Posthemorrhagic hydrocephalus of prematurity outcome

Shunt failure is high when inserted during or immediately following the neonatal period. Overdrainage may be less common in patients with flow-regulated valves. However, if overdrainage is observed, adjusting the setting of a differential pressure valve can effectively treat the overdrainage without the need for invasive shunt revision surgery ¹⁾

Among preterm neonates with Posthemorrhagic hydrocephalus following severe IVH, elevated CSF hemoglobin, ferritin, and iron were associated with more severe early ventricular enlargement (FOHR > 0.6 vs ≤ 0.6 at first ventricular tap) ²⁾.

A prospective multi-center cohort included patients with posthemorrhagic hydrocephalus (PHH) who underwent temporary and permanent Cerebrospinal fluid shunt and Bayley Scales of Infant and Toddler Development Third Edition testing around 2 years of age. CSF proteins in the iron handling pathway were analyzed longitudinally and compared to ventricle size and neurodevelopmental outcomes.

Results: Thirty-seven patients met inclusion criteria with a median estimated gestational age at birth of 25 weeks; 65% were male. CSF hemoglobin, iron, total bilirubin, and ferritin decreased between temporary and permanent CSF diversion with no change in CSF ceruloplasmin, transferrin, haptoglobin and hepcidin; and an increase in CSF hemopexin. Larger ventricle size at permanent CSF diversion was associated with elevated CSF ferritin (p = 0.015) and decreased CSF hemopexin (p = 0.007). CSF levels of proteins at temporary CSF diversion were not associated with outcome, however higher CSF transferrin at permanent CSF diversion was associated with improved cognitive outcome (p = 0.015). Importantly, longitudinal change in CSF iron pathway proteins, ferritin (decrease) and transferrin (increase) were associated with improved cognitive (p = 0.04) and motor (p = 0.03) scores and improved cognitive (p = 0.04), language (p = 0.035) and motor (p = 0.008) scores, respectively.

Longitudinal changes in CSF transferrin (increase) and ferritin (decrease) are associated with improved neurodevelopmental outcomes in neonatal PHH, with implications for understanding the pathogenesis of poor outcomes in PHH ³⁾.

Infants with posthemorrhagic hydrocephalus are at risk for adverse neurodevelopmental outcomes.. · The parent experience of caring for a child with posthemorrhagic hydrocephalus is not well-described. In this interview study, parents described uncertainty, isolation, and hypervigilance.

· These findings call for structured NICU counseling and longitudinal family supports after discharge ⁴⁾.

These patients typically eventually require permanent CSF diversion and are presumed to be indefinitely shunt-dependent.

Loss of sleep-wake cycling, shown by aEEG, has a high positive predictive value for the development of posthaemorrhagic hydrocephalus (PPH) in preterm infants with IVH; therefore, the study of cerebral background activity and in particular of sleep-wake cycling can be used as an early prognostic tool in patients at risk of PPH ⁵⁾.

Since it is evident that posthemorrhagic hydrocephalus of prematurity is associated with long-term effects, such as neurodevelopmental impairments and neuropsychological deficits, the modern perinatal care of preterm infants remains still challenging ^{6) 7)}.

A subset of children with periventricular-intraventricular hemorrhage develop posthemorrhagic hydrocephalus (PHH) with intracranial hypertension, which may lead to reduced consciousness, reduced food intake, and developmental impairments.

Therefore, the focus of care in these children is to avoid posthemorrhagic ventricular dilatation, raised ICP, and hydrocephalus. Surgical treatment for PHH is mostly performed in two steps. For the initial treatment, temporary procedures for cerebrospinal fluid (CSF) drainage can be performed. These include insertion of ventricular access devices, external ventricular drains, and ventriculosubgaleal shunt ^{8) 9) 10) 11) 12) 13) 14)}.

For the definitive treatment of PHH, ventriculoperitoneal shunt implantation is preferred and worldwide accepted method ^{15) 16)}.

Generally, shunt insertion in very premature infants after ventricular hemorrhage is associated with elevated rates of shunt failure and shunt infection compared with older children or adults ^{17) 18)}.

An ICP-correlated aspiration of CSF and ventricular decompression as treatment option of PHH in children was described by ^{19) 20)} and the efficacy evaluated ²¹⁾.

20–50% of infants with periventricular-intraventricular hemorrhage (PIVH) will develop either transient or progressive hydrocephalus (HCP). Grades III and IV are more often associated with progressive ventricular dilatation than are lower grades (however, HCP may develop even after low grade PIVH ²²⁾). Younger gestational age infants may be at lower risk.

Post PIVH hydrocephalus usually occurs 1–3 weeks after the hemorrhage. Probably caused by cellular debris and/or the toxic e ects of blood breakdown products on the arachnoid granulations (communicating HCP), or by an adhesive arachnoiditis in the posterior fossa or rarely by compression or blockage of critical pathways, e.g. at the sylvian aqueduct (obstruc- tive HCP). In a case of HCP following intra-uterine PIVH, aqueductal gliosis was found at autopsy ²³⁾.

An infant with hydrocephalus which had been diagnosed at birth died at one month of age. Subsequent neuropathological studies demonstrated old germinal matrix haemorrhage and complete occlusion of the aqueduct of Sylvius by fibroglial tissue, both of which appeared to be older than four weeks, indicating that they had occurred in utero. These observations are consistent with the occurrence of intra-uterine intraventricular haemorrhage secondary to haemorrhage of the subependymal germinal matrix, leading to occlusion of the aqueduct of Sylvius and hydrocephalus prior to birth ²⁴⁾.