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Table of Contents    
Year : 2021  |  Volume : 69  |  Issue : 8  |  Page : 313-319

Management of Posthemorrhagic Hydrocephalus

Department of Neurosurgery, Global Hospital, Parel, Mumbai, Maharashtra, India

Date of Submission17-Jun-2021
Date of Decision15-Oct-2021
Date of Acceptance18-Oct-2021
Date of Web Publication11-Dec-2021

Correspondence Address:
Dr. Suresh K Sankhla
Room No. 112, 1st Floor OPD, Global Hospital, 35, Dr. E. Borges Road, Parel, Mumbai – 400 012, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0028-3886.332257

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 » Abstract 

Background: Although there are several successful treatment options available today, the optimal management of posthemorrhagic hydrocephalus (PHH) still remains undetermined.
Objective: To evaluate the efficacy and outcomes of contemporary treatment methods and to define current evidence-based management for PHH in premature infants.
Material and Methods: Literature was reviewed to identify and analyze merits and demerits of the currently available temporizing measures and definitive treatment for premature low-birth weight babies with PHH.
Results and Conclusions: Advances in treatment and increased experience have led to redefinition of treatment goals to optimize cognitive neurodevelopment, and quality of life in these premature infants with PHH. Current literature favors early diagnosis and intervention using temporizing measures, and prevention of future complications of PHH with a permanent CSF diversion method such as ventricular shunting or endoscopic third ventriculostomy.

Keywords: Germinal matrix hemorrhage, intraventricular hemorrhage, posthemorrhagic ventricular dilatation, premature, temporizing measures
Key Message: Based on recent literature, the management of PHH is focused mainly on early surgical intervention with the aim to clear intraventricular blood and its breakdown products to reduce cognitive disability, the incidence of multiloculated hydrocephalus, and the need for permanent shunt placement.

How to cite this article:
Nayak N, Sankhla SK. Management of Posthemorrhagic Hydrocephalus. Neurol India 2021;69, Suppl S2:313-9

How to cite this URL:
Nayak N, Sankhla SK. Management of Posthemorrhagic Hydrocephalus. Neurol India [serial online] 2021 [cited 2023 May 31];69, Suppl S2:313-9. Available from:

Germinal matrix hemorrhage, intraventricular hemorrhage (IVH), and posthemorrhagic ventricular dilation (PHVD) are complications of prematurity that may result in cognitive delay, behavioral abnormalities, epilepsy, visual impairment, cerebral palsy, and symptomatic hydrocephalus.[1],[2],[3] Approximately 25%–30% of premature or low birth weight (LBW) infants suffer from IVH and a higher incidence is associated with lower gestational ages and birth weights.[4] Infants weighing less than 1500 g at birth are at a higher risk of developing IVH, and >40% of infants with birth weight 500–750 g are likely to suffer IVH.[5],[6] It has been estimated that approximately 25%–50% of neonates with IVH continue to develop PHVD, and nearly 40% of them require some form of treatment for hydrocephalus.[7],[8],[9],[10],[11]

The management for IVH and its sequelae in premature infants has evolved over past several decades. Although several treatment paradigms have been proposed, there has been no clear consensus on the optimal treatment for PHH and the guidelines or evidence-based recommendations are lacking till date.[12],[13],[14] Presently available therapeutic measures include diuretics, serial lumbar punctures, ventricular access devices (VAD), external ventricular drainage (EVD), and ventriculosubgaleal shunts (VSGS), as well as the permanent cerebrospinal fluid (CSF) diversion methods such as ventriculoperitoneal (VP) shunts and endoscopic third ventriculostomy (ETV). In this report, we review the literature to evaluate the efficacy and outcomes of various treatment modalities available today and make an attempt to outline the current evidence-based management of prematurity-related PHVD/PHH.

 » Diagnosis Top

Most cases of IVH in premature infants are diagnosed on cranial ultrasound (USG) which is performed soon after the birth, either as a routine procedure or following clinical/systemic instability. There are established criteria to estimate severity and protocols for serial USG in preterm LBW babies with IVH/PHVD[15],[16] [Table 1]. The highest risk for bleeding is within the first 48 h after birth and most IVH is diagnosed by day 7.[17],[18] Therefore, a close surveillance in the first week after birth with cranial USG of infants with lower gestational age and LBW is extremely crucial for early diagnosis and successful treatment.
Table 1: Grading of IVH

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Clinical presentation

  1. Clinically Silent: On most occasions, IVH may be clinically silent and is detected only on ultrasound studies. For this reason, many authors recommend routine head USG in any infant born at less than 34 weeks' gestation or has birth weight <1500 g.
  2. Catastrophic: A rapid neurological deterioration may occur over a period of minutes to hours mimicking large intracranial hemorrhage. The clinical signs suggestive of IVH may include a sudden alteration in vital signs like bradycardia or apnea, and/or neonatal seizures. A bulging fontanelle, separation of the sutures, or a rapidly increasing head circumference may suggest the development of acute hydrocephalus.
  3. Saltatory: Symptoms may evolve over a period of hours and days with a fluctuating pattern of decreased alertness, sluggish motor activity, hypotonia, abnormal eye movements, and respiratory difficulties. Clinical evaluation for increasing head circumference, fullness of fontanelle, enlarged scalp veins, and splaying of sutures can be used to assess progressive ventricular dilatation. Other more serious clinical signs of raised Intracranial Pressure (ICP) such as apnea, bradycardia, lethargy, and decreased activity warn urgent neurosurgical intervention.

Management of IVH and PHH

Management of IVH and posthemorrhagic ventriculomegaly in premature infants still remains a challenge to clinicians.[12],[13],[14] The approach to treatment is guided by multiple ongoing factors mainly responsible for primary brain injury and subsequent neurodevelopmental outcomes. Brain damage initially caused by the germinal matrix (GM) hemorrhage is compounded by the toxic effects of the blood and its degradation products, especially iron and free radicals. Additional injury is inflicted by an inflammatory response generated secondary to the release of proinflammatory cytokines. The development of hydrocephalus and raised ICP further worsens the insult to the developing neonatal brain.[17],[18] The overall management of IVH/PHH should thus include aggressive removal of intraventricular blood and its breakdown products, and appropriate treatment for raised ICP due to PHVD/PHH [Table 2]. Currently, there is no consistently proven role of medical management in patients with PHH. A large multicenter randomized controlled trial on acetazolamide and furosemide failed to show any significant benefit in survival or reduction in the need for shunt surgery when used for posthemorrhagic ventricular dilatation.[19] It is also clear from the literature that an early definitive treatment like VPS is mostly unsuitable in LBW premature infants with PHVD/PHH due to extreme fragility and immunological immaturity, and is frequently associated with high risks of anesthetic and surgical morbidity and increased rates of shunt failure.[20],[21] Current management paradigm favors the use of temporizing measures initially during the neonatal period to tide over the crisis in the acute phase until placement of a permanent ventricular shunt is considered necessary and safe.[21],[22] This regimen has by far been the most logical therapeutic option in the management of IVH/PHVD and provides an opportunity to enhance the neurodevelopmental outcome in infants by reducing the complications during treatment, to prevent further progression of hydrocephalus, and to reduce the rates of shunt dependency and shunt revisions.
Table 2: Treatment options in premature infants with IVH/PHH

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Temporizing measures

Commonly used temporizing measures for PHVD/PHH include lumbar punctures (LP), transfontanelle ventricular puncture (VP), EVD, VAD, and VSGS [Table 2],[Table 3],[Table 4]. The choice of temporizing method, however, remains a matter of debate. Meta-analysis of trials from 1980s and 1990s has demonstrated that lumbar punctures and ventricular taps do not reduce permanent shunt dependence or neurological disability.[23] Lumbar puncture is often difficult to perform in clinically unstable infants and its effectiveness tends to reduce on repeated attempts. Ventricular tapping is associated with infection, seizures, and parenchymal injury in the form of hemorrhages and porencephalic cyst formation. EVD is generally not considered as the first choice of treatment for PHH because of the associated higher risk of infection, frequent catheter obstruction, CSF over-drainage, subdural hygroma, hyponatremia, and issues related to the integrity of scalp including wound dehiscence, skin erosion, and CSF leakage.[24],[25] Infants with EVD also require management in the intensive care until the drain is removed or converted to a permanent shunt. VAD partly avoids these limitations and is a good alternative to an EVD.[26] However, VADs are associated with higher risk of infection due to repeated percutaneous tappings and cause fluctuations in intraventricular pressure between very high and very low. The VSG shunts are believed to provide an alternative method for continuous drainage of CSF in the treatment of PHH-related raised ICP. VSGSs have very low infection rate (6%–8%) and other complications like CSF leakage, meningitis, electrolyte imbalance, shunt malfunction, migration of the catheter, and intraparenchymal hemorrhage are rare.[25],[26],[27],[28],[29],[30] Permanent shunt placement is usually required in 60%–85% of cases.[25] The main advantage of VSGS, however, is the shorter hospital stay and the ease with which the patient can be managed by the nursing and medical staff in the outpatient setting [61],[62],[63],[64].
Table 3: Studies showing results in patients with PHH treated by VSGS and NEL

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Table 4: Comparative results of temporizing measures used in premature infants with IVH/PHH

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[Table 2],[Table 3],[Table 4] summarize comparative results of temporizing measures in IVH/PHVD. In a systematic review and meta-analysis of outcome and complications, Badhiwala and colleagues,[13] demonstrated no significant difference in outcome among VAD, EVD, and VSGS. When factors like risks of infection, rates of permanent VPS placement, and subsequent shunt revisions are compared, both VADs and VSGSs are found to be almost equal in effectiveness.[31],[32] Frassanito et al.[30] in a recent publication described a treatment algorithm of combined neuroendoscopic ventricular lavage (NEL) and VSGS in 63 infants with PHH and suggested that the combination is effective and the complication risks including infection and multiloculated hydrocephalus can be reduced significantly.

Timing of intervention is very crucial and several landmark studies in recent years have highlighted the importance of early surgical treatment in premature infants with PHVD.[33],[34],[35],[36] A retrospective comparative study between Dutch and Canadian cohorts found lower rates of cognitive (P = 0.002) and motor (P = 0.03) disability in a cohort whose treatment was initiated when the VI exceeded the 97th centile compared to a cohort whose treatment was initiated in the presence of clinical signs of raised ICP.[34] The early versus late ventricular intervention study (ELVIS) has demonstrated that early intervention (VI exceeding 97th centile or Anterior Horn Width (AHW) >6 mm) is associated with lesser disability than late intervention (VI exceeding 97th centile + 4 mm or AHW 10 mm), and the composite rate of death or disability at 24 months' corrected age was 51% in the late intervention group and 35% in the early intervention group.[35],[36] Regardless of the type of temporizing method used, a close monitoring with regular head circumference measurement, clinical evaluation, and USG assessment is extremely essential to ensure ventricular dilatation is under control.

Intraventricular blood and breakdown products

The toxic effects of blood and its degradation products on the developing brain are well documented.[37],[38],[39] It is therefore not uncommon to see infants whose PHVD has been treated adequately with an appropriate intervention are still suffering from serious neurological disability and even infants with less severe IVH (grades I and II) tend to develop severe neurological deficits, disproportionate to their initial hemorrhage.[36],[37],[38],[39],[40] Thus, there appears a role of early removal of the intraventricular blood and its breakdown products in the management of IVH/PHVD of prematurity. Use of intraventricular fibrinolytic agents has been proposed in the past for the treatment of PHH.[41],[42] However, most of the studies using various intraventricular agents, including recombinant tissue plasminogen activator (rtPA), streptokinase, and urokinase have failed to prove effectiveness in the treatment of IVH.[21]

 » DRIFT Trial Top

The drainage, irrigation, and fibrinolytic therapy (DRIFT) is so far the best study to provide direct evidence in favor of removal of blood from the ventricles to improve outcome.[43] The aim of the trial was to decrease mortality and cognitive, motor, and sensory disabilities. The procedure involves continuous ventricular irrigation for 72 h to allow clearance of blood and its products from the CSF. The trial was suspended prematurely because of suspected treatment futility and increased risk of secondary bleeding. The short-term outcomes from this randomized controlled trial showed no reduction in shunt surgery or death in the DRIFT intervention arm. In the long-term follow-up, however, the study demonstrated a significant reduction in the proportion of children with severe cognitive disability or death at 2 years in the DRIFT arm, from 71% to 54%, and the mean cognitive quotient score was 69.3 in the DRIFT group and 53.7 in the standard treatment group.[44] This benefit was maintained at 10-year follow-up.[45] The DRIFT is not recommended for the treatment of PHH because of its resource-intensive nature and the risk of secondary hemorrhage, but it sparked a number of research efforts and opportunities to investigate optimal treatment approaches for PHH.

 » Neuroendoscopic Ventricular Lavage Top

The hypothesis of NEL was based on the 2-year outcome results of DRIFT trial which showed a lower morbidity and reduced cognitive disability in survivors.[44] The technique involves the use of a neuroendoscope to gently irrigate the ventricular cavity, remove the blood clots from both lateral ventricles through a septostomy, ensure patency of the foramina of Monro, and control bleeding sites under direct visualization [Figure 1]. Unlike the technique used in DRIFT, NEL allows for a complete control of inflow, outflow, and intracranial volume balance in a sterile operative setting. Infusion of thrombolytic or other agents which may increase risk of complications is avoided. The procedure is short and associated with reduced risks of infection and fluctuations in CSF pressure.
Figure 1: Endoscopic view of the right lateral ventricle during neuroendoscopic lavage in a patient who had developed intraventricular hemorrhage and posthemorrhagic hydrocephalus. (a) Heavily blood-stained CSF and unclear view of the intraventricular structures in the beginning of the procedure; (b) Clearer vision and a better view of the ventricular anatomy after continuous fluid irrigation and aspiration of the blood clots; (c) More clear view of the ventricle and the site of a septostomy. Abbreviations: CP - choroid plexus, F - fornix, FM - foramen Monro, S - septostomy, SP - septum pellucidum

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There is no class I evidence of its efficacy but retrospective evaluations have shown encouraging results regarding the procedure safety, reductions in VentriculoPeritoneal Shunt (VPS) insertion and revision rates, and favorable neurodevelopment outcome.[46],[47],[48],[49],[50] Schulz et al.[51] reported a significant reduction in the rates of shunt placement (from 100% to 58%), infection, shunt occlusion, and multiloculated hydrocephalus in the NEL group. There also appears a trend in their series toward a longer period of shunt-free interval in the NEL group compared with the conventional treatment group. This is important because by the time the shunt insertion is required, the CSF characteristics and the patient's age, weight, and general condition improve in favor of a safe shunt surgery. However, the majority of these studies have potential limitations, including the design of the studies, small patient sample, and no systematic assessment of the neurodevelopmental outcomes, and hence warrant validation in an appropriately structured prospective randomized trial.[49]

Permanent CSF diversion

Once temporizing measure is used, a close monitoring with regular head circumference measurement as well as periodic clinical and USG assessments is followed to ensure control of PHVD. A careful evaluation at or around term-equivalent age or when the bodyweight is 1.8–2.0 kg is essential to determine the ongoing need for the temporizing measures and/or to consider permanent CSF diversion in the form of a VPS/ETV. Additional data can be obtained from CSF sampling (protein levels <1.5 g/L, normal cytology, and negative cultures) and brain imaging with MRI. In recent large series of PHH in premature infants, the VPS placement rates have been reported to vary from 58%–100%.[7],[20],[22],[50],[52],[53]

One of many challenges with permanent shunt insertion in premature infants with PHH is infection, which occurs in 13.8% of cases.[52],[54] The high rate of infection in this population is mainly due to an immature immune system and/or an inherent impaired inflammatory response.[55] Also, the thin and fragile skin of LBW infants is vulnerable to frequent wound breaks and ulcerations over the shunt valve and tubing. Thus, delaying the procedure until the infant is grown older is likely to reduce the risk of shunt infection and other complications significantly. Most authors agree that the following must be present for a VP shunt to function in a premature infant with PHH: a mature immune system, an adequate capacity of the peritoneal cavity, the effective elimination of blood products from the CSF, and sufficient thickness of subcutaneous tissue.

Recent literature suggests that shunt failure can occur in up to 12.6% of cases within the first 3 months of VP shunt placement and about 45% of shunts in preterm PHH require shunt revision within 9 months.[52],[54] With regards to shunt infection, results with the currently available silver or antibiotic impregnated catheters are promising and the recent British Antibiotic and Silver Impregnated Catheters for ventriculoperitoneal (Shunts) BASIC study has demonstrated a 3-fold reduction in shunt infection using antibiotic impregnated catheters compared to standard or silver impregnated catheters.[56] There is no clear consensus on the optimal valve type to use in these premature infants. Many authors advocate the use of programmable valves to avoid potential over drainage and complications like subdural hygroma or slit-ventricular syndrome.[57] Peritoneal complications of VP shunts like bowel perforation, necrotizing enterocolitis, pseudocyst formation, and malabsorption of CSF are also common in this population and a ventriculo-atrial shunt can be offered as an alternative, if the diameter of the jugular vein is adequate.

ETV with or without choroid plexus coagulation offers an alternative to a shunt in the treatment of PHH, as it provides an opportunity to avoid shunt dependency.[58],[59],[60] However, with a success rate of 37%–40% and limited data available on its efficacy in PHH, ETV can only be advocated in selected patients in whom imaging shows favorable findings like aqueductal stenosis and triventricular hydrocephalus or a patent, nonscarred prepontine cistern.

 » Conclusions Top

The management of prematurity-related IVH/PHVD and PHH is still evolving and the treatment paradigms are constantly changing from time to time since past few decades. Treatment goals have now shifted from saving life to optimizing cognitive function, neurodevelopment, and quality of life in premature infants. The importance of early intervention with clearing the blood and its breakdown products from the ventricles has been realized by all and the newer techniques have been devised or modified for a safe and effective treatment and improved long-term outcome. In this respect, the preliminary results of newer techniques like VSGSs and NEL are promising. Based on the recent literature, the contemporary management of IVH/PHH recommends early diagnosis and intervention using temporizing measure (VSGS, or NEL) and prevention of future complications of PHH with a permanent CSF diversion method such as ventricular shunting or ETV. Since the cognitive outcomes are now considered more relevant, the future work should be directed toward identifying earlier biomarkers of cognitive neurodevelopmental outcome in order to define safe and more effective treatment protocols.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

 » References Top

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  [Figure 1]

  [Table 1], [Table 2], [Table 3], [Table 4]


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