Artikel
Pseudotumor cerebri syndrome in a child with Alagille syndrome – intracranial pressure dynamics and treatment outcome after ventriculoperitoneal shunting
Pseudotumor cerebri Syndrom bei einem Kind mit Alagille-Syndrom
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Autoren
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Manolis Polemikos
- Medizinische Hochschule Hannover, Klinik für Neurochirurgie, Hannover, Deutschland
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Hans E. Heissler
- Medizinische Hochschule Hannover, Klinik für Neurochirurgie, Hannover, Deutschland
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Hans Hartmann
- Medizinische Hochschule Hannover, Klinik für pädiatrische Nieren-, Leber- und Stoffwechselerkrankungen, Hannover, Deutschland
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Elvis J. Hermann
- Medizinische Hochschule Hannover, Klinik für Neurochirurgie, Hannover, Deutschland
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Joachim Kurt Krauss
- Medizinische Hochschule Hannover, Klinik für Neurochirurgie, Hannover, Deutschland
| Veröffentlicht: | 4. Juni 2021 |
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Gliederung
Text
Objective: Alagille syndrome (AS) is a rare multisystem disease of the liver, heart, eyes, face, skeleton, kidneys, and vascular system. Occurrence of pseudotumor cerebri syndrome (PTCS) in AS is exceedingly rare and, thus far, continuous intracranial pressure (ICP) monitoring has never been utilized before in this subgroup. Owning to their rarity and mostly atypical presentation the diagnosis and natural history of such cases remains uncertain.
Methods: Continuous epidural ICP monitoring. Follow-up after shunt surgery.
Results: We report on an atypical case of PCTS in a patient with a known history of AS who presented at 4 years of age with bilateral papilledema on a routine ophthalmological examination. Visual findings deteriorated after initial treatment with acetazolamide. Continuous ICP monitoring was then utilized to confirm the elevated ICP. ICP monitoring demonstrated massive dynamic ICP changes over time with markedly increased ICP values most of the time. The decomposition of pressure traces revealed pressure waves with amplitudes reaching values up to 60 mmHg for several minutes. B-waves dominated ICP dynamics superposing the slower ICP fluctuations in time. Additionally, a relatively high number of A-wave pressure transients were scattered in the traces. The majority of ICP values were measured to be within the range of 15 to 70 mmHg (83%). Successful treatment with resolution of papilledema was achieved after ventriculoperitoneal shunting but relapsed due to growth related dislocation of the ventricular catheter.
Conclusion: This report brings new insights into the ICP dynamics and the resulting treatment in this possibly underdiagnosed subgroup of PTCS patients. It also highlights the importance of ICP monitoring for diagnosing PCTS in atypical patients and demonstrates that ventriculoperitoneal shunting can provide long-term improvement of symptoms for more than 10 years. Our findings among with previously reported cases suggest that a genetic predisposition makes AS patients susceptible for developing PCTS, whereas associated medical conditions and exogenous factors can trigger or exacerbate intracranial hypertension.
