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Child's Nervous System

, Volume 26, Issue 9, pp 1243–1245 | Cite as

Abdominal cerebrospinal fluid (CSF) pseudocyst presented with inferior vena caval obstruction and hydronephrosis

  • Gilberto Ka Kit Leung
Case Report

Abstract

Purpose

Abdominal cerebrospinal fluid (CSF) pseudocyst is an uncommon complication of ventriculo-peritoneal shunt placement. A large pseudocyst may exert a significant pressure effect, but vascular or urological symptoms are extremely rare. We report an unusual case of a CSF pseudocyt causing inferior vena caval and ureteric obstruction.

Case

A 14-year-old girl had previously undergone ventriculo-peritoneal shunting for congenital hydrocephalus. She developed bilateral ankle edema as the only presenting symptom of a large non-infected cerebrospinal CSF pseudocyst. The associated abdominal distension was initially attributed to obesity, and the patient was unable to communicate due to developmental delay. Imaging studies showed that the pseudocyst was causing inferior vena caval obstruction and bilateral hydronephrosis. The ankle edema and hydronephrosis resolved after aspiration of the pseudocyst and shunt revision.

Conclusion

Children who require CSF shunting may suffer from associated developmental delay and are frequently unable to communicate their symptomatic complaints. This case illustrated the importance of a heightened clinical suspicion in managing these patients in whom shunt failure may present with subtle and obscure signs of lower body venous congestion.

Keywords

Ventriculo-peritoneal shunt Cerebrospinal fluid Pseudocyst Hydronephrosis Complication Inferior vena cava 

Introduction

Abdominal cerebrospinal fluid (CSF) pseudocyst is a known complication of ventriculo-peritoneal (VP) shunting. The overall incidence is less than 1% of shunted patients, and the condition accounts for just under 10% of abdominal complications of shunt placement [2]. Etiologically, an inflammatory process is a frequent predisposing factor,with 40% to 60% of cases being associated with prior infections [4]. The most common presentations are local abdominal signs in adults and symptoms of elevated intracranial pressure and abdominal pain in children [9]. Large pseudocysts tend to be non-infected and may present as giant abdominal masses with compressive symptoms [7]. Upper urinary tract obstruction is an extremely rare complication of CSF pseudocyst [12]. We present an uncommon case of a non-infected pseudocyst causing inferior vena caval and bilateral ureteric obstructions in an obese child, whose only presenting symptom was bilateral ankle edema.

Case report

A 14-year-old obese girl presented with bilateral ankle edema which was noticed by her caregiver 3 days prior. A VP shunt was inserted for congenital hydrocephalus during infancy. She also suffered from hypoxic brain injury at birth and was unable to communicate. The VP shunt had been complicated by repeated mechanical blockages and was revised thrice. The last revision was performed 1 month prior to this presentation. None of the shunt failure was associated with infection. The patient lived at a nursing home and did not indicate any discomfort or symptom.

On examination, the patient was afebrile and alert. There was pitting ankle edema bilaterally up to the mid-shin. The VP shunt was clinically patent and there was no sign of meningitis or raised intracranial pressure. Urine output was good. She had an obese abdomen which on careful examination was found to be tense but without sign of peritonitis. There was shifting dullness. On further questioning, her caregiver had initially attributed her progressive abdominal distension to increasing obesity.

A contrasted computerized tomography (CT) of the abdomen revealed an intra-abdominal cystic mass adjacent to the peritoneal catheter of the VP shunt. The mass was compressing on both ureters, resulting in hydronephrosis (Fig. 1). The inferior vena cava was also compressed (Fig. 2). CT brain showed normal-sized ventricles.
Fig. 1

Contrasted abdominal CT scan showing the pseudocyst and bilateral hydronephrosis (black arrows). The ureters could not be identified

Fig. 2

Contrasted abdominal CT scan showing the pseudocyst and the associated peritoneal shunt catheter. The inferior vena cava was grossly compressed (white arrow)

A diagnosis of CSF abdominal pseudocyst was made, and the peritoneal shunt catheter was externalized. A copious amount of yellowish fluid was aspirated from the abdominal collection. This and the CSF obtained from the proximal catheter were found to have normal biochemisty, and a total cell count of 4 × 106/l. Microbiological cultures were negative for aerobes after 4 days of incubation. No anaerobic culture was performed. Her renal functions were normal. The externalized shunt was later converted to a ventriculo-atrial shunt. The patient’s ankle edema gradually resolved over the course of 2 weeks. A subsequent abdominal CT scan showed resolution of the pseudocyst, hydronephrosis, and vena caval obstruction (Fig. 3).
Fig. 3

Non-contrasted abdominal CT scan performed after shunt revision, showing resolution of the hydronephrosis and inferior vena caval obstruction (white arrows)

Discussion

Urological complications of CSF shunt replacement are extremely rare. Lumbo-ureteral shunt, a historical treatment option for hydrocephalus, was known to result in ureteric obstruction [5]. Both lumbo-peritoneal shunt and the peritoneal catheter of a VP shunt have also been reported to cause hydronephrosis by direct extrinsic compression [3, 11]. Abdominal CSF pseudocyst may develop to a large size and result in upper urinary tract obstruction. Viets et al. described a case of a pseudocyst causing mechanical kinking of a pre-existing ureteral-conduit anastomosis [12]. Piercy et al. also reported a child with myelomeningocele in whom a retrocaval ureter was obstructed by a large pseudocyst [8]. The child in the present case was not known to have any urological anomaly. It was likely that her bilateral ureteric obstructions were the results of direct pressure effect from the pseudocyst which was severe enough to have compressed also the inferior vena cava, causing venous congestion and bilateral ankle edema. To the best of our knowledge, this is the first reported case of an abdominal CSF pseudocyst with these presentations.

CSF shunt failure in children may present with a variety of subtle and obscure symptoms. Some of these children who require shunt placement may also suffer from severe developmental delay and are therefore unable to communicate their bodily complaints. The present case illustrated the importance of exercising a heightened clinical suspicion and the awareness of uncommon clinical presentations of CSF shunt failure in a non-communicable child. The abdominal distension in our patient was initially attributed to obesity by her nursing home caregiver. In an obese child, abdominal distension may indeed be overlooked, and imaging investigations may be indicated when there are signs of lower limb venous congestion, urological obstruction, or renal function impairment.

While a number of cases are sterile and tend to develop ‘silently’ into large abdominal masses without any clinical features of infections or shunt blockage, the majority of CSF pseudocysts are associated with shunt infections [2]. In the present case, CSF culture was reportedly negative but, in retrospect, we had not firmly excluded an infection by Propionibacterium acnes, the demonstration of which would have required anaerobic culture and more prolonged incubation. P. acnes is commonly associated with late-onset shunt infections after repeated surgical procedures and may account for the abdominal pseudocyst formation in our patient [1].

Although some cases may resolve spontaneously without surgical intervention, a large lesion as seen in the present case would require treatment [13]. Management options for abdominal CSF pseudocysts include various types of shunt revisions with or without aspiration or excision of the pseudocyst or the use of temporizing external ventricular drainage [10]. More recently, laparoscopic fenestration of the cyst with repositioning of the peritoneal catheter without shunt replacement has been advocated [6]. This could have been used as an alternative treatment for our patient.

In conclusion, a non-infected abdominal CSF pseudocyst may develop into a large size with the potential of causing inferior vena caval and upper urinary tract obstruction. A heightened clinical suspicion should be exercised for shunted patients who are unable to communicate and who presented with signs of lower body venous congestion.

Notes

Acknowledgement

None

Declaration of interest

None

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Copyright information

© Springer-Verlag 2010

Authors and Affiliations

  1. 1.Division of Neurosurgery, Department of Surgery, The University of Hong KongQueen Mary HospitalHong KongChina

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