Papilledema Differential Diagnosis

Papilledema Differential Diagnosis

Caused by elevated pressure in the cerebrospinal fluid (CSF):

Intracranial Mass Lesions Tumors (gliomas, metastases, meningiomas)

Abscesses

Hematomas

Cerebral Edema Trauma

Hypoxic-ischemic injury

Hypertensive encephalopathy

Hydrocephalus Obstructive (e.g., aqueductal stenosis)

Communicating (e.g., post-meningitic)

Idiopathic Intracranial Hypertension (IIH) Especially in young obese females

Cerebral Venous Sinus Thrombosis May mimic IIH

Meningitis/Encephalitis Infectious or inflammatory processes causing brain swelling

Unilateral or BilateralOptic Neuritis

Multiple sclerosis

Neuromyelitis optica (NMOSD)

MOG-antibody associated disease

Ischemic Optic Neuropathy

Non-arteritic (NAION): common in elderly, associated with vascular risk

Arteritic (AAION): e.g., giant cell arteritis

Infiltrative or Neoplastic Optic Neuropathy

Lymphoma

Leukemia

Sarcoidosis

Toxic/Nutritional Optic Neuropathy

Methanolethambutol

Vitamin B12 deficiency

Congenital Pseudopapilledema

Optic disc drusen

Hypermetropic crowded discs

Other Mimickers

Papillophlebitis (in young patients with retinal vein congestion)

Uveitis (posterior)

Hypertensive retinopathy (Grade IV with disc edema)

Neuroimaging: MRI/MRV to rule out mass lesion, thrombosis

Lumbar Puncture: Measure opening pressure, CSF analysis (after imaging)

OCT: Assess retinal nerve fiber layer thickness

Visual fields: Enlargement of blind spot common in papilledema

Fundus autofluorescence/ultrasound: To detect optic disc drusen

Review

A comprehensive review_article by Susan P. Mollan offers a timely update on the diagnosis and management of papilledema, a condition characterized by bilateral optic disc swelling due to raised intracranial pressure. The article is particularly relevant in the context of increasing cases of idiopathic intracranial hypertension (IIH), strongly correlated with the global rise in obesity 1)

A standout strength of this review lies in its emphasis on differentiating true papilledema from pseudopapilledema, a common diagnostic pitfall. The discussion on the role of optical coherence tomography (OCT) is especially valuable. The inclusion of recent imaging biomarkers and structural OCT changes improves clinical accuracy in distinguishing optic disc edema from congenital anomalies like buried optic nerve head drusen.

The article also highlights the multidisciplinary nature of papilledema care. Effective management relies on collaboration between ophthalmologists and neurologists, with shared responsibilities in both diagnosis and treatment—especially crucial when symptoms such as visual field defects and chronic headache are present.

However, the review could have benefitted from a more in-depth exploration of emerging therapies for cerebrospinal fluid (CSF) regulation. Although newer treatment pathways are mentioned, details on pharmacologic or surgical innovations are limited. Further commentary on the role of neuroimaging advancements, particularly with high-resolution MRV (magnetic resonance venography), would have added a more complete clinical picture.

In conclusion, this article is an essential read for any clinician managing patients with suspected raised intracranial pressure. It combines updated diagnostic strategies with practical insights into team-based care, although it leaves room for more discussion on therapeutic frontiers.


Difficulties occur in the differential diagnosis of papilledema against similar changes of the optic nerve head seen during ophthalmoscopy 2)


Causes of papilledema include intracranial tumors, idiopathic intracranial hypertension (pseudotumor cerebri), subarachnoid hemorrhagesubdural hematoma and intracranial inflammationOptic disc edema may also occur from many conditions other than papilledema, including central retinal artery or vein occlusion, congenital structural anomalies, and optic neuritis 3).


1)

Mollan SP. Papilledema. Continuum (Minneap Minn). 2025 Apr 1;31(2):436-462. doi: 10.1212/CON.0000000000001556. PMID: 40179403.
2)

Serova NK, Eliseeva NM. Zastoinyi disk zritel’nogo nerva kak priznak vnutricherepnoi gipertenzii [Papilledema as a sign of intracranial hypertension]. Vestn Oftalmol. 2022;138(4):87-93. Russian. doi: 10.17116/oftalma202213804187. PMID: 36004596.
3)

Whiting AS, Johnson LN. Papilledema: clinical clues and differential diagnosis. Am Fam Physician. 1992 Mar;45(3):1125-34. PMID: 1543098.

Fourth ventricle tumor surgery complications

Fourth ventricle tumor surgery complications

Fourth ventricle tumor surgery is delicate and carries specific risks due to the complex anatomy of the region. The fourth ventricle lies in the posterior fossa of the brain, nestled between the brainstem and cerebellum. Because of this, complications can involve vital neurological structures.

Cranial Nerve Deficits

Especially CN VI (abducens), CN VII (facial), and CN VIII (vestibulocochlear).

May cause diplopia, facial weakness, or hearing loss.

Cerebellar Dysfunction

Ataxiadysmetriaintention tremor.

Can be transient or permanent.

Brainstem Injury

Risk of respiratory or cardiovascular instability.

It can lead to coma or death in extreme cases.

Mutism / Cerebellar Cognitive Affective Syndrome

Especially in pediatric patients after resection of medulloblastomas or ependymomas.

Characterized by mutism, emotional lability, and executive dysfunction.

Hydrocephalus (pre- or post-operative)

Due to obstruction of CSF pathways (aqueduct or outlets of the fourth ventricle).

May require an external ventricular drain (EVD) or VP shunt.

CSF Leak / Pseudomeningocele

From poor dural closure or pressure buildup.

Risk of infection or wound healing issues.

Meningitis / Ventriculitis

Especially if there’s a CSF leak or prolonged drain use.

Hemorrhage

Intraoperative bleeding from a tumor or surrounding vessels.

Postoperative hematoma causing brainstem compression.

Ischemia

Injury to perforating arteries (e.g., branches of the posterior inferior cerebellar artery – PICA).

It can cause infarcts in the brainstem or cerebellum.

Infection

Seizures (rare for the posterior fossa)

Respiratory compromise (especially in children or after brainstem manipulation)

Wound complications

Ependymomas: Adherence to the floor of the 4th ventricle increases brainstem injury risk.

Medulloblastomas: prone to CSF spread, so thorough surgical and oncologic planning is key.

Choroid plexus tumors: highly vascular, bleeding risk.

In a large multicenter cohort study Persson et al. investigate postoperative word-finding abilities in children undergoing posterior fossa tumor surgery (PFTs), with data from 184 children across Europe. The authors address a critical yet understudied postoperative complication—word-finding difficulty, which goes beyond classic cerebellar mutism syndrome (CMS) and focuses on more subtle higher-order language impairment1)

The study’s key strength lies in its pre- and postoperative comparisons using a speeded picture-naming test, providing quantitative insights into word retrieval speeds. Interestingly, the results reveal no significant change between pre- and postoperative performance on average. This means that while some children improved, others declined, underscoring the heterogeneous outcomes in PFT surgeries.

A striking finding is that 95% of children performed more than two standard deviations slower than age norms after surgery, despite no aggregate decline. This discrepancy suggests that even without a gross drop in individual scores, the cohort as a whole demonstrates clinically significant delays, potentially overlooked in standard assessments.

The study identifies fourth ventricle tumor as a specific risk factor (B = -4.09, p < 0.05), linking it to possible damage of the dentato-thalamo-cortical pathway. This aligns with previous neuroanatomical models implicating cerebellar-thalamo-cortical circuits in language function, not just motor planning.

From a clinical perspective, these findings call for routine postoperative language screening—even in patients who do not develop mutism—to detect subtle deficits that may affect communication and academic performance. Furthermore, early intervention strategies may be needed, particularly for children with tumours in higher-risk locations.

This study contributes valuable evidence supporting the existence of subtle yet functionally important postoperative language impairments in children with PFTs. It urges the neurooncology and neuropsychology communities to broaden the scope of postoperative assessments to include not only mutism but also word-finding and higher cognitive-linguistic functions.


1)

Persson K, Grønbæk J, Tiberg I, Fyrberg Å, Castor C, Andreozzi B, Frič R, Hauser P, Kiudeliene R, Mallucci C, Mathiasen R, Nyman P, Pizer B, Sehested A, Boeg Thomsen D; CMS study group. Postoperative word-finding difficulties in children with posterior fossa tumours: a crosslinguistic European cohort study. Childs Nerv Syst. 2025 Mar 12;41(1):128. doi: 10.1007/s00381-025-06787-4. PMID: 40075014; PMCID: PMC11903548.

Racial disparities in hydrocephalus treatment

Racial disparities in hydrocephalus treatment

Several studies of administrative data have noted higher mortality rates for Black/African American children with shunted hydrocephalus. A longitudinal study of children with hydrocephalus secondary to myelomeningocele showed lower lifetime rates of shunt revision in minority children compared to White children, indicating a possible disparity in hydrocephalus treatment. The goal of this study is to identify racial and ethnic disparities in mortality or shunt revision rates by using the Hydrocephalus Clinical Research Network (HCRN) hydrocephalus registry sample.

The HCRN registry was queried for patients with shunted hydrocephalus for whom data on all lifetime hydrocephalus procedures were available. Patients with a primary shunt placement before 2023 were included, with follow-up extending through March 19, 2024. A Cox proportional hazards model was created to determine the effect of race and ethnicity on mortality while controlling for age at initial shunt placement, sex, hydrocephalus etiology, gestational age at birth, and the presence of complex chronic conditions. Similarly, a proportional means model was used to evaluate the association with the lifetime number of shunt revision surgeries. The author hypothesized that when controlling for other variables, minority children would have higher mortality and fewer shunt revision surgeries than White children.

A total of 5656 children were included in the analysis of mortality. There were 579 deaths. Race and ethnicity were independently associated with mortality, with Black (HR 1.32, 95% CI 1.05-1.65), other non-White (HR 1.39, 95% CI 1.03-1.86), and Hispanic (HR 1.50, 95% CI 1.22-1.84) children having a higher mortality rate than White children. In the analysis of 4081 children with shunts, Hispanic ethnicity was also independently associated with fewer total shunt revisions (HR 0.84, 95% CI 0.72-0.98).

In children with hydrocephalus, when controlling for other factors, there is a higher mortality rate among Hispanic, Black, and other non-White children, and fewer shunt revisions among Hispanic children. These findings highlight important potential disparities in hydrocephalus treatment 1).


Patient race (i.e., White; Native Hawaiian, or other Pacific Islander) was found to be associated with iNPH development. Meanwhile, after excluding those with cerebrovascular disease, cardiovascular risk factors were not found associated with iNPH. Lastly, iNPH cases were more inclined to have a history of alcohol use disorder and prior psychiatric disorder. Overall, this data reveals that a racial disparity exists amongst iNPH, as well as highlights the role of various cardiovascular and psychiatric risk factors, which can potentially provide direction in etiology elucidation 2).


Among preterm infants with intraventricular hemorrhage and resultant PHH, black infants and those insured by Medicaid have significantly increased mortality but these 2 effects are independent. Further studies are needed to fully understand the factors affecting these racial and socioeconomic disparities 3).


Findings in a study, that utilized US population-level data, suggest the presence of racial and socioeconomic status outcome disparities following pediatric CSF shunting procedures 4).


A retrospective chart review was performed on all pediatric patients who underwent ventriculoperitoneal shunting from 1990-2010 at the Department of Neurological Surgery, University of Rochester Medical Center, 601 Elmwood Ave., Box 670, Rochester, NY, 14642, USA. Race and insurance type were recorded and assessed against specific outcome measures to statistically compare complication rates.

A complete record was found for 373 patients who received 849 shunting procedures at the institution. No differences were found between racial groups and insurance type for overall shunt survival, total revision number, or average time to failure. However, nonwhite patients spent an average of 3 days longer in the hospital at initial shunting (p = 0.04), and those with public insurance stayed for 5 days longer (p = 0.002). Patients with public insurance were more likely to present with shunt failure from outside hospitals (p = 0.005) and be born prematurely (p < 0.001). Private patients were more likely to have a neoplasm present at the time of initial shunt placement (p = 0.003).

While the overall revision rate was not affected by race or insurance status, there were significant delays in discharge for patients with public insurance. Moreover, potential disparities in outpatient access to primary physicians and specialists may be affecting care 5)



The literature examining racial and ethnic disparities in pediatric hydrocephalus reveals consistent evidence that minority populations, particularly Black, Hispanic, and other non-White children, experience worse outcomes compared to their White counterparts. The primary study by Rocque et al. (2025) using the Hydrocephalus Clinical Research Network (HCRN) registry strengthens the case for systemic inequities in healthcare delivery and outcomes.

1. Strengths of the Primary Study (Rocque et al., 2025)

Large, prospective dataset: With 5,656 children included in the mortality analysis and 4,081 in the shunt revision cohort, the study offers robust statistical power. Rigorous methodology: The use of Cox proportional hazards models and control for confounding variables (e.g., age at initial shunt, gestational age, chronic conditions) increases the reliability of the observed associations. Novel findings: The association of higher mortality in minority children and fewer revisions in Hispanic children, despite controlling for clinical variables, points to care process disparities rather than purely biological explanations.

2. Limitations and Interpretative Cautions

Causality remains unclear: The study is observational. While associations are strong, they do not establish causality. The lower revision rate among Hispanic children could either reflect undertreatment, barriers to access, or better surgical outcomes — though the higher mortality suggests the former. Socioeconomic data not directly integrated: While race and ethnicity are analyzed, insurance status, income level, and neighborhood-level SES indicators are not included. This limits insight into the complex interplay between race and class. Data source limitations: The HCRN centers may not be fully representative of all geographic or institutional contexts, potentially introducing bias. Synthesis with Supporting Literature The findings of Rocque et al. are echoed across several studies:

Jin et al. (2016) and Attenello et al. (2015) highlight that racial and economic disparities are independent predictors of increased mortality in hydrocephalus and related pathologies (e.g., PHH). Medicaid coverage — a proxy for low SES — independently correlates with worse outcomes, reinforcing the notion that both race and poverty are crucial risk factors.

Walker et al. (2014) found no differences in shunt survival or revision numbers, but nonwhite and publicly insured children had longer hospital stays and were more likely to present with complications from outside facilities, suggesting disparities in pre- and post-hospital care access, rather than in acute management.

Ghaffari-Rafi et al. (2020), while focused on iNPH, support that race (and potentially psychiatric comorbidities) may play a role in disease development and care patterns. Though not directly comparable to pediatric hydrocephalus, these data emphasize broader racialization of neurological care.

Key Themes and Implications

A. Structural and Institutional Bias These disparities may arise from implicit bias, differential access to care, differences in follow-up protocols, or parental engagement shaped by historical mistrust in healthcare institutions. Fewer shunt revisions in Hispanic children, despite higher mortality, may suggest under-recognition or under-treatment of shunt failures.

B. Socioeconomic Determinants

Insurance status, hospital of origin, and perinatal history (e.g., prematurity) are proxies for healthcare fragmentation and unequal resources. The intersection of race and poverty likely amplifies risks.

C. Need for Systems-Level Interventions

Enhance equity in post-operative follow-up and early complication detection. Implement community-based interventions and education programs to support families from underserved populations. Broaden inclusion of socioeconomic variables in large-scale registries like HCRN to better understand root causes.

The consistent signal across studies — that racial and socioeconomic disparities affect outcomes in pediatric hydrocephalus — underscores an urgent need for targeted policy, educational, and clinical interventions. The findings from the HCRN dataset should galvanize the neurosurgical community to address not only technical outcomes but also systemic inequities in pediatric neurosurgical care.


1)

Rocque BG, Jensen H, Reeder RW, Rozzelle CJ, Kulkarni AV, Pollack IF, McDowell MM, Naftel RP, Jackson EM, Whitehead WE, Pindrik JA, Isaacs AM, Strahle JM, McDonald PJ, Tamber MS, Hankinson TC, Browd SR, Hauptman JS, Krieger MD, Chu J, Riva-Cambrin J, Limbrick DD, Holubkov R, Kestle JRW, Wellons JC. Racial disparities in hydrocephalus mortality and shunt revision: a study from the Hydrocephalus Clinical Research Network. J Neurosurg Pediatr. 2025 Mar 21:1-9. doi: 10.3171/2024.12.PEDS24371. Epub ahead of print. PMID: 40117669.
2)

Ghaffari-Rafi A, Gorenflo R, Hu H, Viereck J, Liow K. Role of psychiatric, cardiovascular, socioeconomic, and demographic risk factors on idiopathic normal pressure hydrocephalus: A retrospective case-control study. Clin Neurol Neurosurg. 2020 Jun;193:105836. doi: 10.1016/j.clineuro.2020.105836. Epub 2020 Apr 28. PMID: 32371292.
3)

Jin DL, Christian EA, Attenello F, Melamed E, Cen S, Krieger MD, McComb JG, Mack WJ. Cross-Sectional Analysis on Racial and Economic Disparities Affecting Mortality in Preterm Infants with Posthemorrhagic Hydrocephalus. World Neurosurg. 2016 Apr;88:399-410. doi: 10.1016/j.wneu.2015.12.046. Epub 2015 Dec 28. PMID: 26732967.
4)

Attenello FJ, Ng A, Wen T, Cen SY, Sanossian N, Amar AP, Zada G, Krieger MD, McComb JG, Mack WJ. Racial and socioeconomic disparities in outcomes following pediatric cerebrospinal fluid shunt procedures. J Neurosurg Pediatr. 2015 Jun;15(6):560-6. doi: 10.3171/2014.11.PEDS14451. Epub 2015 Mar 20. PMID: 25791773.
5)

Walker CT, Stone JJ, Jain M, Jacobson M, Phillips V, Silberstein HJ. The effects of socioeconomic status and race on pediatric neurosurgical shunting. Childs Nerv Syst. 2014 Jan;30(1):117-22. doi: 10.1007/s00381-013-2206-5. Epub 2013 Jun 30. PMID: 23811830.