Conservative and operative management of iatrogenic craniocerebral disproportion—a case-based review

• Published in: Child's nervous system Vol. 35; no. 1; pp. 19 - 27
• Main Authors: Beez, Thomas, Munoz-Bendix, Christopher, Ahmadi, Sebastian Alexander, Messing-Jünger, Martina, Steiger, Hans-Jakob, Röhrig, Andreas
• Format: Journal Article
• Language: English
• Published: Berlin/Heidelberg Springer Berlin Heidelberg 01.01.2019
• Subjects: Case-Based Update; Child; Child, Preschool; Clinical Decision-Making; Conservative Treatment; Female; Humans; Hydrocephalus - therapy; Iatrogenic Disease; Infant, Newborn; Infant, Premature; Intracranial Hemorrhages - complications; Intracranial Hypertension - etiology; Male; Medicine; Medicine & Public Health; Neurosciences; Neurosurgery; Neurosurgical Procedures - methods; Prognosis; Skull - abnormalities; Skull - surgery; Treatment Outcome; Ventriculoperitoneal Shunt - adverse effects; Slit ventricles; Shunt; Intracranial hypertension; Chronic overdrainage
• ISSN: 0256-7040; 1433-0350
• DOI: 10.1007/s00381-018-3981-9

Introduction Craniocerebral disproportion (CCD) can occur as a sequela after shunting in early infancy. It can be understood as a disorder closely related to slit ventricle syndrome and chronic overdrainage syndrome. Here, we present two exemplary cases and summarize the pathophysiological, diagnostic, and therapeutic approaches to CCD. Clinical presentation Two premature babies underwent shunting for posthemorrhagic hydrocephalus and presented in later childhood with recurrent episodes of symptomatic raised intracranial pressure (ICP) at 2 and 8 years of age, respectively. Diagnosis and management Both patients had unchanged ventricular size on cranial imaging and fulfilled the clinical diagnostic criteria of CCD. After confirming shunt patency, ICP monitoring was performed to diagnose intermittent intracranial hypertension. Different treatment pathways were pursued: While readjustment of a programmable shunt valve was sufficient to alleviate the raised ICP in the first case, a cranial expansion surgery was necessary in the second case. Outcome and conclusions Both children were treated successfully after thorough assessment and careful choice of treatment approaches. This review provides detailed insight into CCD and highlights the importance of individual and critical decision-making in these complex patients.