Permanent Visual Impairment due to Delayed Diagnosis of Shunt Malfunction in Case of Lack of Typical Features of Increased Intracranial Pressure and Unchanged Ventricular Size.

Objective: We present the clinical features, imaging, and management of 5 cases of visual impairment as the primary manifestation ventriculoperitoneal (V-P) shunt malfunction.

Methods: We retrospectively reviewed the medical records of 126 patients of V-P shunt malfunction in Shanghai Children's Medical Center between 2015 and 2020. Medical records including all hospital admissions were reviewed and follow-up data were collected.

Results: Five children (3.97%) had visual impairment as the primary manifestation of V-P shunt malfunction, with a mild or no headache. Four broken distal shunt catheters and one proximal catheter blockage were confirmed intraoperatively and cured by surgery. None of the patients had a definite improvement in ophthalmic examinations after 4-52 months of follow-up.

Conclusion: Visual impairment as the primary manifestation of V-P shunt malfunction was uncommon and could be easily missed or misdiagnosed as the only problem for lack of typical features of increased intracranial pressure and unchanged ventricular size. Earlier definitive diagnosis and surgical intervention could prevent a further development of the visual loss caused by V-P shunt malfunction.