Novel Insights from Clinical Practice Received: December 13, 2013 Accepted after revision: April 27, 2014 Published online: July 29, 2014

Pediatr Neurosurg 2013;49:236–239 DOI: 10.1159/000363330

Foreign Body Granuloma Secondary to Ventriculo-Peritoneal Shunt: A Rare Scenario with a New Insight Christopher Paul Millward a Sandra Perez da Rosa a Dawn Williams a George Kokai b Aideen Byrne c Benedetta Pettorini a  

 

 

 

 

 

Departments of a Neurosurgery, b Histopathology and c Paediatrics, Alder Hey Children’s NHS Foundation Trust, Liverpool, UK  

 

 

Established Facts • Allergic reactions to silicone shunts are a rare but poorly understood cause of shunt failure. • Aseptic cerebrospinal fluid (CSF) with CSF eosinophilia is pathognomonic in such allergic reactions; hypoallergenic ‘extracted’ shunt systems can be used to overcome shunt allergy.

Novel Insights • We recognise non-allergic intolerance to a silicone shunt as a cause of shunt rejection, requiring multiple shunt revision surgeries.

Key Words Granuloma · Ventriculo-peritoneal shunt · Shunt revisions

Abstract Ventriculo-peritoneal shunts are used extensively for the management of hydrocephalus and frequently present with complications such as shunt blockage and infection. Cerebrospinal fluid (CSF) eosinophilia and allergic responses to the shunt itself are rare, poorly understood but increasingly recognised complications. Here, the authors describe a child who required multiple shunt revision surgeries due to exten-

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sive scalp tenderness overlying the shunt tubing and persistent severe headaches despite having a normal working shunt and no CSF infection or eosinophilia. Histological investigation of excised tissue during the last shunt revision demonstrated fibrosis with scar tissue and chronic inflammatory infiltrate with foreign body giant cells and few abortive granulomata. This was felt to represent a foreign body reaction to the shunt. A hypoallergenic ‘extracted’ shunt was trialled (extracted Delta® valve and extracted ventricular and peritoneal catheters; Medtronic) and the child has had no further shunt revisions and is currently asymptomatic 1 year after the insertion. © 2014 S. Karger AG, Basel

Christopher Paul Millward Department of Neurosurgery Alder Hey Children’s NHS Foundation Trust Eaton Rd, Liverpool L12 2AP (UK) E-Mail christopherpaulmillward @ gmail.com

Introduction

The shunting of cerebrospinal fluid (CSF) due to hydrocephalus is one of the most commonly performed procedures in neurosurgery [1]. The imbalance between the generation, reabsorption and circulation of CSF is usually overcome surgically with the use of a ventriculoperitoneal (VP) shunt which diverts excessive CSF into the peritoneal cavity. The failure rates of VP shunts are estimated to be 30% within the first year, with only 15% being complication free at 10 years [2]. The most common reasons for shunt failure are blockage and infection [3, 4]. An abnormal reaction to shunt components has become rare since the introduction of silicone shunts in 1955 by Pudenz et al. [5]. However, CSF eosinophilia and allergic responses to silicone shunts, although poorly understood, are being increasingly reported within the literature [6–13]. We describe a more unusual case of repeated shunt revisions in a child due to severe pain and tenderness in the scalp overlying the shunt tubing. Histological investigations demonstrated foreign body granuloma formation but, unlike other reported cases of shunt rejection, we did not observe CSF eosinophilia. Management was successful with an ‘extracted’ hypoallergenic shunt (extracted Delta® valve and extracted ventricular and peritoneal catheters; Medtronic).

Case Report

Fig. 1. T1-weighted coronal MRI image – hydrocephalus with

slightly more dilation of the right ventricle compared with the left ventricle.

on the right side of his head corresponding to the location of the shunt. These symptoms continued and 2 months later a left frontal intracranial pressure monitor was inserted for 48 h. Pressure was recorded on the low side and, following failed medical management with amitriptyline, a MIETHKE proSA® anti-siphon device was inserted – set at 30 to manage the slit ventricle syndrome. Following this operation, the patient’s condition improved. After 4 months, the child once again presented with severe pain over the shunt site from behind the ear all the way to the thorax. The shunt was revised, with the valve and distal catheter being replaced with a plain one, but once again CSF was negative for infection and no eosinophilia was observed. A subcutaneous biopsy taken at the time of the revision demonstrated some fibrosis with chronic inflammatory infiltrate, mainly around congested vessels, and foreign body granuloma formation (fig. 2). It was decided that a hypoallergenic shunt may be of benefit. Therefore, an extracted shunt (extracted Delta valve and extracted ventricular and peritoneal catheters; Medtronic) was inserted. The child has had no further shunt revisions and is currently asymptomatic 1 year after the insertion.

This 14-year-old male child first presented to the neurosurgical department at Alder Hey Children’s NHS Foundation Trust at the age of 10 years with a 9-month history of episodic, severe and diffuse headache exacerbated by movement. Neurological examination and fundoscopy were entirely normal. MRI demonstrated hydrocephalus with dilation of the right lateral ventricle slightly more than the left – confirmed intra-operatively during the first endoscopy to be due to a membrane at the foramen of Munro (fig. 1). The patient underwent image-guided endoscopic foraminoplasty and septum pellucidotomy in July 2010. Although the postoperative period was uneventful, the headaches continued and further imaging demonstrated increased dilation of the right lateral ventricle. Therefore, a right-sided VP shunt (antibiotic-impregnated) was inserted to manage slit ventricle syndrome and the headaches resolved. At 11 months after the VP shunt insertion the patient presented with tenderness along the shunt tube at the head and neck. On examination, redness was seen on the skin overlying the tubing behind the ear. This was felt to represent a delayed shunt infection which required removal of the shunt, external ventricular drain insertion and 2 weeks of antibiotics. CSF at that time was negative for infection and no eosinophilia was observed. A new shunt was then inserted. The patient continued to complain of pain localised

By far the most frequent and challenging complications of shunt placement for the management of hydrocephalus are infection and blockage, despite advancing operative protocols [14, 15], antibiotic impregnated catheters [16] and endoscopic strategies for their insertion [17]. However, allergic or foreign body reactions to stan-

Foreign Body Granuloma and Shunts

Pediatr Neurosurg 2013;49:236–239 DOI: 10.1159/000363330

Discussion

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b

Color version available online

a

Fig. 2. a Overview of the skin sample with

continuous epithelial cover, fibrosis in the upper dermis and scar tissue formation in its deeper part (bottom half) where cuffs of chronic inflammatory cell infiltrate are seen around vessels (arrow). HE. Original magnification ×20. b Abortive foreign body granuloma in deep dermis (hashtag). HE. Original magnification ×40. c Cluster of foreign body giant cells with engulfed foreign material (dissolved during tissue processing – white areas). HE. Original magnification ×160.

dard VP shunts represent a rare, poorly understood but important complication. A number of authors have discussed the possible aetiology of what can be described as aseptic shunt malfunction such as that which we describe here. Persistent CSF eosinophilia is reported in the majority of cases, suggesting a silicone allergy [6–13]. Furthermore, studies have attempted to quantify the allergic response to silicone shunts such as the report by VandeVord et al. [13] in 2004. They showed that patients who had aseptic shunt malfunction had a higher rate of protein deposition and increased levels of autoantibodies to the extracted surface proteins compared with those who had normal-functioning shunts [13]. In our case, the patient had repeated episodes of severe scalp pain localised over the shunt tubing, coupled with histological evidence of foreign body-type granuloma formation in the tissue resected from around the shunt during shunt revision. Our patient never had CSF eosinophilia, which excluded the more typical allergic reaction. Transcutaneous exposure to silicon was not performed as this is not a valid test of allergy to silicone. The patient did not have any allergies to antibiotics. We have never removed a shunt due to CSF infection (which was never demonstrated after CSF analysis following each revision) or blockage. This case was successfully managed with the insertion of a hypoallergenic extracted shunt (extracted Delta valve and extracted ventricular and peritoneal catheters; Medtronic). Extracted shunts undergo an extensive solvent extraction process to remove unbound silicone oil and other impurities from the surface of the shunt, which have been suggested to interact with the immune system [18]. 238

Pediatr Neurosurg 2013;49:236–239 DOI: 10.1159/000363330

c

To our knowledge, only a single case is reported in the literature that has demonstrated a need for, and the successful use of, an extracted shunt system for hydrocephalus. Ellis et al. [14] describe a child with repeated shunt revision and CSF eosinophilia, but with aseptic CSF on all occasions, with evidence of inflammatory material causing shunt blockage. The patient has remained free of shunt malfunction for more than 2 years. Extracted silicone has also been used in patients with peritoneal silicone reactions and has resulted in a reduction in complications observed; however, histology was not available to support this [9]. Unlike the case of Ellis et al. [14], our patient did not have CSF eosinophilia, which is normally a diagnostic marker of allergic reactions to shunts [19] or shunt obstruction due to inflammatory mass, but only a local foreign body reaction to the shunt which made the presence of the shunt intolerable. We suggest that, when managing shunt failure, silicone intolerance should be differentiated from silicone allergy and the more common causes of shunt failure, infection and blockage, in order to avoid repeated and unnecessary shunt revisions.

Disclosure Statement The authors have no conflicts of interest to declare.

Millward /Perez da Rosa /Williams /Kokai / Byrne /Pettorini  

 

 

 

 

 

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14 Ellis MJ, Kazina CJ, Del Bigio MR, McDonald PJ: Treatment of recurrent ventriculoperitoneal shunt failure associated with persistent cerebrospinal fluid eosinophilia and latex allergy by use of an ‘extracted’ shunt. J Neurosurg Pediatrics 2008;1:237–239. 15 Jimenez DF, Keating R, Goodrich JT: Silicone allergy in ventriculoperitoneal shunts. Childs Nerv Syst 1994;10:59–63. 16 Aryan HE, Meltzer HS, Park MS, Bennett RL, Jandial R, Levy ML: Initial experience with antibiotic-impregnated silicone catheters for shunting of cerebrospinal fluid in children. Childs Nerv Syst 2005;21:56–61. 17 Kurschel S, Eder HG, Schleef J: CSF shunts in children: endoscopically assisted placement of the distal catheter. Childs Nerv Syst 2005; 21:52–55. 18 Goldblum RM, Pelley RP, O’Donell AA, Pyron D, Heggers JP: Antibodies to silicone elastomers and reactions to ventriculoperitoneal shunts. Lancet 1992;340:510–513. 19 Wiersbitzky SKW, Ahrens N, Becker TH, Panzig B, Abel J, Stenger RD: The diagnostic importance of eosinophil granulocytes in the CSF of children with ventricular-peritoneal shunt systems. Acta Neurol Scand 1998, 97: 201–203.

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Foreign body granuloma secondary to ventriculo-peritoneal shunt: a rare scenario with a new insight.

Ventriculo-peritoneal shunts are used extensively for the management of hydrocephalus and frequently present with complications such as shunt blockage...
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