Arrested Hydrocephalus in Childhood: Case Series and Review of the Literature

Yannick Hurni; Andrea Poretti; Jacques Schneider; Raphael Guzman; Gian Paolo Ramelli

doi:10.1055/s-0038-1660854

Neuropediatrics, Table of Contents

Neuropediatrics 2018; 49(05): 302-309
DOI: 10.1055/s-0038-1660854

Original Article

Georg Thieme Verlag KG Stuttgart · New York

Arrested Hydrocephalus in Childhood: Case Series and Review of the Literature

Authors

Yannick Hurni

¹Pediatric Department of Southern Switzerland, Neuropediatric Unit, San Giovanni Hospital, Bellinzona, Switzerland
Andrea Poretti^*

²Section of Pediatric Neuroradiology, Division of Pediatric Radiology, Russell H. Morgan Department of Radiology and Radiological Sciences, The Johns Hopkins University School of Medicine, Baltimore, United States of America
Jacques Schneider

³Department of Radiology, Hirslanden Klinik Aarau, Aarau, Switzerland
Raphael Guzman

⁴Department of Neurosurgery, University Children's Hospital, UKBB, Basel, Switzerland
Gian Paolo Ramelli

¹Pediatric Department of Southern Switzerland, Neuropediatric Unit, San Giovanni Hospital, Bellinzona, Switzerland

Abstract

Introduction Hydrocephalus can be progressive or spontaneously arrested. In arrested hydrocephalus, the balance between production and absorption of the cerebrospinal fluid is restored. Patients are mostly asymptomatic, and no surgical treatment is necessary for them.

Methods We performed a two-center consecutive case series study, aimed at investigating the safety of nonsurgical management of hydrocephalus in selected pediatric patients. We retrospectively selected all consecutive patients, suspected to suffer from arrested hydrocephalus and referred to our two institutions between January 2011 and December 2013. Data on clinical and radiological follow-up were collected until June 2017.

Results Five children diagnosed with arrested hydrocephalus were included in the study. All patients presented macrocephaly as the main presenting sign. Associated mild-to-moderate stable motor disorders were assessed in four out of five cases. Typical symptoms and signs associated with acute raised intracranial pressure were absent in all patients. Magnetic resonance imaging studies showed ventriculomegaly in all patients. A diagnosis of arrested hydrocephalus was made in all five cases based on stable clinical and radiological findings during the initial observation. Conservative management based on active surveillance was, therefore, proposed. During the follow-up period, we observed stable or improved conditions in four out of five patients, while the remaining patient presented progressive hydrocephalus.

Discussion Making a distinction between arrested and progressive hydrocephalus is fundamental, because of the opposed appropriate management. Any newly discovered case of hydrocephalus, not characterized by clear signs of progressive hydrocephalus, should benefit from active surveillance before any definitive decision is taken.

Keywords

hydrocephalus - arrested hydrocephalus - macrocephaly - childhood

Full Text

References

References
1 Rekate HL. The definition and classification of hydrocephalus: a personal recommendation to stimulate debate. Cerebrospinal Fluid Res 2008; 5: 2
2 Del Bigio MR. Neuropathological changes caused by hydrocephalus. Acta Neuropathol 1993; 85 (06) 573-585
3 Laurence KM, Coates S. The natural history of hydrocephalus. Detailed analysis of 182 unoperated cases. Arch Dis Child 1962; 37: 345-362
4 Schick RW, Matson DD. What is arrested hydrocephalus?. J Pediatr 1961; 58: 791-799
5 Johnston I, Teo C. Disorders of CSF hydrodynamics. Childs Nerv Syst 2000; 16 (10-11): 776-799
6 O'Hayon BB, Drake JM, Ossip MG, Tuli S, Clarke M. Frontal and occipital horn ratio: a linear estimate of ventricular size for multiple imaging modalities in pediatric hydrocephalus. Pediatr Neurosurg 1998; 29 (05) 245-249
7 Braegger C, Jenni O, Konrad D, Molinari L, Staubli S. “Courbes de Croissance Validées par la Société Suisse de Pédiatrie”. Swiss Society of Paediatrics. Available at: http://www.swiss-paediatrics.org/sites/default/files/2017-08/Perzentilen_2012_09_15_SGP_f.pdf . Updated September 12, 2012. Accessed March10, 2018
8 Yashon D, Jane JA, Sugar O. The course of severe untreated infantile hydrocephalus. Prognostic significance of the cerebral mantle. J Neurosurg 1965; 23 (05) 509-516
9 Bech RA, Juhler M. Unusual clinical manifestations of disturbed CSF dynamics in hydrocephalic children. Childs Nerv Syst 2000; 16 (07) 446-449 , discussion 450
10 Mataró M, Poca MA, Sahuquillo J. , et al. Cognitive changes after cerebrospinal fluid shunting in young adults with spina bifida and assumed arrested hydrocephalus. J Neurol Neurosurg Psychiatry 2000; 68 (05) 615-621
11 Kirkpatrick M, Engleman H, Minns RA. Symptoms and signs of progressive hydrocephalus. Arch Dis Child 1989; 64 (01) 124-128
12 Rudolph D, Sterker I, Graefe G, Till H, Ulrich A, Geyer C. Visual field constriction in children with shunt-treated hydrocephalus. J Neurosurg Pediatr 2010; 6 (05) 481-485
13 McAuley D, Paterson A, Sweeney L. Optic nerve sheath ultrasound in the assessment of paediatric hydrocephalus. Childs Nerv Syst 2009; 25 (01) 87-90
14 Singhal A, Yang MM, Sargent MA, Cochrane DD. Does optic nerve sheath diameter on MRI decrease with clinically improved pediatric hydrocephalus?. Childs Nerv Syst 2013; 29 (02) 269-274
15 Matsumae M, Hirayama A, Atsumi H, Yatsushiro S, Kuroda K. Velocity and pressure gradients of cerebrospinal fluid assessed with magnetic resonance imaging. J Neurosurg 2014; 120 (01) 218-227
16 Dinçer A, Kohan S, Ozek MM. Is all “communicating” hydrocephalus really communicating? Prospective study on the value of 3D-constructive interference in steady state sequence at 3T. AJNR Am J Neuroradiol 2009; 30 (10) 1898-1906
17 Barkovich AJ, Kjos BO, Norman D, Edwards MS. Revised classification of posterior fossa cysts and cystlike malformations based on the results of multiplanar MR imaging. AJR Am J Roentgenol 1989; 153 (06) 1289-1300
18 Kiefer M, Eymann R, von Tiling S, Müller A, Steudel WI, Booz KH. The ependyma in chronic hydrocephalus. Childs Nerv Syst 1998; 14 (06) 263-270
19 Kulkarni AV, Drake JM, Armstrong DC, Dirks PB. Measurement of ventricular size: reliability of the frontal and occipital horn ratio compared to subjective assessment. Pediatr Neurosurg 1999; 31 (02) 65-70
20 Leliefeld PH, Gooskens RH, Tulleken CA. , et al. Noninvasive detection of the distinction between progressive and compensated hydrocephalus in infants: is it possible?. J Neurosurg Pediatr 2010; 5 (06) 562-568
21 Leliefeld PH, Gooskens RH, Braun KP. , et al. Longitudinal diffusion-weighted imaging in infants with hydrocephalus: decrease in tissue water diffusion after cerebrospinal fluid diversion. J Neurosurg Pediatr 2009; 4 (01) 56-63
22 Leliefeld PH, Gooskens RH, Vincken KL. , et al. Magnetic resonance imaging for quantitative flow measurement in infants with hydrocephalus: a prospective study. J Neurosurg Pediatr 2008; 2 (03) 163-170
23 Schulz M, Ahmadi SA, Spors B, Thomale U-W. Intracranial pressure measurement in infants presenting with progressive macrocephaly and enlarged subarachnoid spaces. Acta Neurochir Suppl (Wien) 2012; 114: 261-266
24 Lundberg N. Continuous recording and control of ventricular fluid pressure in neurosurgical practice. Acta Psychiatr Scand Suppl 1960; 36 (149) 1-193
25 Iborra J, Pagès E, Cuxart A, Poca A, Sahuquillo J. Increased intracranial pressure in myelomeningocele (MMC) patients never shunted: results of a prospective preliminary study. Spinal Cord 2000; 38 (08) 495-497
26 Lilja A, Andresen M, Hadi A, Christoffersen D, Juhler M. Clinical experience with telemetric intracranial pressure monitoring in a Danish neurosurgical center. Clin Neurol Neurosurg 2014; 120: 36-40
27 Oi S, Di Rocco C. Proposal of “evolution theory in cerebrospinal fluid dynamics” and minor pathway hydrocephalus in developing immature brain. Childs Nerv Syst 2006; 22 (07) 662-669
28 Shen XQ, Miyajima M, Ogino I, Arai H. Expression of the water-channel protein aquaporin 4 in the H-Tx rat: possible compensatory role in spontaneously arrested hydrocephalus. J Neurosurg 2006; 105 (6, Suppl): 459-464
29 Greitz D, Greitz T, Hindmarsh T. A new view on the CSF-circulation with the potential for pharmacological treatment of childhood hydrocephalus. Acta Paediatr 1997; 86 (02) 125-132
30 Luciano MG, Skarupa DJ, Booth AM, Wood AS, Brant CL, Gdowski MJ. Cerebrovascular adaptation in chronic hydrocephalus. J Cereb Blood Flow Metab 2001; 21 (03) 285-294
31 Johnston M. The importance of lymphatics in cerebrospinal fluid transport. Lymphat Res Biol 2003; 1 (01) 41-44 , discussion 45
32 Johnston M, Zakharov A, Papaiconomou C, Salmasi G, Armstrong D. Evidence of connections between cerebrospinal fluid and nasal lymphatic vessels in humans, non-human primates and other mammalian species. Cerebrospinal Fluid Res 2004; 1 (01) 2
33 Greitz D. Radiological assessment of hydrocephalus: new theories and implications for therapy. Neurosurg Rev 2004; 27 (03) 145-165 , discussion 166–167
34 Yashon D. Prognosis in infantile hydrocephalus: past and present. J Neurosurg 1963; 20: 105-111
35 Brookshire BL, Fletcher JM, Bohan TP, Landry SH, Davidson KC, Francis DJ. Verbal and nonverbal skill discrepancies in children with hydrocephalus: a five-year longitudinal follow-up. J Pediatr Psychol 1995; 20 (06) 785-800
36 Del Bigio MR, Wilson MJ, Enno T. Chronic hydrocephalus in rats and humans: white matter loss and behavior changes. Ann Neurol 2003; 53 (03) 337-346
37 Hagberg B, Sjorgen I. The chronic brain syndrome of infantile hydrocephalus. A follow-up study of 63 spontaneously arrested cases. Am J Dis Child 1966; 112 (03) 189-196
38 Rickert CH, Grabellus F, Varchmin-Schultheiss K, Stöss H, Paulus W. Sudden unexpected death in young adults with chronic hydrocephalus. Int J Legal Med 2001; 114 (06) 331-337