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Published on: June 28, 2024
Children with dorsal midbrain syndrome as a result of pineal tumors
Mary Ellen Hoehn1, Julie Calderwood2, Thomas O'Donnell1
1Hamilton Eye Institute, Department of Ophthalmology, University of Tennessee Health Science Center, Memphis, Tennessee; Department of Surgery, St. Jude Children's Research Hospital, Memphis, Tennessee.
Insights
Pediatric pineal tumors frequently cause dorsal midbrain syndrome, impacting vision. Most children experienced persistent symptoms after treatment, highlighting the need for further research into effective interventions.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Neurology
Background:
- Dorsal midbrain syndrome (DMS), also known as Parinaud syndrome, is a recognized complication of pineal region tumors.
- Limited data exists on DMS outcomes, particularly in pediatric populations.
- This study focuses on ophthalmic outcomes in children with pineal tumors.
Purpose of the Study:
- To report the ophthalmic outcomes in children diagnosed with pineal tumors.
- To assess the incidence and characteristics of DMS in pediatric patients with pineal tumors.
- To evaluate treatment responses and residual findings in affected children.
Main Methods:
- Retrospective chart review of pediatric patients with pineal region tumors.
- Ophthalmologic evaluations were conducted to identify DMS criteria: limited upgaze, pupillary light-near dissociation, and convergence retraction nystagmus.
- Descriptive statistics were used to analyze DMS rates and treatment outcomes.
Main Results:
- DMS was present in 51% of 35 pediatric patients with pineal tumors.
- The most common DMS findings were limited upgaze (89%), pupillary light-near dissociation (83%), and convergence retraction nystagmus (50%).
- Only 41% of treated patients showed improvement, with complete resolution in just 12%.
Conclusions:
- Pineal tumors in children have a high incidence of dorsal midbrain syndrome.
- Most pediatric patients with pineal tumors and DMS exhibit residual ophthalmic findings post-treatment.
- Further investigation is warranted to improve outcomes for these patients.
Background:
Dorsal midbrain syndrome (also known as Parinaud syndrome and pretectal syndrome) is a well-known complication of tumors of the pineal region. However, there are few reports regarding outcomes, especially in children. The purpose of this study was to report the ophthalmic outcomes in a group of children with pineal tumors treated at a single institution.
Methods:
The medical records of pediatric patients diagnosed with pineal region tumors and evaluated at our ophthalmology clinic were studied retrospectively. Descriptive statistics were used to assess rate of dorsal midbrain syndrome, defined as one or more of the following: limitation of upgaze, pupillary light-near dissociation, and convergence retraction nystagmus. Treatment outcomes were recorded.
Results:
A total of 35 subjects (age range, 5 months to 20 years) were included, 18 (51%) of whom were found to have dorsal midbrain syndrome. Of those 18, 16 patients (89%) had limitation of upgaze, 15 (83%) had pupillary light-near dissociation, and 9 (50%) had convergence-retraction nystagmus. Convergence insufficiency was noted in 5 patients (28%); exotropia (either intermittent or constant), in 9 (50%). Improvement in dorsal midbrain syndrome findings following treatment was seen in 7 of 17 patients (41%), but only 2 (12%) experienced complete resolution. Treatment consisted of surgery, radiation, and/or chemotherapy.
Conclusions:
In our study cohort of children with pineal tumors have a high incidence of dorsal midbrain syndrome. Most cases had residual findings after treatment.
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