Ventriculoperitoneal shunt with a rare twist: small-bowel ischemia and necrosis secondary to knotting of peritoneal catheter

Case report

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Small-bowel ischemia and necrosis due to knotting of the peritoneal catheter is an extremely rare complication related to a ventriculoperitoneal shunt (VPS). A 3-month-old girl, with a history of Chiari II malformation and myelomeningocele (MM) after undergoing right occipital VPS insertion and MM repair at birth, presented to the emergency department with a high-grade fever. Examination of a CSF sample obtained via shunt tap raised suspicion for the presence of infection. Antibiotic therapy was initiated, and subsequently the VPS was removed and an external ventricular drain was placed. Intraoperatively, as attempts at pulling the distal catheter from the scalp incision were met with resistance, the distal catheter was cut and left in the abdomen while the remainder of the shunt system was successfully removed. While the patient was awaiting definitive shunt revision surgery to replace the VPS, she developed abdominal distension due to small-bowel obstruction. An emergency exploratory laparotomy revealed a knot in the distal catheter looping around and strangulating the distal ileum, causing small-bowel ischemia and necrosis in addition to the obstruction. A small-bowel resection with ileostomy was performed, with subsequent placement of ventriculoatrial shunt for treatment of hydrocephalus. The authors report this exceedingly rare clinical scenario to highlight the fact that any retained distal catheter must be carefully managed with immediate abdominal exploration to remove the distal catheter to avoid bowel necrosis as pulling of a knotted peritoneal catheter may strangulate the bowel and cause ischemia, with significant clinical morbidity and possible mortality.

Abbreviations used in this paper:KUB = kidney/ureter/bladder; MM = myelomeningocele; VPS = ventriculoperitoneal shunt.

Small-bowel ischemia and necrosis due to knotting of the peritoneal catheter is an extremely rare complication related to a ventriculoperitoneal shunt (VPS). A 3-month-old girl, with a history of Chiari II malformation and myelomeningocele (MM) after undergoing right occipital VPS insertion and MM repair at birth, presented to the emergency department with a high-grade fever. Examination of a CSF sample obtained via shunt tap raised suspicion for the presence of infection. Antibiotic therapy was initiated, and subsequently the VPS was removed and an external ventricular drain was placed. Intraoperatively, as attempts at pulling the distal catheter from the scalp incision were met with resistance, the distal catheter was cut and left in the abdomen while the remainder of the shunt system was successfully removed. While the patient was awaiting definitive shunt revision surgery to replace the VPS, she developed abdominal distension due to small-bowel obstruction. An emergency exploratory laparotomy revealed a knot in the distal catheter looping around and strangulating the distal ileum, causing small-bowel ischemia and necrosis in addition to the obstruction. A small-bowel resection with ileostomy was performed, with subsequent placement of ventriculoatrial shunt for treatment of hydrocephalus. The authors report this exceedingly rare clinical scenario to highlight the fact that any retained distal catheter must be carefully managed with immediate abdominal exploration to remove the distal catheter to avoid bowel necrosis as pulling of a knotted peritoneal catheter may strangulate the bowel and cause ischemia, with significant clinical morbidity and possible mortality.

Abbreviations used in this paper:KUB = kidney/ureter/bladder; MM = myelomeningocele; VPS = ventriculoperitoneal shunt.

Ventriculoperitoneal shunt (VPS) placement is one of the most common procedures performed by neurosurgeons throughout the world. Ever since the initial shunt system was developed in the 1950s, there have been major advances in the technology. Nevertheless, the neurosurgical community is still plagued by significant shunt-related complications such as infection, proximal or distal catheter occlusion, valve malfunction, and pseudocyst formation. A recent study from a large national database showed a 30-day complication rate of 12.9% and a 1-year complication rate of 28.8%,1 with various other historical studies reporting complication rates up to 59% at 1 year.8,12 The most common VPS complications include shunt malfunction and infection. Other unusual complications such as distal catheter migration into the heart, scrotum, or bowel have also been reported.5,7,11,19 Knotting of the distal catheter is very rare but generally present with a shunt block and subsequent malfunction, which often only require simple distal revisions.4,6,9,10,13–16,18,20–22 Small-bowel necrosis due to knotting of the peritoneal catheter is an exceedingly rare complication of a VPS, with only 1 previously described case in the literature.18 We report only the second case of bowel necrosis due to knotting of peritoneal catheter, and we provide a comprehensive review of the literature while considering the clinical relevance of this rare complication.

Case Report

History and Presentation

A 3-month-old girl with a history of Chiari II malformation and myelomeningocele (MM) after undergoing right occipital VPS placement and MM repair at birth presented to the emergency department with a high-grade fever. Examination of a CSF sample obtained via shunt tap was positive for Gram-negative rods, raising suspicion for shunt infection. Computed tomography of the head showed stable ventricle size; a shunt series was ordered but was not completed in the emergency department.

Management

The patient was started on broad-spectrum antibiotics while awaiting culture results. She was taken to the operating room immediately for VPS removal and external ventricular drain placement. Intraoperatively, attempts at pulling out the peritoneal catheter were met with great resistance. At the time, it was thought that the peritoneal catheter was trapped due intraabdominal adhesions; thus, the peritoneal catheter was cut with the distal part retained in the peritoneal cavity. The rest of the shunt system was removed, and the external ventricular drain was placed. Postoperative abdominal radiography showed knotting of the retained peritoneal catheter in the right lower quadrant (Fig. 1). Because the final CSF cultures were negative and the patient was doing well initially after surgery, the retained abdominal catheter was left in place; the plan was to remove it when a new VPS was being inserted after the child had medically stabilized. However, the patient developed clinical and radiographic signs of small-bowel obstruction over the next few days (Fig. 2). She was taken to the operating room for exploratory laparotomy, which revealed knotting of the peritoneal catheter around distal ileum that was causing bowel ischemia and necrosis. Resection of the necrotic bowel and ileostomy were performed.

Fig. 1.
Fig. 1.

Postoperative KUB (kidney/ureter/bladder) radiograph obtained after initial shunt removal demonstrating knotting of the retained distal catheter and knot.

Fig. 2.
Fig. 2.

Upper gastrointestinal tract series demonstrating failure of contrast pressing into large intestines consistent with bowel obstruction.

Postoperative Course

The patient was in stable condition, with improved clinical status after surgery. Postoperative abdominal radiography showed complete resolution of bowel obstruction and distention (Fig. 3). A ventriculoatrial shunt was subsequently placed and the patient was eventually discharged to home in stable condition.

Fig. 3.
Fig. 3.

Postoperative abdominal KUB radiograph acquired after resection of necrotic bowel and removal of knotted peritoneal catheter, demonstrating resolution of bowel distension.

Discussion

There are about 36,000 shunt-related procedures performed in the United States each year.1 Spontaneous formation of a knot of the distal VPS catheter in the abdomen is an unusual complication; only 12 cases have been reported in the literature to date.4,6,9,10,13–16,18,20–22 Bowel ischemia and necrosis due to strangulation of intestines by the knotted peritoneal catheter can occur, and this complication is exceedingly rare, with only 1 case previously reported in the literature.18 The case reported here represents only the second occurrence of bowel necrosis caused by a knotted distal shunt catheter. Previously reported cases and associated complications are summarized in Table 1.

TABLE 1:

Summary of previously reported VPS complications due to distal catheter knotting*

Authors & YearAge, SexReason for VPSPresentationManagement
Murtagh et al., 198016 mos, Fcongenital HCPbowel obstructionlaparotomy & removal of knotted catheter
Starreveled et al., 19987 days, Fcongenital HCPbowel ischemia & necrosisbowel resection & VAS
Toshifumi et al., 200163 yrs, Mhead injury & HCPbowel obstructionlaparotomy & removal of knotted catheter
Chopra et al., 200425 yrs, Fbrain tumor & HCPshunt malfunctionremoval of knotted catheter
Woerdeman & Hanlo, 200610 days, MChiari II & MMshunt malfunctionuntangling of catheter
Eftekhar & Hunn, 20083.5 yrs, MIVH & HCPshunt malfunctionuntangling of catheter
Mohammed et al., 201114 yrs, Mcongenital HCPshunt malfunctionVPS replacement
Borcek et al., 20123 yrs, Mhead injury & HCPshunt malfunctionVPS replacement
Mohindra & Sharma, 201210 yrs, MCrouzon's syndrome & HCPincidental (skin abscess)observation
Lo et al., 201310 yrs, Fcongenital HCPshunt malfunction & disconnectionVPS replacement
Fekete et al., 20132 mos, NRIVH & HCPshunt malfunctionremoval of knotted catheter
Ul-Haq et al., 20138 yrs, Mcongenital HCPshunt malfunctionVPS replacement
present case3 mos, FChiari II & MMbowel ischemia & necrosisbowel resection & VAS

HCP = hydrocephalus; IVH = intraventricular hemorrhage; NR = not reported; VAS = ventriculoatrial shunt.

Cases associated with bowel ischemia and necrosis.

The extremely low incidence of such a complication makes it a difficult phenomenon to apprehend, and the exact mechanism of spontaneous knotting of the peritoneal catheter remains a matter of speculation. Some authors have hypothesized that longer catheter length, smaller diameter, greater elasticity, and a crowded intraabdominal space are potential risk factors for spontaneous formation of a knot in the peritoneal catheter.9,15 Others have suggested that vigorous intestinal peristalsis could also contribute to spontaneous knotting.22 Raymer and Smith performed an interesting physical experiment by placing a piece of string in a rotating cubic box, and they found that the probability of spontaneous knot formation increased with longer string length, increased flexibility, increased total number of rotations, slower rotational speed, and larger cube volume.17 Although this physical experiment does not totally simulate the physiological milieu of shunt catheter in the peritoneal cavity, it does provide some insight into the plausible mechanism of spontaneous knot formation. Perhaps a more physiologically comparable phenomenon is spontaneous knotting of the umbilical cord during pregnancy, which has a reported incidence of 1.22%.3 Bakas et al. reviewed several large retrospective studies and concluded that long umbilical cords, male embryos, and multiparity (larger uterine volume) were correlated with umbilical cord knot formation.2 The physical experiment and physiological phenomenon described above both seem to suggest that excessively long catheter length in the abdomen probably plays an important role in knot formation. Interestingly, 11 of the 13 known cases (including the present case) of peritoneal catheter knotting occurred in the pediatric patients, in whom a longer relative length of catheter in the peritoneal cavity might be present due to their small physical size. It is also interesting to note that 1 of the 2 adult patients was a 25-year-old pregnant woman, highlighting the fact that a crowded peritoneal space and increased peristalsis may also contribute to knot formation.22 Though not proven, consideration may be given to shortening the length of the distal catheter in very young infants to reduce this complication.

A majority (8 of 13) of patients with peritoneal catheter knots presented with shunt malfunction along with one case in which the knot was an incidental finding (Table 1). The remaining 4 cases had bowel obstruction as part of the symptoms, and bowel necrosis only occurred in 2 patients (including the present case). Both cases of bowel ischemia and necrosis occurred after initial unsuccessful attempts to remove the peritoneal catheters, and retrieval of the retained catheters was done in a delayed fashion. It is likely that the excessive length of distal catheter was looping around a segment of bowel, and pulling of distal catheter during attempted removal actually tightened the loop, resulting in knot formation and strangulation of the bowel. Delayed recognition of the problem and lack of prompt removal of the retained catheter eventually led to bowel necrosis. Therefore, it is prudent to carefully study preoperative shunt series to check for any existing loop or knot formed by the peritoneal catheter before shunt revision procedures. If resistance is met during distal catheter removal, one should refrain from using excessive pulling force, since pulling may tighten an existing knot/loop and strangulate the bowel. Prompt general surgery consultation should be obtained in such circumstances to remove the distal catheter to prevent more serious complications such as bowel necrosis. In the setting of a suspected infection, prompt removal of retained catheter is even more important for eradicating a potential nidus and facilitating the clearance of infection.

Conclusions

Bowel ischemia and necrosis secondary to peritoneal catheter knotting is a rare but serious potential complication that all neurosurgeons should bear in mind. Any retained distal catheter must be carefully investigated and an intraoperative general surgery consult should be strongly considered. Excessive pulling of a looped/knotted peritoneal catheter may strangulate the bowel. The value of a shunt series should not be underestimated as it can provide a clue to the presence of such a complication. Immediate general surgery consultation should be obtained when difficult distal catheter extraction is encountered.

Disclosure

Dr. Moftakhar is a consultant for Covidien and Penumbra.

Author contributions to the study and manuscript preparation include the following. Conception and design: Kasliwal, Tan. Acquisition of data: Tan. Analysis and interpretation of data: Kasliwal, Tan. Drafting the article: Kasliwal, Tan. Critically revising the article: Kasliwal, Moftakhar, Munoz. Reviewed submitted version of manuscript: Kasliwal, Tan, Munoz. Approved the final version of the manuscript on behalf of all authors: Kasliwal. Study supervision: Moftakhar, Munoz.

References

  • 1

    Al-Tamimi YZSinha PChumas PDCrimmins DDrake JKestle J: Ventriculoperitoneal shunt 30-day failure rate: a retrospective international cohort study. Neurosurgery 74:29342014

    • Search Google Scholar
    • Export Citation
  • 2

    Bakas PPapadakis EHassiakos DLiapis A: Second-trimester miscarriage and umbilical cord knot. Case report and review of the literature. Clin Exp Obstet Gynecol 40:4484512013

    • Search Google Scholar
    • Export Citation
  • 3

    Blickstein IShoham-Schwartz ZLancet M: Predisposing factors in the formation of true knots of the umbilical cord—analysis of morphometric and perinatal data. Int J Gynaecol Obstet 25:3953981987

    • Search Google Scholar
    • Export Citation
  • 4

    Borcek AOCivi SGolen MEmmez HBaykaner MK: An unusual ventriculoperitoneal shunt complication: spontaneous knot formation. Turk Neurosurg 22:2612642012

    • Search Google Scholar
    • Export Citation
  • 5

    Chong JYKim JMCho DCKim CH: Upward migration of distal ventriculoperitoneal shunt catheter into the heart: case report. J Korean Neurosurg Soc 44:1701732008

    • Search Google Scholar
    • Export Citation
  • 6

    Chopra IGnanalingham KPal DPeterson D: A knot in the catheter—an unusual cause of ventriculo-peritoneal shunt blockage. Acta Neurochir (Wien) 146:105510572004

    • Search Google Scholar
    • Export Citation
  • 7

    Chung JJYu JSKim JHNam SJKim MJ: Intraabdominal complications secondary to ventriculoperitoneal shunts: CT findings and review of the literature. AJR Am J Roentgenol 193:131113172009

    • Search Google Scholar
    • Export Citation
  • 8

    Cochrane DDKestle J: Ventricular shunting for hydrocephalus in children: patients, procedures, surgeons and institutions in English Canada, 1989–2001. Eur J Pediatr Surg 12:Suppl 1S6S112002

    • Search Google Scholar
    • Export Citation
  • 9

    Eftekhar BHunn A: Ventriculoperitoneal shunt blockage due to spontaneous knot formation in the peritoneal catheter. Case report. J Neurosurg Pediatr 1:1421432008

    • Search Google Scholar
    • Export Citation
  • 10

    Fekete GNagy APataki IBognar LNovák L: Shunt insufficiency due to knot formation in the peritoneal catheter. Ideggyogy Sz 66:2772792013

    • Search Google Scholar
    • Export Citation
  • 11

    Fewel MEGarton HJL: Migration of distal ventriculoperitoneal shunt catheter into the heart. Case report and review of the literature. J Neurosurg 100:2 Suppl Pediatrics2062112004

    • Search Google Scholar
    • Export Citation
  • 12

    Kestle JDrake JMilner RSainte-Rose CCinalli GBoop F: Long-term follow-up data from the Shunt Design Trial. Pediatr Neurosurg 33:2302362000

    • Search Google Scholar
    • Export Citation
  • 13

    Lo WBRamirez RRodrigues DSolanki GA: Ventriculoperitoneal shunt disconnection associated with spontaneous knot formation in the peritoneal catheter. BMJ Case Reports 2013. (http://casereports.bmj.com/content/2013/bcr-2013-009590.long) [Accessed June 8 2014]

    • Search Google Scholar
    • Export Citation
  • 14

    Mohammed WWiig UCaird J: Spontaneous knot; a rare cause of ventriculoperitoneal shunt blockage. Br J Neurosurg 25:1131142011

  • 15

    Mohindra SSharma M: Spontaneous knotting of peritoneal catheter: a report of an asymptomatic patient. J Pediatr Neurosci 7:1511532012

    • Search Google Scholar
    • Export Citation
  • 16

    Murtagh FRQuencer RMPoole CA: Extracranial complications of cerebrospinal fluid shunt function in childhood hydrocephalus. AJR Am J Roentgenol 135:7637661980

    • Search Google Scholar
    • Export Citation
  • 17

    Raymer DMSmith DE: Spontaneous knotting of an agitated string. Proc Natl Acad Sci U S A 104:16432164372007

  • 18

    Starreveld YPoenaru DEllis P: Ventriculoperitoneal shunt knot: a rare cause of bowel obstruction and ischemia. Can J Surg 41:2392401998

    • Search Google Scholar
    • Export Citation
  • 19

    Sturdee SWTimothy JTyagi A: Total extrusion of a cranial peritoneal shunt per rectum. J Clin Neurosci 9:1992002002

  • 20

    Toshifumi KNobuhiro STatsuro I: [Intesitinal obstruction caused by ventriculoperitoneal shunt knot. A case report.]. J Iwate Med Assoc 53:43462001. (Jpn)

    • Search Google Scholar
    • Export Citation
  • 21

    Ul-Haq AAl-Otaibi FAlshanafey SSabbagh MDAl Shail E: Ventriculoperitoneal shunt peritoneal catheter knot formation. Case Rep Neurol Med 2013:6284932013

    • Search Google Scholar
    • Export Citation
  • 22

    Woerdeman PAHanlo PW: Ventriculoperitoneal shunt occlusion due to spontaneous intraabdominal knot formation in the catheter. Case report. J Neurosurg 105:3 Suppl2312322006

    • Search Google Scholar
    • Export Citation

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Article Information

Contributor Notes

Address correspondence to: Manish K. Kasliwal, M.D., M.Ch., Department of Neurosurgery, 1725 W. Harrison St., Ste. 855, Rush University Medical Center, Chicago, IL 60612. email: manish_kasliwal@rush.edu.Please include this information when citing this paper: published online July 4, 2014; DOI: 10.3171/2014.6.PEDS1418.

© AANS, except where prohibited by US copyright law.

Headings
Figures
  • View in gallery

    Postoperative KUB (kidney/ureter/bladder) radiograph obtained after initial shunt removal demonstrating knotting of the retained distal catheter and knot.

  • View in gallery

    Upper gastrointestinal tract series demonstrating failure of contrast pressing into large intestines consistent with bowel obstruction.

  • View in gallery

    Postoperative abdominal KUB radiograph acquired after resection of necrotic bowel and removal of knotted peritoneal catheter, demonstrating resolution of bowel distension.

References
  • 1

    Al-Tamimi YZSinha PChumas PDCrimmins DDrake JKestle J: Ventriculoperitoneal shunt 30-day failure rate: a retrospective international cohort study. Neurosurgery 74:29342014

    • Search Google Scholar
    • Export Citation
  • 2

    Bakas PPapadakis EHassiakos DLiapis A: Second-trimester miscarriage and umbilical cord knot. Case report and review of the literature. Clin Exp Obstet Gynecol 40:4484512013

    • Search Google Scholar
    • Export Citation
  • 3

    Blickstein IShoham-Schwartz ZLancet M: Predisposing factors in the formation of true knots of the umbilical cord—analysis of morphometric and perinatal data. Int J Gynaecol Obstet 25:3953981987

    • Search Google Scholar
    • Export Citation
  • 4

    Borcek AOCivi SGolen MEmmez HBaykaner MK: An unusual ventriculoperitoneal shunt complication: spontaneous knot formation. Turk Neurosurg 22:2612642012

    • Search Google Scholar
    • Export Citation
  • 5

    Chong JYKim JMCho DCKim CH: Upward migration of distal ventriculoperitoneal shunt catheter into the heart: case report. J Korean Neurosurg Soc 44:1701732008

    • Search Google Scholar
    • Export Citation
  • 6

    Chopra IGnanalingham KPal DPeterson D: A knot in the catheter—an unusual cause of ventriculo-peritoneal shunt blockage. Acta Neurochir (Wien) 146:105510572004

    • Search Google Scholar
    • Export Citation
  • 7

    Chung JJYu JSKim JHNam SJKim MJ: Intraabdominal complications secondary to ventriculoperitoneal shunts: CT findings and review of the literature. AJR Am J Roentgenol 193:131113172009

    • Search Google Scholar
    • Export Citation
  • 8

    Cochrane DDKestle J: Ventricular shunting for hydrocephalus in children: patients, procedures, surgeons and institutions in English Canada, 1989–2001. Eur J Pediatr Surg 12:Suppl 1S6S112002

    • Search Google Scholar
    • Export Citation
  • 9

    Eftekhar BHunn A: Ventriculoperitoneal shunt blockage due to spontaneous knot formation in the peritoneal catheter. Case report. J Neurosurg Pediatr 1:1421432008

    • Search Google Scholar
    • Export Citation
  • 10

    Fekete GNagy APataki IBognar LNovák L: Shunt insufficiency due to knot formation in the peritoneal catheter. Ideggyogy Sz 66:2772792013

    • Search Google Scholar
    • Export Citation
  • 11

    Fewel MEGarton HJL: Migration of distal ventriculoperitoneal shunt catheter into the heart. Case report and review of the literature. J Neurosurg 100:2 Suppl Pediatrics2062112004

    • Search Google Scholar
    • Export Citation
  • 12

    Kestle JDrake JMilner RSainte-Rose CCinalli GBoop F: Long-term follow-up data from the Shunt Design Trial. Pediatr Neurosurg 33:2302362000

    • Search Google Scholar
    • Export Citation
  • 13

    Lo WBRamirez RRodrigues DSolanki GA: Ventriculoperitoneal shunt disconnection associated with spontaneous knot formation in the peritoneal catheter. BMJ Case Reports 2013. (http://casereports.bmj.com/content/2013/bcr-2013-009590.long) [Accessed June 8 2014]

    • Search Google Scholar
    • Export Citation
  • 14

    Mohammed WWiig UCaird J: Spontaneous knot; a rare cause of ventriculoperitoneal shunt blockage. Br J Neurosurg 25:1131142011

  • 15

    Mohindra SSharma M: Spontaneous knotting of peritoneal catheter: a report of an asymptomatic patient. J Pediatr Neurosci 7:1511532012

    • Search Google Scholar
    • Export Citation
  • 16

    Murtagh FRQuencer RMPoole CA: Extracranial complications of cerebrospinal fluid shunt function in childhood hydrocephalus. AJR Am J Roentgenol 135:7637661980

    • Search Google Scholar
    • Export Citation
  • 17

    Raymer DMSmith DE: Spontaneous knotting of an agitated string. Proc Natl Acad Sci U S A 104:16432164372007

  • 18

    Starreveld YPoenaru DEllis P: Ventriculoperitoneal shunt knot: a rare cause of bowel obstruction and ischemia. Can J Surg 41:2392401998

    • Search Google Scholar
    • Export Citation
  • 19

    Sturdee SWTimothy JTyagi A: Total extrusion of a cranial peritoneal shunt per rectum. J Clin Neurosci 9:1992002002

  • 20

    Toshifumi KNobuhiro STatsuro I: [Intesitinal obstruction caused by ventriculoperitoneal shunt knot. A case report.]. J Iwate Med Assoc 53:43462001. (Jpn)

    • Search Google Scholar
    • Export Citation
  • 21

    Ul-Haq AAl-Otaibi FAlshanafey SSabbagh MDAl Shail E: Ventriculoperitoneal shunt peritoneal catheter knot formation. Case Rep Neurol Med 2013:6284932013

    • Search Google Scholar
    • Export Citation
  • 22

    Woerdeman PAHanlo PW: Ventriculoperitoneal shunt occlusion due to spontaneous intraabdominal knot formation in the catheter. Case report. J Neurosurg 105:3 Suppl2312322006

    • Search Google Scholar
    • Export Citation
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