The ventriculocholecystic shunt: two case reports and

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Feb 27, 2013 - lowing her arrival at our unit, plasminogen deficiency was suspected and ..... shunt in the treatment of refractory hydrocephalus: a review of the.
British Journal of Neurosurgery, 2013; Early Online: 1–4 © 2013 The Neurosurgical Foundation ISSN: 0268-8697 print / ISSN 1360-046X online DOI: 10.3109/02688697.2013.771135

ORIGINAL ARTICLE

The ventriculocholecystic shunt: two case reports and a review of the literature A. K. Demetriades1, I. Z. Haq1, J. Jarosz2, D. McCormick3 & S. Bassi1 1Department of Neurosurgery, King’s College Hospital, Denmark Hill, London, UK, 2Department of Neuroradiology,

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King’s College Hospital, Denmark Hill, London, and 3Department of Paediatric Neurology, King’s College Hospital, Denmark Hill, London, UK

The distal end of the shunt catheter was identified, and subsequently, the gallbladder was exposed. Two purse string sutures were applied to the fundus of the gallbladder, and a cholecystostomy was made between these sutures with a monopolar diathermy. The distal end of the shunt catheter was connected to a straight connector with the slit end of the catheter secured with silk tie sutures. This was passed into the gallbladder for a length of 5 cm and the purse strings tightened over the connector. A 4’0 vicryl was used to close the deep layers of muscle, fascia and fat, and a 4’0 monocryl was used to close skin. A low-pressure one-way valve was also used.

Abstract We describe the re-siting of ventriculoperitoneal shunts to the gallbladder in two children. The first child had a rare case of hydrocephalus associated with plasminogen deficiency. She had had multiple VP shunt revisions due to non-absorption of CSF from the peritoneum. The second had craniopharyngiomarelated hydrocephalus with once again a non-absorbing peritoneum. We report no surgical complications in the revisions for both the cases, and there has been a subsequent follow-up of 46 and 28 months, respectively, without incident. A review of the relevant literature describing the use and the performance of ventriculocholecystic shunts in comparison with other ventricular shunts is considered.

Case reports

Keywords: hydrocephalus; plasminogen deficiency; ventriculocholecystic shunt; ventriculobiliary shunt; ventriculogallbladder shunt; ventriculoperitoneal shunt

Case 1 A 9-month-old girl child was admitted for assessment of her VP shunt, having been treated at another neurosurgical centre prior to this point. Previously, her antenatal ultrasound had shown macrocephaly, and postdelivery, she was confirmed to have hydrocephalus. At 5 weeks of age, she had a VP shunt inserted. Initially, the shunt appeared to be working well and she was discharged. However, she was readmitted with a tense, shiny abdomen, and an abdominal ultrasound confirmed the accumulation of a large amount of intra-abdominal fluid with evidence of worsening hydrocephalus on CT scan. She had 1100 ml of fluid drained from the abdomen under ultrasound guidance with temporary symptomatic improvement. At 4 months of age, she had been diagnosed with ligneous conjunctivitis and was treated with topical fresh frozen plasma and steroid eye drops. Her VP shunt was subsequently revised eight times due to recurrent malfunction before she presented to our department at 9 months. Following her arrival at our unit, plasminogen deficiency was suspected and haematological assessment confirmed low levels of plasminogen on two occasions, 13 IU/dl and

Introduction In complicated ventriculoperitoneal (VP) shunts, particularly in the case of a non-absorbing peritoneum,1 the gallbladder has occasionally been used as a site for CSF diversion. Surgeons, however, uncommonly use the cholecystic system to drain CSF, due to perceived complications and technical difficulties.2 Another reason for this reluctance is that ventriculocholecystic (VC) (or ventriculobiliary or ventriculogallbladder) shunts are occasionally mistaken as VP shunts on plain abdominal radiographs prior to a revision.3 However, VC shunts have had good rates of success.4–7 We report the cases of two children whose VP shunts were re-sited to the gallbladder.

Surgical technique A right subcostal incision was made. Any residual fluid due to previous shunt failures was drained from the abdomen.

Correspondence: Mr. A. K. Demetriades, Department of Neurosurgery, King ’s College Hospital, Denmark Hill, London SE5 9RS, UK. E-mail: andreas. [email protected] Received for publication 12 February 2012; accepted 24 January 2013

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19 IU/dl (70–140 IU/dl), confirming the diagnosis of congenital plasminogen deficiency. Three weeks postadmission, she underwent a third ventriculostomy and Ommaya reservoir insertion. Postoperatively, however, her hydrocephalus persisted. A few days later, the distal end of her shunt was re-sited to the gallbladder under the paediatric surgical and paediatric neurosurgical teams (Fig. 1). In this case, we elected not to use a VA shunt because the fibrinogen deficiency would cause the catheter in the atrium to block. During surgery, sheets of fibrin were noted in the abdomen and also at the sites of peritoneal trauma from previous VP shunts. This was consistent with the abnormal fibrinolysis seen in plasminogen deficiency, and the likeliest reason for non-absorption of CSF from the peritoneum. On the eleventh postoperative day, there was clinical and radiographic evidence of fluid overload and diuretics were commenced. Current follow-up, over 46 months postoperatively, has shown the patient to be progressing developmentally. There have been no problems with her VC shunt.

Case 2 A three-and-a-half-year-old boy was referred from a district hospital with a 3-week history of unsteadiness and visual disturbance, and a 2-month history of occasional morning vomiting and headaches. His antenatal and developmental history was otherwise unremarkable. The initial brain MRI showed an 8 ⫻ 4 cm multiseptated, lobulated, cystic lesion in the sellar and suprasellar region. There was compression of the optic chiasm and optic tracts with posterior displacement of the brainstem. This caused aqueductal compression and dilatation of the third and fourth ventricles leading to hydrocephalus. The child underwent endoscopic fenestration of the cyst into the lateral ventricle and also had an external-ventricular drain (EVD) inserted. Results of an intraoperative biopsy revealed a suprasellar craniopharyngioma (WHO grade 1). Three weeks later the EVD was replaced with a left-frontal VP shunt without incident. However, at 10-week follow-up in clinic, the patient was presented with abdominal distension and was admitted as an emergency. Due to CSF not being absorbed by the peritoneum, it was decided that the distal end of the

Fig. 1. Shunt radiographic series showing the course of a ventriculocholecystic shunt in the first patient.

Fig. 2. Abdominal, lateral and frontal skull radiographic views of the ventriculocholecystic shunt in the second patient.

shunt would be re-sited to the gallbladder, as the child had congenital cardiac disease and a VA shunt was not felt to be ideal (Fig. 2). Current follow-up at 28 months reveals the child to be having no complications with the revised shunt.

Discussion Benefits and indications of VC shunts In 1959, Smith et al. were the first to describe shunts draining to the biliary tree.5 Their arguments for proposing the cholecystic system as a CSF receptacle included the ability to adequately control intracranial pressure, the low risk of infection due to the sterility of bile and also the opportunity for electrolyte reabsorption from the small intestine.5 In addition, it would be pertinent to note that the lytic action of bile breaks down proteins in CSF that prevent absorption and that the gallbladder itself is not essential to patient survival. In a study describing the placement of VC shunts in 25 children, West and colleagues outlined several indications for VC shunting. These included shunt infection; massive ascites following shunt insertion and intra-abdominal pathology causing non-absorption of CSF.6 Other indications described in the literature include multiple shunt revisions,6,7 abdominal pseudocysts8,9 and ascites secondary to shunts in optic chiasmal hypothalamic astrocytomas.9 Consideration to a VC shunt can also be given when there are reasons to avoid the peritoneum and venous systems, such as the presence of a Hickman line or a Portacatheter, and the absence of central veins on venography. A recent review by Girotti and colleagues4 summarised 59 cases of VC shunts obtained from separate studies.2,5,10,11 They found significant inter-study variability in the rates of long-term successful outcomes for paediatric VC shunts, ranging from 25% to 100%. However, on average, approximately 63% of patients across these studies were not infected or obstructed. The authors stated that this is, in fact, comparable with VP shunt complication rates.4 A group

Ventriculocholecystic shunt 3 in Florida has had similar success rates with VC shunts in paediatric patients, with 61% of patients having no shunt complications.1

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Complications of VC shunts Though the results in support of VC shunts are encouraging, the difficulties with this approach should be considered. Surfield and Klein reported the re-siting of a VP shunt in a 7-year-old boy to the gallbladder, due to a distal abscess.12 Eleven years after insertion, the patient was presented with postprandial right upper quadrant pain associated with nausea and vomiting. He was found to have gallstones encrusted on the shunt tubing, giving him symptomatic cholelithiasis. A similar report by Fountas et al. reported a bile calculus causing a distal obstruction in the VC shunt catheter.13 The authors went as far as suggesting that a VC shunt could be part of the bile calculus formation mechanism. Barami et al. reported the case of a 22-year-old patient who had multiple shunt insertions and failures (atrium, pleura and peritoneum). A low-pressure lumbar-gallbladder shunt was subsequently placed in this patient with a one-way valve. Postoperatively, the patient presented with disabling headaches and seizures. Her lumbar puncture returned bile-stained. After shunt externalisation and stabilisation of the patient, the lumbar shunt was reconnected. The patient presented 2 months later with increasing back pain and paraesthesia in her legs. The MRI showed dense arachnoiditis and obliteration of the CSF space. Although the one-way valve placed between the lumbar space and the gallbladder displayed no signs of bile reflux, the authors proposed that transient raised intra-abdominal pressure from coughing postoperatively may have caused the valve to fail, hence the bile leakage.14 In West’s study, two patients with VC shunts had complications of gallbladder atony. This, however, was successfully treated with cholecystokinin and the atony was concluded to be narcotic induced.5,15 Cholecystitis causing retrograde16 and descending17 VP shunt infections have also been reported. It would not be unreasonable to consider cholecystitis as an even more significant potential complication in VC shunting. Finally, in possibly the most serious complication seen with the VC shunt, Bernstein and Hsueh reported a 5-year-old girl with a VC shunt dying due to acute pulmonary oedema, suggesting that reflux of bile caused an aseptic meningitis and irritation of the brain stem, which in turn led to the onset of pulmonary oedema.18 It is generally accepted that contraindications to VC shunts include previous cholecystectomy, cholelithiasis, and haematological conditions with a predisposition to gall stone formation such as sickle cell disease and hereditary spherocytosis.

the preferred method to treat hydrocephalus, successes with VA shunts date back to 1952 with studies now showing better outcomes as the techniques improve.15,19 When the outcomes of VP and VA shunts are compared, some diversity has been noted in the literature.7,20,21 A comprehensive study by Borgbjerg et al. compared the durability of VA and VP shunts and concluded that there was not a statistically significant difference in shunt revision rates between the two types. However, it is notable that VA shunts are associated with more serious complications and mortality rates in comparison with VP shunts.22 Ventriculopleural shunts are used reluctantly due to the significant risks of pneumothorax and symptomatic CSF effusions. These risks, however, have been reduced with the use of anti-siphon devices in the pleura to prevent such respiratory complications.23 As with all shunts, in order to achieve an optimal outcome, detailed preoperative assessment, technical proficiency and close postoperative follow-up and management are essential. In our cases, we elected not to use a VA shunt because, in the first case, the fibrinogen deficiency would cause the catheter in the atrium to block, and in the second case, the child had congenital cardiac disease and a VA shunt was not felt to be ideal.

Conclusion The use of VC shunts can be a viable alternative when neither VP shunting nor VA shunting is possible. The reluctance to use VC shunts is mainly due to concerns with potential complications as well as with technical considerations. Reports on long-term successful outcomes with VC shunts are encouraging. We report two cases of VC shunts placed in children with a non-absorbing peritoneum. One of these is an extremely rare case of plasminogen deficiency, with impaired peritoneal CSF absorption, having previously had eight shunt revisions, and one of the first ever treated with a VC shunt. Current follow-up of 46 and –28 months has revealed no complications. Our experience, as well as that of others,24 illustrates that the operation is a technically simple one to perform. We feel that as VC shunts become more widely used, surgeons will be more comfortable with the simplicity of the procedure. Furthermore, if future results support the current VC shunt outcome data, surgical teams may consider this option earlier and/or more frequently especially in complex cases where there are good reasons to avoid the peritoneum and venous systems. Declaration of interest: The authors report no declarations of interest. The authors alone are responsible for the content and writing of the paper.

VP shunt alternatives In the cases of recurrent VP shunt malfunction, different sites for CSF drainage have been described. These include the cardiac atria, lung pleura, the stomach, fallopian tubes and the saphenous vein.4,10 Until now, the most common alternatives to the VP shunt have been ventriculoatrial (VA) and ventriculopleural shunts.4 Though VP shunting is now

References 1. Aldana PR, James HE, Postlethwait RA . Ventriculogallbladder shunts in pediatric patients. J Neurosurg Pediatr 2008;1:284–7. 2. Novelli PM, Reigel DH. A closer look at the ventriculo-gallbladder shunt for the treatment of hydrocephalus. Pediatr Neurosurg 1997;26:197–99.

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3. Rajaraman V, Schlenk R, Schulder M. Beware of the ventriculogallbladder shunt. Pediatr Radiol 2000;30:274–5. 4. Girotti ME, Singh RR, Rodgers BM. The ventriculo-gallbladder shunt in the treatment of refractory hydrocephalus: a review of the current literature. Am Surg 2009;75:734–7. 5. Smith GW, Moretz WH, Pritchard WL. Ventriculo-biliary shunt; a new treatment for hydrocephalus. Surg Forum 1958;9:701–5. 6. West KW, Turner MK , Vane DW, et al. Ventricular gallbladder shunts: an alternative procedure in hydrocephalus. J Pediatr Surg 1987;22:609–12. 7. Keucher TR, Mealey J Jr. Long-term results after ventriculoatrial and ventriculoperitoneal shunting for infantile hydrocephalus. J Neurosurg 1979;50:179–86. 8. Mobley LW III, Doran SE, Hellbusch LC. Abdominal pseudocyst: predisposing factors and treatment algorithm. Pediatr Neurosurg 2005;41:77–83. 9. Pal K, Jindal V. Ventriculo cholecystic shunt in the management of hydrocephalus. Indian Pediatr 2007;44:435–7. 10. Ketoff JA , Klein RL, Maukkassa KF. Ventricular cholecystic shunts in children. J Pediatr Surg 1997;32:181–3. 11. Olavarria G, Reitman AJ, Goldman S, Tomita T. Post-shunt ascites in infants with optic chiasmal hypothalamic astrocytoma: role of ventricular gallbladder shunt. Childs Nerv Syst 2005;21:382–4. 12. Surfield GA , Klein RL. Case report of symptomatic cholelithiasis after ventricular cholecystic shunt. J Pediatr Surg 2006;41:1933–4. 13. Fountas KN, Kassam MA , Grigorian AA . A rare, delayed complication of a ventriculogallbladder shunt Case report and review of the literature. Neurosurg Focus 2007;22: E12. 14. Barami K, Sood S, Ham S, Canady A . Chemical meningitis from bile reflux in a lumbar-gallbladder shunt. Pediatr Neurosurg 1998;29:328–30.

15. Zhang J, Qu C , Wang Z, et al. Improved ventriculoatrial shunt for cerebrospinal fluid diversion after multiple ventriculoperitoneal shunt failures. Surg Neurol 2009;72: S29–33; discussion S33–34. 16. Chang CW, Nagashima G, Fujimoto T, et al. Conspicuous endoscopic appearance of ventriculitis caused by coagulasenegative staphylococci. J Infect Chemother 2007;13:177–9. 17. Martínez-Lage JF, Girón Vallejo O, López López-Guerrero A , et al. Acute cholecystitis complicating ventriculo-peritoneal shunting: report of a case and review of the literature. Childs Nerv Syst 2008;24:777–9. 18. Bernstein RA , Hsueh W. Ventriculocholecystic shunt A mortality report. Surg Neurol 1985;23:31–7. 19. Nulsen FE, Spitz EB. Treatment of hydrocephalus by direct shunt from ventricle to jugular vein. Surg Forum 1951:399–403. 20. Surov A , Koman G, Behrmann C, Strauss C, Kornhuber M. A rare cause of ventriculoatrial shunt malfunction. Clin Neurol Neurosurg 2009;111:310–11. 21. Vernet O, Campiche R, de Tribolet N. Long-term results after ventriculoatrial shunting in children. Childs Nerv Syst 1993;9: 253–5. 22. Borgbjerg BM, Gjerris F, Albeck MJ, Hauerberg J, Børgesen SV. A comparison between ventriculo-peritoneal and ventriculoatrial cerebrospinal fluid shunts in relation to rate of revision and durability. Acta Neurochir (Wien) 1998;140:459–64; discussion 465. 23. Martínez-Lage JF, Torres J, Campillo H, et al. Ventriculopleural shunting with new technology valves. Childs Nerv Syst 2000;16: 867–71. 24. Stringel G, Turner M, Crase T. Ventriculo-gallbladder shunts in children. Childs Nerv Syst 1993;9:331–3.