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A diagnostic dilemma in pediatric proptosis-from suspected malignancy to benign orbital cavernous venous
Mohd-Asyraaf Abdul-Kadir1,2, Muhammad Adri Mohamed Shafit3, Adzura Salam4
1Department of Ophthalmology, Kulliyyah of Medicine, International Islamic University Malaysia, Kuantan, Pahang, Malaysia. akmasyraaf@unimas.my.
Insights
Rapidly progressive proptosis in children, often mimicking malignancy, can be caused by rare cavernous venous malformations. Acute enlargement due to hemorrhage highlights the need for prompt diagnosis and intervention to preserve vision.
Area of Science:
- Ophthalmology
- Pediatric Oncology
- Vascular Malformations
Background:
- Rapidly progressive proptosis in children presents diagnostic challenges, often mimicking orbital malignancy.
- Differentiating causes of pediatric proptosis is critical for appropriate management.
Purpose of the Study:
- To report a rare case of pediatric cavernous venous malformation presenting with acute enlargement.
- To emphasize the importance of accurate diagnosis and timely intervention in pediatric orbital masses.
Main Methods:
- A case of a 3-year-old boy with rapidly progressive proptosis is presented.
- Diagnostic imaging, including MRI, revealed intraconal and extraconal orbital masses with vascular channels.
- Surgical debulking and histopathological analysis confirmed cavernous venous malformation with thrombosed vessels.
Main Results:
- The pediatric patient presented with acute worsening of proptosis, fever, and lethargy.
- Imaging demonstrated lesion enlargement with intralesional hemorrhage and displacement of orbital structures.
- Histopathology confirmed cavernous venous malformation, a rare pediatric orbital diagnosis.
Conclusions:
- Acute enlargement of cavernous venous malformations can mimic malignant or infectious orbital diseases in children.
- Accurate clinical assessment, targeted imaging, and histopathological confirmation are essential for diagnosing pediatric orbital masses.
- Early multidisciplinary intervention is crucial for preserving vision in cases of rapidly progressing pediatric proptosis.
Background:
Rapidly progressive proptosis in children can be caused by multiple causes. Distinguishing between these is challenging, especially when presentations mimic malignancy.
Case Presentation:
A healthy Malay 3-year-old boy presented with a 1-week history of painless, progressive left eye proptosis. Initial imaging showed intraconal and extraconal orbital masses with prominent vascular channels, suggesting a vascular lesion. Two weeks later, he developed fever, lethargy, and worsening proptosis. Repeat imaging revealed lesion enlargement with intralesional hemorrhage, displacement of orbital structures, and a dilated superior ophthalmic vein. Differential diagnoses included rhabdomyosarcoma, orbital cellulitis, and venolymphatic malformation. Anterior orbitotomy and debulking were performed, and histopathology confirmed cavernous venous malformations with thrombosed vessels. At 6 months, there was no recurrence, but dense corneal scarring left the visual prognosis uncertain.
Conclusions:
Cavernous venous malformation is rare in children and usually progresses slowly. Acute enlargement from intralesional hemorrhage can mimic malignant or infectious disease. In pediatric orbital masses, careful clinical assessment, targeted imaging, and histopathological confirmation remain essential. Early multidisciplinary intervention is key to preserving vision when rapid progression threatens the eye.
