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Purpose <p>Cerebrospinal Fluid (CSF) shunt placement during infancy remains the standard treatment for pediatric hydrocephalus. However, ultra-late shunt malfunction after an extended interval of regular follow-up is poorly characterized. This study describes the clinical features, presumed pathophysiology, and management of CSF shunt dysfunction occurring decades after extended intervals without regular follow-up.</p> Methods <p>We retrospectively evaluated patients treated for shunt malfunction at our institution between 2007 and 2025. Inclusion criteria included infant CSF shunt placement during infancy, an extended interval of regular neurosurgical follow-up, prior to malfunction, and post-adolescent shunt dysfunction. Patient demographics, underlying diseases, shunt history, clinical presentation, presumed causes, treatment, and outcomes were extracted. Cognitive development was classified as none, mild, moderate, or severe.</p> Results <p>Seven patients (median age 24&#xa0;years; range 21–52&#xa0;years; four males, three females) met the inclusion criteria. All experienced distal catheter-related complications including obstruction (<i>n</i> = 5) and fracture (<i>n</i> = 2). Among obstructions, four indicated infection-related pathology; one patient developed a large intra-abdominal pseudocyst. Five patients presented with intracranial hypertension; two had fever or localized abdominal/subcutaneous swelling without ventriculomegaly. All underwent initial external drainage followed by shunt revision or conversion. Recurrent distal obstruction occurred in three females with peritoneal inflammation. Four patients had mild-to-severe cognitive impairment; three were independent.</p> Conclusion <p>Ultra-late CSF shunt dysfunction may occur decades after extended intervals of regular follow-up, primarily due to distal catheter obstruction and infection-related pathology. Symptoms may be atypical, and recurrence is common in cases with peritoneal inflammation. These findings emphasize the importance of lifelong surveillance and heightened clinical vigilance for patients with a history of pediatric CSF shunt placement.</p>

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Ultra-late cerebrospinal fluid shunt malfunction following an extended interval of regular follow-up: a case series

  • 期刊论文

  • 发表日期：

    2026年4月8日

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  • Hime Suzuki,
  • Asuka Sasao,
  • Kyoya Sakashita,
  • Yoshifumi Horita,
  • Koji Okuda,
  • Takeshi Mikami

摘要

Purpose

Cerebrospinal Fluid (CSF) shunt placement during infancy remains the standard treatment for pediatric hydrocephalus. However, ultra-late shunt malfunction after an extended interval of regular follow-up is poorly characterized. This study describes the clinical features, presumed pathophysiology, and management of CSF shunt dysfunction occurring decades after extended intervals without regular follow-up.

Methods

We retrospectively evaluated patients treated for shunt malfunction at our institution between 2007 and 2025. Inclusion criteria included infant CSF shunt placement during infancy, an extended interval of regular neurosurgical follow-up, prior to malfunction, and post-adolescent shunt dysfunction. Patient demographics, underlying diseases, shunt history, clinical presentation, presumed causes, treatment, and outcomes were extracted. Cognitive development was classified as none, mild, moderate, or severe.

Results

Seven patients (median age 24 years; range 21–52 years; four males, three females) met the inclusion criteria. All experienced distal catheter-related complications including obstruction (n = 5) and fracture (n = 2). Among obstructions, four indicated infection-related pathology; one patient developed a large intra-abdominal pseudocyst. Five patients presented with intracranial hypertension; two had fever or localized abdominal/subcutaneous swelling without ventriculomegaly. All underwent initial external drainage followed by shunt revision or conversion. Recurrent distal obstruction occurred in three females with peritoneal inflammation. Four patients had mild-to-severe cognitive impairment; three were independent.

Conclusion

Ultra-late CSF shunt dysfunction may occur decades after extended intervals of regular follow-up, primarily due to distal catheter obstruction and infection-related pathology. Symptoms may be atypical, and recurrence is common in cases with peritoneal inflammation. These findings emphasize the importance of lifelong surveillance and heightened clinical vigilance for patients with a history of pediatric CSF shunt placement.