AUTHOR(S): Weninger, Manfred, M.D.; Salzer, Hans Robert, M.D.; Pollak, Arnold, M.D.; Rosenkranz, Martina, M.D.; Vorkapic, Peter, M.D.; Korn, Alexander, M.D.; Lesigang, Christoph, M.D. Department of Neonatology, University Children's Hospital (MW, HRS, AP, MR) and Department of Neurosurgery (PV, AK), University of Vienna, and Developmental Diagnostic Center (CL), Vienna, Austria Neurosurgery 31; 52-58, 1992 ABSTRACT: TWENTY-SEVEN NEWBORN INFANTS (birth weight, 1503 ± 776 g; gestational age, 31 ± 3 wk) (mean ± standard deviation) with rapidly progressive posthemorrhagic hydrocephalus and increased intracranial pressure were treated by external ventricular drainage. The progression of hydrocephalus was arrested during the drainage period in each patient. The drainage was kept in place for 23 ± 9 days, the longest drainage period being 48 days. In 16 of 23 surviving patients, progressive ventricular dilation recurred after removal of the drainage, requiring a definitive shunt implantation (nine ventriculoatrial, seven ventriculoperitoneal). For the remaining seven infants, no further therapy was necessary. Implantation of the permanent shunt was done days 28 to 88 (body weight, 2400 ± 950 g). Bacterial cultures from cerebrospinal fluid and/or the tip of the ventriculostomy catheter were negative in 175 instances and positive in 11 instances (7 patients). No clinical or biochemical evidence of ventriculitis was noted. Four of the 27 patients died of causes unrelated to external ventricular drainage. Twenty-three infants survived. Seventeen of 23 survivors suffered from intraventricular hemorrhage Grade 3; in 7, neurological and developmental outcomes were classified as normal; 9 patients experienced mild to moderate paresis and/or mild to moderate developmental delay; and only 1 patient was severely retarded. Six patients with parenchymal lesions had severe motor and/or developmental handicaps. We consider external ventricular drainage an effective and safe therapy in newborn infants with rapidly progressive posthemorrhagic hydrocephalus and increased intracranial pressure. The ultimate outcome, however, depends mainly on the mode and the extent of the primary brain lesion. KEY WORDS: External ventricular drainage; Intraventricular hemorrhage; Newborn infants; Posthemorrhagic hydrocephalus; Ventriculostomy Posthemorrhagic hydrocephalus (PHH) is a serious sequelae of intraventricular hemorrhage (IVH) in newborns (2,14). In some infants, increased intracranial pressure (ICP) results from rapid ventricular dilation,

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which causes further damage to the brain and impairment of neurological outcome (17,25). Several treatment modalities were described, including medications to decrease cerebrospinal fluid (CSF) production and intermittent drainage procedures (18, 30,34) . This report summarizes our experience with the placement of tunneled ventriculostomy catheters for continuous external ventricular drainage (EVD) in newborn infants with rapidly progressive PHH and elevated ICP. There are few studies referring to this method, mainly in adults and without using bedside ultrasound imaging for assessment of the treatment results (7,8,14,29,33). We also report the outcomes of these children. PATIENTS AND METHODS Clinical data On the basis of the limited success rate that we experienced with multiple tapping in premature infants with progressive PHH, EVD was introduced as an alternative method. Between July 1984 and June 1989, 27 newborns (18 male, 9 female) admitted to the Department of Neonatology, University Childrens' Hospital Vienna, Austria, were included in the study. The mean birth weight was 1503 ± 776 g (mean ± standard deviation), and the mean gestational age was 31 ± 3 weeks. Table 1 gives details of the patients' data. Diagnosis of intraventricular hemorrhage and rapidly progressive posthemorrhagic hydrocephalus Ultrasonography was performed within the first 24 hours of life at 2- to 3-day intervals during the first week, and thereafter as clinically indicated. This was done through the anterior fontanel with a mechanical sector scanner Mark 600 (ATL, Seattle, WA) equipped with 7.5- and 5-MHz rotary scan heads. After the insertion of the EVD, ultrasound investigations were performed weekly while the infants were in the hospital. IVH was graded by the method of Papile et al. (26), and PHH was assessed by the method of Levene (16). PHH was defined as rapidly progressive if ventricular dilation was accompanied by a growth of the head circumference of more than 2 cm within 1 week and/or if other clinical signs of increased ICP, such as bulging fontanel, widening of the sutures, apnea, bradycardia, or seizures, occurred. Technique of external ventricular drainage Ventriculostomy was performed within 24 hours after the diagnosis of rapidly progressive PHH was confirmed, except when immediate intervention was required. The EVD was inserted under sterile conditions at the neonatal intensive care unit while the infant was lying in the supine position in the incubator. The catheter (Cordis; Cordis Corporation, Miami, FL) was placed into the dilated anterior horn of the right lateral ventricle except for those with a large clot in the right ventricle, when the left side was used. After the head was shaved and disinfected the skin and dura were incised at the lateral angle of the

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Neurosurgery 1992-98 July 1992, Volume 31, Number 1 52 External Ventricular Drainage for Treatment of Rapidly Progressive Posthemorrhagic Hydrocephalus Clinical Study

Follow-up studies Infants were examined at 3-month intervals in our Developmental Diagnostic Center. The first examination was performed before hospital discharge. The follow-up included a full neurological evaluation, Denver developmental screening test, and hearing and vision tests. Cerebral palsy was diagnosed according to the usual criteria and defined as such only if paresis was a constant finding. Transient neurological dysfunction for the first year of life that later resulted in a normal neurological development was recorded as normal. Hemiplegias were graded as mild (+) and moderate (++) forms, and diplegias and quadriparesis as mild (+), moderate (++), and severe (+++) forms (1). Developmental delay was graded as mild (+) if delay was less than 15%, moderate (++) if delay was less than 30%, and severe (+++) if delay was less than 50% according to the normative data of the Griffiths' (10) assessment of development. RESULTS

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Cerebral pathologies Nineteen infants exhibited IVH Grade 3, and eight infants exhibited IVH Grade 4. Five of 19 infants with IVH Grade 3 had additional brain pathologies: three had periventricular leukomalacia, one had a primary intraparenchymal hemorrhage, and one developed atrophy of the brain. Ventriculostomy and definitive shunt A total number of 47 catheters (including 12 revisions and 8 subsequent implantations) were inserted in 27 newborn infants. Pressure measurement immediately after the implantation of the catheter revealed increased ICP (>12 cm H2O) in all patients. The mean age at placement of the first EVD was 14 days (range, 3-28 d). The mean total time in place (including all EVDs) was 23 days (range, 10-48 d). The longest time in place without a change of the catheter was 48 days. The average volume of CSF drained per day was 18 ml (range, 6-40 ml). Details are given in Table 2. EVD led to a striking decrease in ventricular size in all infants within 2 to 3 days. Three patients died before the removal of the EVD. Seven of the remaining 24 infants did not experience a relapse of ventriculomegaly after the removal of the EVD and required no further therapy in contrast to 17 children who suffered recurrence of ventricular dilation. Of these, 11 were in stable clinical condition, allowing a definitive shunt implantation, whereas in 6 of 17 infants, one or two subsequent implantations of EVD were necessary because of poor clinical condition before a definitive shunt procedure was possible (Table 2). One patient with a VP shunt died at the age of 62 days from cardiac failure unrelated to the shunt procedure. Ten infants received a VA shunt, and seven received a VP shunt (Table 2). The implantation of the permanent shunt was performed at days 28 and 88 of life (body weight at that time, 2400 ± 950 g). Complications of external ventricular drainage No serious complications were noted such as epidural, subdural, or intracerebral hemorrhages associated with the passage of the ventricular catheter or during the treatment course. In 12 of 47 EVDs, revision of the EVD was necessary because of accidental dislocation, blockage of the catheter, or leakage of the CSF from the puncture site. No ventriculostomy-related infections, e.g., skin infections at the site of implantation or ventriculitis, were noted in this study. Of 186 cultures obtained from CSF or from the tip of the catheter, 175 were negative. Eleven cultures from 7 patients were positive (Table 3); however, neither the clinical condition nor the CSF chemistry and cytology indicated a true infection. Thus, the results of these cultures were considered as exogenous contamination, and no treatment was given. Except in 2 patients (Patients 6 and 19), who suffered from septicemia before the insertion of the EVD, the antibiotic therapy was altered by the clinician according to culture results. In both of those patients, definitive shunts were implanted 4 or 5 days

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anterior fontanel. A needle catheter was positioned into the ventricle and when CSF flow was achieved, the needle was replaced by the ventricular catheter. Depending on the size of the lateral ventricles, the catheter was advanced 1 to 3 cm. During this procedure, the end of the catheter was clamped to avoid loss of CSF. A second incision was performed 3 to 5 cm away from the catheter, and the proximal end of the catheter was led there through a subcutaneous tunnel. The free end of the catheter was fixed on the skin with sutures and connected to a special drainage system that included a scale for the registration of the ICP. The connection was wrapped in sterile gauze covered with iodine ointment (Betaisadona; Mundipharma GmbH, Limburg/Lahn, Germany). The drip chamber was placed at a level of 8 cm of water so that the CSF was draining only when the ICP was higher than the chosen level. We tried to obtain a total volume of 10 to 15 ml of CSF/kg body weight per day, which was controlled by elevating or lowering the level of the drip chamber. The drainage system was clamped when the infant was very active and during interventions, so that there were no uncontrolled losses of CSF. The CSF collection bag was changed every 24 hours, and the whole drainage system was changed every other day. All patients with an EVD were on antibiotics. Specimens of CSF were obtained for determination of glucose, protein, and cultures whenever the system was changed. In addition, the tip of the catheter was cultured after removal. The ventriculostomy catheters were generally removed when the ICP was 8 cm H2O or less over several days and when the amount of CSF obtained decreased significantly. If the ventricular system remained enlarged after the removal of the EVD and if the infant was found to be in stable condition, a definite shunt was placed. A ventriculoatrial (VA) shunt was implanted if the protein concentration in the CSF was below 200 mg%. In all other cases, a ventriculoperitoneal (VP) shunt was performed.

Outcome Four patients died, 2 from cardiac failure associated with bronchopulmonal dysplasia (BPD), 1 patient from anuria, and 1 from hepatic failure. In 23 survivors, follow-up studies over a period of 18 to 62 months were performed (Table 1). In 17 of 23 surviving patients, an ultrasound of the brain revealed an IVH Grade 3. Seven of 17 patients with IVH Grade 3 exhibited a normal neuromotor development, 6 had spastic paresis and mild to moderate developmental delay, and 3 were mildly to moderately retarded without neurological handicap. Only 1 patient who developed atrophy of the brain was severely retarded. Six of 7 patients with IVH Grade 3 and normal neurological outcome had a definitive shunt. In patients with IVH Grade 3, additional pathologies found by ultrasound such as periventricular leukomalacia and primary intraparenchymal hemorrhage had an unfavorable effect on final outcome. The same was true for other variables such as low birth weight, low gestational age, hemodynamically effective patent ductus arteriosus, and BPD (Table 4). All six survivors with IVH Grade 4 had a poor neurological outcome. Three had quadriparesis and moderate to severe retardation, two had diplegia and severe retardation, and one was severely retarded (Table 1). DISCUSSION PHH is the most important complication of IVH in newborn infants. In patients with slowly progressive PHH and spontaneous involution of ventricular dilation, as well as in patients with mild PHH without symptoms of increased ICP, there may be no need for therapeutic intervention. If the PHH is rapidly increasing and accompanied by rising ICP, early correction of the hydrocephalus and reduction of the ICP is mandatory. There is experimental evidence of brain injury caused by increased ICP, including axonal stretching and gliosis of axons. These changes were reversible by early intervention (24,28,32,35). Clinical observations, such as studies of cerebral blood flow velocity (12), visual evoked potentials (23), and CSF markers of hypoxia (4), also suggested an impairment of brain function by increased ICP, which could be corrected by the treatment of the ventricular dilation. Several methods have been proposed for the treatment of PHH including medical therapy with acetazolamide, furosemide, and inositol for reducing the CSF production rate (18,30). PHH, however, results from impairment of the CSF absorption due to obliterative arachnoiditis and only to a lesser extent from overproduction of CSF (3,31). In addition, medical therapy is effective after several days and CSF production is reduced by only one third (22). Thus, medical therapy appears to be indicated only in infants with slowly progressive PHH and not in those in the acute state of increased ICP. Other modalities include serial lumbar punctures, serial ventricular punctures, the placement of a

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subcutaneous ventricular catheter reservoir, the placement of a ventriculoperitoneal shunt, and the insertion of an external ventricular drainage. Serial lumbar puncture is most frequently used for temporary amelioration of neonatal PHH (9,15,19,27,34). There are, however, significant drawbacks to this method (5). First, this method is only effective if there is a free communication between the ventricles and the lumbar subarachnoid space. Second, critically ill, premature infants will suffer from recurrent stress caused by the frequent painful punctures over a short period of time (15). Third, these sick premature infants have to remain in a lateral position for at least 15 to 60 minutes, which interferes with their care during the procedure. Fourth, if serial lumbar punctures are effective, relatively large amounts of CSF (10-20 ml) are removed within a short period. This might influence cerebral circulation and lead to further cerebral damage (22). With serial ventricular punctures, one has direct access to the enlarged ventricles. Repetitive ventricular taps, however, would be at the expense of additional parenchymal damage and possible porencephaly at the aspiration site. Ventricular subcutaneous catheter reservoirs have not received much clinical attention as yet because of side effects such as skin breakdown and wound infection (20). The placement of ventriculoperitoneal shunts in low-birthweight infants within the first 3 weeks of life, subsequent to lumbar punctures, were associated with an overall infection rate of 50%, a revision rate of 68%, and a median number of four revisions in these patients (13). The hypothesis of our study was that EVD would offer a better alternative for the treatment of rapidly increasing PHH on the basis of the continuous and controlled decrease of ICP and the rapid detection of occult shunt dysfunction. Surprisingly, the EVD was well tolerated even in the sickest premature infants for over several weeks. We were able to continuously register ICP and simultaneously adjust flow velocity if necessary, which we would not have been able to do by other methods. By this means, we could prevent uncontrolled and rapid CSF drainage, which in turn might cause additional problems such as subdural hematoma or effusion. Moreover, the procedure was performed in the intensive care unit within 15 to 20 minutes without removing the patient from the incubator. The rate of complication for EVD in our series was low in contrast to earlier reports of infections associated with a longer duration of EVD (21). Although the mean duration of EVD in our patients was 23 days (maximum, 48 d), no infection of the CSF occurred. Tunneling of the catheter and strict sterile conditions for the implantation of the catheter and for the change of the drainage system seem to be crucial for preventing infections. Similarly, no infections were reported by Harbaugh et al. (11) in a small group of seven infants with a similar mean duration of drainage. Two larger studies using the tunneling technique also showed no infection; however, these studies differed substantially with regard to patient selection and methodology (8,29).

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Received, October 3, 1991. Accepted, October 28, 1991. Reprint requests: M. Weninger, M.D., Department of Neonatology, University Children's Hospital Vienna, Währinger-Gürtel 18-20, A-1090 Wien, Austria.

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Kreusser et al. (14) reported an infection rate of 10% with EVD without the tunneling technique, although the duration of the EVD was only 5 to 7 days. The accidental dislocation of the catheter in very active infants and blockage of the tube with blood clots or high protein content of CSF requiring revision of the catheter are potential complications. All patients with IVH Grade 4 had a poor neurodevelopmental outcome. The early control of hydrocephalus did not seem to improve their neurological outcome, and treatment with EVD might be of limited value in these patients. Similarly, previous studies indicated that preexisting parenchymal injury is the most important determinant of neurological outcome (2,6). Although outcome in patients with parenchymal lesions is generally poor, a recent randomized trial of early tapping in neonatal posthemorrhagic ventricular dilation showed a significant reduction of additional impairments (34). In contrast, 41% of patients with IVH Grade 3 showed a normal neurodevelopmental outcome, and no patient exhibited severe paresis or multiple handicaps. Patients with IVH Grade 3 seem to benefit from the early intervention of rapidly progressive PHH and perhaps have improvement in their subsequent neurological function. Randomized, prospective controlled studies are, however, necessary to confirm these assumptions because other variables such as low gestational age, low birth weight, or BPD were associated with poor neurological outcome in patients with IVH Grade 3. Moreover, controlled multicenter studies are necessary to evaluate the benefit and risk of EVD in comparison with other treatment modalities currently used.

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COMMENTS This article details the results of a treatment with external ventricular drainage of a small series of 27 children born with progressive hydrocephalus, and the authors have included the functional outcome of their series. Although their results are as good as anything published, the methodology has been tried by multiple centers in this country and most have now abandoned the use of external ventricular drainage. Most centers use reservoirs with intermittent tapping to gradually lower the intracranial pressure. As the hydrocephalus comes under control, the decision to shunt or remove the reservoir is made. In our experience, the risk of infection with the use of extraventricular drainage is significantly increased. Seven of 27 patients in this series had positive cultures but were not considered to have ventriculitis. The reservoir in our hands, and as reported by others, has an extremely low incidence of infection. In fact, in our experience, the incidence of infection is higher with repeat lumbar punctures than it is with the use of the reservoir. Most of the children requiring a shunt had ventriculoatrial shunts placed. The long-term complications of a ventriculoatrial shunt are such that I consider placement of the distal catheter in the heart to be the last choice. David G. McLone Chicago, Illinois The work reviewed here is extremely important for two reasons. It provides excellent data relative to the safety and efficacy of tunneled external ventricular drains in the management of rapidly progressive hydrocephalus in the preterm infant who has suffered an intraventricular hemorrhage. The management of this condition is controversial with management possibilities including intermittent ventricular or lumbar punctures, brain dehydration, early internal shunting, or intermittent tapping with implanted reservoirs. This study was a retrospective analysis of one of these treatment modalities, but the treatment was very successful and impressively safe. The zero percent infection rate is testimony to the care with which the systems were placed and maintained, but in these high-risk infants in potentially contaminated environment, even with enough experience, ventriculitis is probably inevitable in a small percentage of patients.

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21.

The other very important point made by the authors relates to the positive long-term outcome of children managed in this way. This is particularly true of children suffering Grade 3 hemorrhages. Six of 23 of their patients who survived did not require a permanent shunt. Only 1 of their 17 patients with posthemorrhagic hydrocephalus from Grade 3 hemorrhages was profoundly retarded. Seven of 16 had normal neuromotor development, and 10 had significant handicaps. All six survivors of Grade 4 hemorrhage had a poor neurological outcome. These data are extremely important to have when counseling families regarding the treatment of posthemorrhagic hydrocephalus. This article strongly supports an aggressive posture regarding the treatment of children with Grade 3 intraventricular hemorrhage who have rapidly progressive ventriculomegaly. Although different neonatal intensive care units vary as to their ability to manage ventriculostomy drainage, this work clearly shows that when properly managed, tunneled external ventricular drainage is an effective way to manage this condition.

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Harold L. Rekate Phoenix, Arizona

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Table 1. Patients' Data and Study Outcome Redistribution of this article permitted only in accordance with the publisher’s copyright provisions. Downloaded from https://academic.oup.com/neurosurgery/article-abstract/31/1/52/2751932 by East Carolina University Health Sciences Library user on 17 March 2018

Table 3. Positive Cultures of CSF or Ventriculostomy Catheter (n = 11)a

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Table 2. Characteristics of Study Population (External Ventricular Drainage Shunt)a

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Table 4. Clinical Risk Factors and Outcome in Patients with Intraventricular Hemorrhage Grade 3

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External ventricular drainage for treatment of rapidly progressive posthemorrhagic hydrocephalus.

Twenty-seven newborn infants (birth weight, 1503 +/- 776 g; gestational age, 31 +/- 3 wk) (mean +/- standard deviation) with rapidly progressive posth...
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