertension (IIH) (also called pseudotumour cerebri and, previously, benign intracranial hypertension) is a syndrome of raised intracranial pressure in the absence of an intracranial mass lesion, enlargement of the cerebral ventricles, or venous sinus thrombosis. IIH affects predominantly obese women of childbearing age (>90%). The condition has considerable morbidity from permanent visual loss (up to 25% of cases) and chronic disabling headaches, which result in poor quality of life. Patients presenting acutely with papilloedema must be evaluated urgently for secondary causes of raised intracranial pressure (e.g. space occupying lesion and venous thrombosis). After this, the priority is to assess accurately the threat to vision. In most patients, the condition becomes chronic and the disease burden is mostly from chronic headaches, which need active management, alongside visual monitoring. This chapter does not cover paediatric IIH.


Author(s):  
Jonathan A. Micieli ◽  
Beau B. Bruce ◽  
Caroline Vasseneix ◽  
Richard J. Blanch ◽  
Damian E. Berezovsky ◽  
...  

ABSTRACT:Objectives:To determine whether optic disc hemorrhages (ODH) and cotton wool spots (CWS) at presentation are associated with worse visual outcomes in pediatric patients with idiopathic intracranial hypertension (IIH).Methods:Retrospective institutional review of 100 eyes of 50 consecutive pediatric IIH patients (aged 16 years or less) who had baseline optic disc photographs before or within 30 days of their diagnostic lumbar puncture and initiation of medical treatment. Optic disc photographs were independently graded by three ophthalmologists in a standardized manner. Visual function was assessed using visual acuity (VA) and visual field grade (VFG).Results:At least one ODH was found in 41% of eyes, at least one CWS was found in 27% of eyes, and 20% of eyes had both ODH and CWS. At presentation, Frisén grade was associated with the presence of CWS (p = 0.013) and showed no association with ODH (p = 0.060). When controlling for Frisén grade, ODH and CWS were not associated with worse VA or VFG at final follow-up. Severe ODH were associated with worse VA and VFG at presentation (p < 0.03), but not at final follow-up. Severe CWS at presentation was strongly associated with a worse Humphrey mean deviation of 5.0 dB (95% confidence interval 1.6–8.3) at final follow-up (p = 0.002).Conclusion:When controlling for the severity of papilledema, ODH do not provide any additional prognostic value in pediatric IIH patients. Frisén grade and severe CWS at presentation were independently associated with worse visual outcomes at the final follow-up.


Neurosurgery ◽  
2007 ◽  
Vol 60 (6) ◽  
pp. 1039-1044 ◽  
Author(s):  
Basel Abu-Serieh ◽  
Keyvan Ghassempour ◽  
Thierry Duprez ◽  
Christian Raftopoulos

Abstract OBJECTIVE Recent reports have shown promising short- to medium-term results in patients with refractory idiopathic intracranial hypertension (IIH) treated using the stereotactic ventriculoperitoneal shunting (SVPS) technique. However, the long-term clinical efficacy of this technique remains questionable. This report provides the long-term results of SVPS in treating refractory IIH patients. METHODS We reviewed the medical charts of nine consecutive patients (mean age, 26.4 yr; range, 4–63 yr) treated using either a frame-based or frameless SVPS technique for IIH. RESULTS The mean postoperative follow-up period was 44.3 months (range, 6–110 mo). Before shunting procedures were performed, each patient presented with intractable headache, and five patients (55.6%) had mild to moderate visual deficits. The last follow-up assessment showed that after shunting was performed, eight patients (89%) were headache-free. Only one patient had recurrent headache; however, this patient's pain was much less frequent and severe than before the shunting procedure was completed and was concomitant with recent weight increase. Visual deficits were resolved in three patients and remained stable in two who already had optic nerve atrophy before shunting was completed. Twelve SVPS procedures were performed on our patients. Nine shunt revisions were needed in six patients because of infection (n = 5, including two revisions in one patient), valve dysfunction (n = 2), distal obstruction (n = 1), and ventricular catheter malpositioning (n = 1). No patient had proximal catheter obstruction. CONCLUSION Given the favorable long-term outcome of the SVPS technique for refractory IIH, we are encouraged to apply this procedure on our patients. More invasive approaches should be reserved for patients who have SVPS failure.


2018 ◽  
Vol 130 (1) ◽  
pp. 136-144 ◽  
Author(s):  
Michael Karsy ◽  
Hussam Abou-Al-Shaar ◽  
Christian A. Bowers ◽  
Richard H. Schmidt

OBJECTIVEIdiopathic intracranial hypertension (IIH), or pseudotumor cerebri, is a complex and difficult-to-manage condition that can lead to permanent vision loss and refractory headaches if untreated. Traditional treatment options, such as unilateral ventriculoperitoneal (VP) or lumboperitoneal (LP) shunt placement, have high complication and failure rates and often require multiple revisions. The use of bilateral proximal catheters has been hypothesized as a method to improve shunt survival. The use of stereotactic technology has improved the accuracy of catheter placement and may improve treatment of IIH, with fewer complications and greater shunt patency time.METHODSThe authors performed a retrospective chart review for all patients with IIH who underwent stereotactic placement of biventriculoperitoneal (BVP) shunt catheters from 2008 to 2016 at their institution. Bilateral proximal catheters were Y-connected to a Strata valve with a single distal catheter. We evaluated clinical, surgical, and ophthalmological variables and outcomes.RESULTSMost patients in this series of 34 patients (mean age 34.4 ± 8.2 years, mean body mass index 38.7 ± 8.3 kg/m2; 91.2% were women) undergoing 41 shunt procedures presented with headache (94.1%) and visual deficits (85.3%). The mean opening pressure was 39.6 ± 9.0 cm H2O. In addition, 50.0% had undergone previous unilateral shunt placement, and 20.6% had undergone prior optic nerve sheath fenestration. After BVP shunt placement, there were no cases of proximal catheter obstruction and only a single case of valve obstruction at 41.9 months, with a mean follow-up of 24.8 ± 20.0 months. Most patients showed improvement in their headache (82.4%), subjective vision (70.6%), and papilledema (61.5% preoperatively vs 20.0% postoperatively, p = 0.02) at follow-up. Additional primary complications included 4 patients with migration of their distal catheters out of the peritoneum (twice in 1 patient), and an infection of the distal catheter after catheter dislodgment. The proximal obstructive shunt complication rate in this series (2.9%) was lower than that with LP (53.5%) or unilateral VP (37.8%) shunts seen in the literature.CONCLUSIONSThis small series suggests that stereotactic placement of BVP shunt catheters appears to improve shunt survival rates and presenting symptoms in patients with IIH. Compared with unilateral VP or LP shunts, the use of BVP shunts may be a more effective and more functionally sustained method for the treatment of IIH.


2015 ◽  
Vol 8 (6) ◽  
pp. 594-595 ◽  
Author(s):  
Michael R Levitt ◽  
Felipe C Albuquerque ◽  
Andrew F Ducruet ◽  
M Yashar S Kalani ◽  
Celene B Mulholland ◽  
...  

BackgroundThe effect of dural venous sinus stenting has been investigated for the treatment of idiopathic intracranial hypertension (IIH) but the effect of stenting on the long-term patency of the cortical draining veins, especially the vein of Labbé (VOL), remains unknown.MethodsWe reviewed our database of 38 patients with IIH with 41 stented dural venous sinuses between October 2006 and December 2014. Demographic, clinical, and radiological data were reviewed. Follow-up catheter angiographic data were included when available.ResultsStent placement spanned the ostium of the VOL in 35 patients (92.1%), with no immediate effect on the drainage of the VOL. Follow-up angiography (mean 35.1 months, range 1.7–80.7 months) was available in 24 patients, 21 of whom had stents spanning the VOL ostium. The VOL remained patent without occlusion or drainage alteration in all 21 patients. There were no immediate or long-term intracranial complications.ConclusionsDural venous sinus stenting for patients with IIH does not affect the immediate or long-term patency of the VOL and is not associated with intracranial complications.


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