Novel Insights from Clinical Practice Pediatr Neurosurg 2014–15;50:152–156 DOI: 10.1159/000381030

Received: October 16, 2014 Accepted after revision: February 14, 2015 Published online: April 25, 2015

Ventriculobiliary Shunts, Another Option Mónica Rivero-Garvía a Giovanni Pancucci a Juan Morcillo b Ana Millán b Javier Márquez-Rivas a a

Unit of Pediatric Neurosurgery, and b Department of Pediatric Surgery, Virgen del Rocío Hospital, Seville, Spain

Established Facts • The basic management of hydrocephalus includes shunts to the peritoneum and atrium. • There are particularly complex patients in whom it is necessary to look for atypical places for implanting the distal catheter.

Novel Insights • The third option in hydrocephalus is uncertain. • In our short experience, a ventriculo-gallbladder shunt is a good option when there is not abdominal hypertension.

Abstract The basic management of hydrocephalus includes shunts to the peritoneum and atrium. However, there are particularly complex patients in whom it is necessary to look for atypical places for implanting the distal catheter. Since 2000, 1,325 shunts have been implanted in pediatric patients. Only 3 patients required a ventriculobiliary shunt. We report 3 cases: a 7-year-old boy with a surgically treated complex heart disease, a 16-month-old girl with hydrocephalus secondary to a brain tumor and multiple bacteremias secondary to an in-

© 2015 S. Karger AG, Basel 1016–2291/15/0503–0152$39.50/0 E-Mail [email protected] www.karger.com/pne

fection of the central venous reservoir, and a 4-year-old girl with nonreabsorptive hydrocephalus caused by intraventricular bleeding due to premature birth, necrotizing enterocolitis and shunt infections with abdominal pseudocysts, which caused multiple abdominal septations and, finally, a nonreabsorptive peritoneum. At present, cases 1 [45 months after ventriculobiliary shunt (VBS)] and 3 (27 months after VBS) are symptom free, while case 2 (14 months after VBS) died of infectious respiratory complications. The gold standard for the treatment of nonreabsorptive hydrocephalus is a ventriculoperitoneal shunt, the second option is a ventriculoatrial shunt, and the third option is uncertain. In our short experience, a ventriculo-gallbladder shunt is a good option when there is no abdominal hypertension. © 2015 S. Karger AG, Basel

Mónica Rivero-Garvía Unit of Pediatric Neurosurgery Virgen del Rocío Hospital, C/ Manuel Siurot s/n ES–41013 Seville (Spain) E-Mail monicargarvia @ msn.com

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Key Words Gallbladder shunt · Nonreabsorptive peritoneum · Atypical shunt · Vascular shunt · Ventriculobiliary shunt

Color version available online

Introduction

Hydrocephalus is a common condition in the neurosurgeon’s everyday clinical practice. Basic management includes shunts to the peritoneum and atrium in the immense majority of patients. However, there are particularly complex patients in whom it is necessary to look for imaginative places for implanting the distal catheter [1– 6]. We report on our experience with ventriculobiliary shunts (VBS) in the pediatric population. a

Case Report Since 2000, 1,325 shunts have been implanted in pediatric patients. Only 3 patients required a VBS, in 4 procedures.

b

Fig. 1. a Case 1, nonreabsorptive peritoneum due to multiple abdominal surgeries. b Case 2, significant abdominal distention due

to the accumulation of free peritoneal fluid.

Case 1 A male patient, 7 years old, carrying a ventriculoperitoneal shunt (VPS) due to hydrocephalus associated with a surgically treated complex heart disease (pulmonary atresia with intact septum and right ventricle with sinusoids) that caused secondary pulmonary hypertension. Since the implantation, the patient had suffered numerous valve infections. The last two malfunctions were caused by a distal dysfunction caused by infection-related abdominal pseudocysts. In the last valve malfunction, the patient presented with a nonreabsorptive peritoneum containing a large quantity of septated fluid, with negative cerebrospinal fluid (CSF) cultures (fig. 1a). The intracranial hypertension rendered it necessary to exteriorize the shunt and look for an alternative location for the distal catheter. After ruling out vascular shunts due to the history of heart disease, the pleural shunt because of the chronic respiratory failure and the bladder shunt due to repeated urine infections, it was decided to implant a VBS. a

Case 3 A female patient, 4 years old, with nonreabsorptive hydrocephalus caused by intraventricular bleeding due to premature birth. During the first months of life, the patient underwent surgery on

Ventriculobiliary Shunts, Another Option

b

Fig. 2. Plain abdominal X-ray of case 2. a The displacement of the intestinal loops by the free fluid is shown. b VBS.

multiple occasions, first because of necrotizing enterocolitis and subsequently due to shunt infections and abdominal pseudocysts, which caused multiple abdominal septations and, finally, a nonreabsorptive peritoneum. After running out of venous accesses for the shunt (the right internal jugular vein was thrombosed by a ventriculoatrial shunt, and both deep femoral veins were compromised by ventriculofemoral shunts), it was decided to perform a third ventriculostomy and aqueductoplasty in an attempt to control the hydrocephalus. After failure of this procedure, it was decided to implant the VBS (fig. 3a–c). Six months later, the patient showed a subcutaneous accumulation of fluid in the abdomen, requiring reoperation to reconnect a disconnected biliary catheter.

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Case 2 A female patient, 16 months old, with hydrocephalus associated with a teratoid rhabdoid tumor, operated on when she was 10 months old. After initially controlling the hydrocephalus with an endoscopic ventriculostomy, prior to excising the tumor, the first VPS was implanted in the 14th month of life, due to a nonreabsorptive hydrocephalus. During chemotherapy, the patient suffered multiple bacteremias caused by Staphylococcus epidermidis and Staphylococcus aureus, associated with an infection of the central venous reservoir. Accordingly, after multiple S. epidermidis infections, when the patient presented with a valve malfunction due to a nonreabsorptive peritoneum at the age of 25 months, vascular shunts were ruled out, and it was decided to place a VBS (fig. 2a, b).

Fig. 3. Case 3, sequence of atypical shunts a

b

c

Color version available online

required by the patient: ventriculofemoral (a, b) and VBS (c).

Fig. 4. Surgical technique and sealing of the

entry point.

Discussion

The gold standard for the treatment of nonreabsorptive hydrocephalus is VPS. When the patient has special features that compromise reabsorption from the perito154

Pediatr Neurosurg 2014–15;50:152–156 DOI: 10.1159/000381030

neum (multiple abdominal surgeries, intestinal pseudoocclusion, tumor ascites etc.) or which cause abdominal hypertension (morbid obesity, adhesions after multiple surgeries that compartmentalize the abdominal cavity etc.), alternative sites to the abdominal cavity have been proposed, with the atrial shunt being the most common second choice. The ventriculoatrial shunt, although it is more physiological, is associated with a number of added problems in younger pediatric patients: normally, the open technique is used, and in neonates or infants this causes proximal thrombosis of the internal jugular vein as it has a diameter similar to the distal catheter. Furthermore, the extra length of catheter that can be implanted to reduce the need for growth-related revisions is shorter. This means that, in many patients, this second location is temporary and not reusable if there is a total Rivero-Garvía/Pancucci/Morcillo/Millán/ Márquez-Rivas

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Technique We perform a parietal trepanation to place the ventricular catheter. The gallbladder is located by intraoperative ultrasound. A subcostal incision is made above the gallbladder. When the fundus is located, a minimum incision is performed, a catheter is inserted (4–5 cm), a purse-string suture is performed around it, and it is fastened to the gallbladder wall. We seal the entry point with adhesive collagen dressing (fig. 4). The rest of the catheter is left free in the abdominal cavity, and a section of the distal tube is left above the closed abdominal wall for placing a straight connector to perform revisions, if necessary.

tal pressure of the classic VPS and the VBS would be the same). Perform an echo-Doppler of both jugular and femoral veins to check for patency. Perform an abdominal ultrasound scan to verify the status of the gallbladder (in case 3, gallbladder stones were diagnosed, and all the stones were removed during the same surgery), and, in the favorable cases, perform an endoscopic ventriculostomy, as in cases 2 and 3, with invasive intracranial monitoring, before deciding to place a ventriculofemoral shunt. In the first case, the patient had a nonreabsorptive peritoneum as a result of multiple valve infections and abdominal surgeries. The second option, a ventricular access device, was ruled out because the jugular venous return was compromised by the prior heart surgery; the pleural shunt was contraindicated because the patient had pulmonary hypertension with chronic hypoxemia due to his complex heart disease and because of the possibility that the pleural effusion of CSF could exacerbate the existing chronic respiratory failure. Shunting to dural sinuses was ruled out because of the venous hypertension, as was an attempt to perform a ventriculostomy due to the presence of a tetraventricular dilation in all the neuroimaging tests. Other locations, such as ureteral or bladder locations, were not considered due to the history of repeated urine infections. In the second case, any shunt to the venous tree was ruled out because of the repeated bacteremias caused by the manipulation of the central venous reservoir during prior chemotherapy and the need to continue the chemotherapy for another year. After the failed attempt in the initial management with endoscopic ventriculostomy, prior to excision of the tumor and the tetraventricular morphology in the imaging tests, it was decided to place a ventriculobiliary shunt. One year after surgery, the patient suffered a further valve infection requiring its removal. The new shunt placed was a standard ventriculoperitoneal shunt, with no distal complications. Fourteen months after this surgery, the patient died of an atypical pneumonia. In the third case, the history of ipsilateral jugular thrombosis, the bilateral femoral thromboses caused by previous shunting procedures, the repeated urine infections and the functional failure of the ventriculostomy and aqueductoplasty rendered it necessary to shunt to the gallbladder. At present, cases 1 (45 months after VBS) and 3 (27  months after VBS) are symptom free, while case 2 (14  months after VBS) died of infectious respiratory complications.

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thrombosis of the vein lumen, as the use of the contralateral vein may disrupt intracranial venous return, leading to intracranial hypertension due to the increased pressure in the longitudinal sinus and causing the patient’s death. If the patient shows complications arising from this second choice and the causes that prevent placing it in the abdominal cavity are not resolved, it will be necessary to try less used solutions, such as femoral, pleural, bladder, gallbladder, ureteral or dural sinus shunts [1– 7]. Femoral shunts modify the venous return from the legs and may cause thrombosis of the inferior vena cava. These shunts are the third choice in our Unit, when the more commonly used shunts have failed [3], as there is the possibility of using both femoral veins at different times. In our experience, patients were not maintained on aspirin or other platelet inhibitors like a ventriculoatrial shunt. Pleural shunts usually have an age limit in pediatric patients, as they are not recommended in patients under 4 years of age (and therefore were ruled out immediately in cases 2 and 3) and are usually temporary due to the existence of pleural septations. Shunts to the urinary system should not be performed in patients with repeated urine infections, which are very common among the hospitalized, catheterized population, and are therefore not used in our Unit. Shunts to dural sinuses need patent sinuses, without intracranial venous hypertension as an added cause of the hydrocephalus. There is also a high risk of thrombosis of the sinuses after placing the catheter, which may cause the patient’s death. Shunts to gallbladders may be a potential problem causing swings in intracranial pressure with high-fat diets or with colelitiasis. In our cases, the patients did not follow the strictest low-fat diet but general recommendations for reducing the fat in their meals. No patients presented problems for this reason. Our series consists of particularly complex patients both because of their underlying disease and because of their young age. The sequence in the decision tree was: exteriorize the shunt at the abdomen to control the intracranial hypertension caused by distal functional obstruction. Obtain CSF cultures to check for CSF sterility. Study the abdominal pressure using nasogastric tubes to rule out abdominal hypertension as a cause of distal shunt malfunction (a situation in which the ventriculobiliary shunt would be contraindicated; being an intra-abdominal shunt, the dis-

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Pal K, Jindal V: Ventriculo cholecystic shunt in the management of hydrocephalus. Indian Pediatr 2007;44:435–437. Weiner GM, Bui CJ, Steele RW: An infant with an MRSA ventriculo-gallbladder shunt infection. Clin Pediatr (Phila) 2011;50:269–271. Frim DM, Lathrop D, Chwals WJ: Intraventricular pressure dynamics in ventriculocholecystic shunting: a telemetric study. Pediatr Neurosurg 2001;34:73–76.

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Ventriculobiliary Shunts, Another Option.

The basic management of hydrocephalus includes shunts to the peritoneum and atrium. However, there are particularly complex patients in whom it is nec...
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