Neonatal posthemorrhagic hydrocephalus from prematurity: pathophysiology and current treatment concepts

A review

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Object

Preterm infants are at risk for perinatal complications, including germinal matrix–intraventricular hemorrhage (IVH) and subsequent posthemorrhagic hydrocephalus (PHH). This review summarizes the current understanding of the epidemiology, pathophysiology, management, and outcomes of IVH and PHH in preterm infants.

Methods

The MEDLINE database was systematically searched using terms related to IVH, PHH, and relevant neurosurgical procedures to identify publications in the English medical literature. To complement information from the systematic search, pertinent articles were selected from the references of articles identified in the initial search.

Results

This review summarizes the current knowledge regarding the epidemiology and pathophysiology of IVH and PHH, primarily using evidence-based studies. Advances in obstetrics and neonatology over the past few decades have contributed to a marked improvement in the survival of preterm infants, and neurological morbidity is also starting to decrease. The incidence of IVH is declining, and the incidence of PHH will likely follow. Currently, approximately 15% of preterm infants who suffer severe IVH will require permanent CSF diversion. The clinical presentation and surgical management of symptomatic PHH with temporary ventricular reservoirs (ventricular access devices) and ventriculosubgaleal shunts and permanent ventriculoperitoneal shunts are discussed. Preterm infants who develop PHH that requires surgical treatment remain at high risk for other related neurological problems, including cerebral palsy, epilepsy, and cognitive and behavioral delay. This review highlights numerous opportunities for further study to improve the care of these children.

Conclusions

A better grasp of the pathophysiology of IVH is beginning to impact the incidence of IVH and PHH. Neonatologists conduct rigorous Class I and II studies to advance the outcomes of preterm infants. The need for well-designed multicenter trials is essential because of the declining incidence of IVH and PHH, variations in referral patterns, and neonatal ICU and neurosurgical management. Well-designed multicenter trials will eventually produce evidence to enable neurosurgeons to provide their smallest, most vulnerable patients with the best practices to minimize perioperative complications and permanent shunt dependence, and most importantly, optimize long-term neurodevelopmental outcomes.

Abbreviations used in this paper:

AHW = anterior horn width; cPVL = cystic periventricular leukomalacia; EGA = estimated gestational age; EPO = erythropoietin; ETV = endoscopic third ventriculostomy; ICP = intracranial pressure; IL = interleukin; IVH = germinal matrix–intraventricular hemorrhage; LP = lumbar puncture; MDI = Bayley Mental Developmental Index; NICU = neonatal ICU; paCO2 = partial pressure of CO2; PDI = Bayley Psychomotor Developmental Index; PHH = posthemorrhagic hydrocephalus; PHVD = posthemorrhagic ventricular dilation; PVHI = periventricular hemorrhagic infarction; PVL = periventricular leukomalacia; rEPO = recombinant EPO; rtPA = recombinant tissue plasminogen activator; TGF = transforming growth factor; VP = ventriculoperitoneal; VSG = ventriculosubgaleal.

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