Introduction

The peritoneum remains the first receptacle choice for CSF diversion. However, in some instances, the VPS is rendered infeasible due to local complications. In these cases, the VGS, which is usually used as a last resort, becomes a viable option. Herein, we performed a VGS, on a 6-month-old girl, for progressive hydrocephalus secondary to neonatal intraventricular hemorrhage (IVH) as a first therapeutic option. Her peritoneum was found to be prone to VPS failure.

Case presentation

We present the case of a 6-month-old girl with a history of preterm birth and neonatal intensive care unit (NICU) stay for respiratory distress, grade III IVH and necrotizing enterocolitis (NEC) handled conservatively. Furthermore, both her jugular veins were compromised by the implementation of Broviac lines. At follow-up, we noticed a progressive increase in head circumference. Computed tomography (CT) scan (Fig. 1) demonstrated significant communicating hydrocephalus indicating CSF diversion.

Fig. 1
figure 1

Preoperative cranial CT scan showing communicating hydrocephalus

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On admission, the patient was afebrile, alert with good activity. Physical examination findings were normal besides macro crania and wide tense anterior fontanel. However, her mother consistently mentioned episodic abdominal pain and mild fever. Complete blood count, C-reactive protein, and procalcitonin were normal. Stool test and urinalysis did not identify any potential pathogens. Abdominal CT scan and ultrasound (Fig. 2) unveiled thickening of the appendicular wall and mesenteric infiltration. Although rare, chronic appendicitis was the most likely diagnosis.

Fig. 2
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Abdominal ultrasound demonstrating a 15*20 mm hypoechoic mass in the right lower quadrant (green discontinued line), suggesting appendicitis

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Considering that peritoneum might be hostile to VPS and her neonatal complications, we had few options for CSF. Since we had to open the abdomen for surgical exploration, the gallbladder seemed a simple accessible option for distal end placement. Surgery was planned with a pediatric surgeon. A coelioscopic first look showed extensive intraperitoneal adhesions; therefore, we converted to a median supra-umbilical laparotomy. After adhesiolysis and mobilizing the caecum, the appendix appeared to be inflammatory with a cluster of mesenteric lymph nodes. An appendectomy was then performed. The pediatric surgeon presented the dome of the gallbladder into surgical view. The distal catheter was then embedded into the fundus throw a 5-mm incision and fixed with a purse-string suture around it. A 30-cm-loop of the distal catheter was left free in the abdominal cavity to fit with future growth. The infant was discharged home in stable condition 7 days after surgery. Histological findings confirmed the diagnosis of chronic appendicitis.

The current 60 months post-operatively follow-up finds normal neurological development and complete relief of abdominal symptoms. Moreover, control X-ray and sonographies (Fig. 3) were obtained, and no migration of the catheter’s tip has been noted.

Fig. 3
figure 3

Post-operative imaging X-ray A and ultrasound B showing the tip of the distal catheter (yellow arrow) embedded into the gallbladder (green asterisk) and the intraperitoneal catheter (blue arrow)

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Discussion

Management of hydrocephalus, when the peritoneum is possibly hostile to CSF shunting, can be challenging. The gallbladder proved to be an effective and safe alternative and has been increasingly popular since first described by Smith et al. (1958) [1].

In previous studies, VGS has always been considered for cases in which VPS or ventriculo arterial shunt (VAS) are no longer feasible as a second or third-line salvage treatment [2,3,4,5,6].

To our knowledge, there are only five pediatric cases reporting VGS as a de novo procedure (Table 1). West et al. [6] described a patient with short bowel syndrome related to NEC, where VAS and VPS were not possible. Pal and Jindal [4] reported a similar case to ours with good clinical outcomes and revision-free until 3 years postoperatively. Aldana et al. [2] reported a case of an infant with no previous shunt but with multiple prior abdominal surgeries. Pancucci et al. [5] recorded a case of a 4-month infant presented with obstructive hydrocephalus caused by optic-chiasmal hypothalamic glioma. They stated that a high protein CSF rate predicts VPS failure since it may be responsible for ascites and non-resorptive peritoneum. Our child developed a progressive hydrocephalus and abdominal signs related to chronic appendicitis. Considering peritoneum hostility, poor venous access, and history of neonatal respiratory distress, we opted for VGS as a first-line and definitive treatment.

Table 1 VGS as a first-line treatment in pediatric hydrocephalus
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We suggest classifying the predictor factors of VPS failure into two groups: CSF-related disorders and abdominal-related conditions. Indeed, CSF with a high protein concentration, specifically caused by optic-chiasmal hypothalamic glioma [5, 7,8,9], craniopharyngioma [10], plasminogene deficiency [3], and tuberculosis [5, 11], may be subject for proteinaceous ascites and therefore VPS failure. In these cases, the gallbladder is an efficient receptacle: CSF will be excreted and then absorbed in the intestinal tract. Nevertheless, the lytic action of bile may catabolize fibrinous adhesion around the distal shunt tip CSF [1, 5, 8, 11].

Abdominal-related conditions include multiple prior abdominal surgeries, peritoneal adhesions, ascites, pseudo-occlusion, and peritonitis [5, 12, 13]. Commonly, in these cases, surgeons use the gallbladder to drain CSF as last resort, electing the atrium or the pleural cavity for the second viable receptacle. This disinclination to VGS is not counselled by standard guidelines, but mainly as a consequence of potential complications (Table 2) and technical concerns [9, 11,12,13,14,15,16,17,18,19,20,21, 24,25,−26]. From our perspective, the VGS placement technique, as detailed by Morosanu et al. [19], was a little different compared to VPS. It could be planned with pediatric surgery teams. Furthermore, laparoscopic access makes this approach even more valuable [5, 10]. Neonatal history of NEC may be recognized as another abdominal-related cause predicting VPS failure by leading to an inflammatory peritoneal disease and a higher incidence of distal shunt obstructions [22].

Table 2 Potential complications specific to VGS system
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Reisner et al. [23] suggest that prior to deciding on VGB, an extensive evaluation should be conducted including a liver function test, ultrasound imaging of the gallbladder, or preferably a CT scan of the abdomen. Bile culture and CSF volume assessment is not mandatory. When a CSF external drainage is previously placed, a surgeon should be aware that a high CSF flow may overwhelm the gallbladder [17]. Up to date, our child remained complication-free. If a revision is ever needed, a surgeon must be aware that retrieval of the distal end should be under visual control in order to prevent bile leakage.

Conclusion

VGS placement requires a conventional preoperative assessment and rigorous surgical technique. There have been five documented pediatric cases that have undergone VGS as de novo shunt system. Our case adds to the existing literature a detailed illustration and long outcome data. If future reports support the current results, surgical teams may legitimately resort to VGS earlier in selected cases.