Bonna Benjamin, MD

The imaging studies shown are from 2 children with cancer who underwent placement of 9.6 French left subclavian central venous catheters (CVCs) to facilitate treatment. Fracture of the catheters with subsequent embolization of the distal fragment to the pulmonary arteries was noted at about 18 months after placement. Findings suggestive of impending fracture were missed in previous radiographs. In both cases, an interventional radiologist removed the fragment via percutaneous catheterization of the right femoral vein.

 is from an 8-year-old boy with acute lymphoblastic leukemia. Fluoroscopy confirmed the catheter tip to be at the junction of the superior vena cava and right atrium with no kinks. At about month 19, during an attempt to access the CVC, the patient had pain in his left shoulder. A chest radiograph revealed a fractured piece of the CVC tubing overlying the left lung in the aortopulmonary window region. The distal fragment was lodged in the left main pulmonary artery. The patient could not recall, nor did he complain of, any traumatic events or symptoms that could be attributed to the fracture. On review of previous chest films, an intermittent focal narrowing of the catheter tubing-“pinch-off sign” (POS)-was found. The catheter fracture site (

, arrow) corresponded with the area where the POS was noted.

 are from a 17-year-old boy with Ewing sarcoma of the left hemipelvis. Fluoroscopy was used to confirm catheter placement, and blood return and ease of flushing the catheter were verified. During a routine clinic visit 18 months later, the patient complained of a cough and “feeling funny.” A chest radiograph was read as normal. A CT scan of the chest, obtained for routine evaluation of possible metastasis, was also interpreted as normal. However, embedded in the radiologist’s report was the comment, “A unipolar transvenous pacemaker has been placed with its tip in the right ventricular apex.” What the radiologist noticed was actually a fractured piece of CVC tubing (

, arrow). Careful inspection of the patient’s earlier “normal” chest radiograph showed the fractured piece of tubing within the right atrium and ventricle (

, arrow). A second chest radiograph showed the tubing fragment lodged in the right pulmonary artery extending into the right lung (

, arrows). No POS was seen in a review of the patient’s radiographic records.

Catheter fracture and embolization of the fractured distal tubing is a rare, lesser-known risk of long-term central venous catheterization. Major risks associated with long-term CVC use are infection, obstruction, and deep venous thrombosis.

 Fracture with embolization should be watched for in any patient with an indwelling CVC. This complication is known to occur in children of all ages, including infants.

 When a long-term CVC is inserted percutaneously into the subclavian vein, the incidence of fracture with distal embolization is 1.6%, with a range of 0.1% to 2.1%.

 This commonly occurs between 6 months and 1 year after implantation.

 At our institution,  we had 2 such occurrences in 132 CVCs implanted between 2000 and 2008, for an incidence of 1.5%.

Fracture with embolization may present with signs or symptoms as serious as cardiac arrhythmia

 or as an incidental finding on routine chest imaging. The POS may be the earliest radiographic indication of impending catheter fracture. The POS is thought to occur in 1% of long-term subclavian catheters, with an associated risk of fracture estimated at 40%.

 A significant contributing factor is the gradual breakdown of the tubing secondary to compression between the clavicle and first rib as the tubing passes toward the subclavian vein through the tissue in the costoclavicular space.

Common sites for embolization of the distal fragment are the central veins, right atrium, right ventricle, and pulmonary arteries. Surprisingly, most patients are asymptomatic.  The most common symptom is pain or pressure during an attempt to use the damaged catheter.

 Other common symptoms are cough, palpitations, and swelling around the port site caused by fluid extravasation. Sustained ventricular tachycardia, although rare, may also occur.

 Retrieval of the distal fragment is commonly via a percutaneous vascular route; in some cases, open thoracotomy or even electing to leave the fragment in place may be preferable.

In the first case, a POS was present in several of the patient’s chest films but was missing in others. This is probably because of the positioning of his arm. When evaluating patients with a CVC and possible intermittent POS, radiographs taken with the arms in adduction and abduction may need to be compared.

 Placing the arms in these positions at the time of insertion may also reveal sites of impingement under fluoroscopy.

 Awareness of the POS allows for replacement of the catheter, if deemed necessary, and spares the patient the risk of complications.

The second case highlights the necessity of careful inspection of all radiographic images ordered and their respective reports. The “funny feeling” the patient had been experiencing may have been related to cardiac arrhythmias caused by stimulation of the right ventricle by the detached tubing fragment.

 Kapadia S, Parakh R, Grover T, Yadav A. Catheter fracture and cardiac migration of a totally implantable venous device. 

 Courcoux MF, Jouvet P, Bonnet D, et al. Intravascular rupture of a central venous catheter in a premature infant: retrieval by a nonsurgical technique [in French]. 

 Mirza B, Vanek VW, Kepensky DT. Pinch-off  syndrome: case report and collective review of the literature. 

 Biffi R, Orsi F, Grasso F, et al. Catheter rupture and distal embolisation: a rare complication of central venous ports. 

 Gowda MR, Gowda RM, Khan IA, et al. Positional ventricular tachycardia from a fractured mediport catheter with right ventricular migration-a case  report. 

 Schlangen JT, Debets JM, Wils JA. The “pinch-off phenomenon”: a radiological symptom for potential fracture of an implanted permanent subclavian catheter system. 

 Hinke DH, Zandt-Stastny DA, Goodman LR, et al. Pinch-off syndrome: a complication of implantable subclavian venous access devices. 

 Cheng CC, Tsai TN, Yang CC, Han CL. Percutaneous retrieval of dislodged totally implantable central venous access system in 92 cases: experience in a single hospital. 

 Hayari L, Yalonetsky S, Lorber A. Treatment strategy in the fracture of an implanted central venous catheter. 

 Adwan H, Gordon H, Nicholls E. Are routine chest radiographs needed after fluoroscopically guided percutaneous insertion of central venous catheters in children? 

Articles

Subclavian Central Venous Catheter Fracture and Embolization

A 4-month-old girl was admitted to a rural hospital with nonbilious vomiting and bloody stools that began the prior evening. The parents reported that the infant had periods of excessive irritability mixed with periods of calm. She had no fever, exposure to illness, or surgical history.

Results of a complete blood cell count and chemistry panel were normal. Plain abdominal radiographs revealed dilated loops of bowel with air-fluid levels, indicative of bowel obstruction (

Figure 1 - The plain abdominal film and left lateral decubitus film of a 4-month old girl show dilated loops of bowel (A) and air-fluid levels (B), indicative of bowel obstruction. The cause was an intussusception with a pathological lead point.

The patient was referred to a regional medical center for pediatric consultation. Her temperature was 36.6C (98F); heart rate, 162 beats per minute; respiration rate, 38 breaths per minute; and blood pressure, 122/89 mm Hg. The abdomen was markedly distended with hyperactive bowel sounds. Fullness, without a palpable mass, was noted over the central abdomen. There were no external anal fissures or rectal prolapse. On digital rectal examination, no masses were palpated; the stool was dark red and had the consistency of mucus.

Neither a pneumatic enema nor a hydrostatic enema with water-soluble contrast showed the level of obstruction or abnormality. Because there was a clear obstruction that was not corrected by radiological means, a laparotomy was performed. It revealed an ileoileal intussusception with a pathological lead point (PLP) (

). The cause of the obstruction was a mass, consistent with an adenomyoma that protruded into the bowel lumen. At discharge 5 days later, the infant was consuming a regular diet (of formula) appropriate for her age. 

Figure 2 - The ileoileal intussusception before reduction is shown (A). An external view shows the pathological lead point-a hamartoma of the small bowel (B). 

 In children with intussusception, the proximal bowel telescopes or becomes entrapped within the distal bowel.

Most cases of intussusception (roughly 80%) occur before the second year of life-typically, between 3 months and 2 years of age.

 and related to lymphatic tissue hypertrophy.

Children with a PLP are often older than 5 years and have one of the following comorbidities

     Polyps (as in Peutz-Jeghers     syndrome). 

These children are also less likely to have ileocolic intussusception than younger persons without cormordid conditions.

In the patient discussed here, the PLP was a hamartoma of the small bowel- a rare cause of intussusception in children.

 The classic clinical features are irritability and colicky pain. The parents may describe alternating intervals of well-being and extreme irritability. During an irritable period, the child may flex the lower extremities toward the abdomen. Some patients present with profound lethargy. 

Bloody diarrhea, or "currant jelly" stool, is the result of ischemic insult sustained when the venous blood supply to the mesentery and bowel becomes obstructed within the intussusception. Subsequent edema further obstructs arterial blood flow and results in the passage of a dark red, mucus-like stool.

 At this point, a rectal examination often reveals currant jelly stool on the examiner's gloved finger. 

 Vomiting is usually nonbilious when the intussusception is ileocolic. 2 When the intussusception is higher in the GI tract, bilious vomiting may be present. In this setting, consider other diagnoses associated with bilious vomiting, such as mid-gut volvulus. 

 In children with intussusception, a mass may be palpated in the upper abdomen. Because of the lack of bowel created by the intussusception, emptiness in the right lower quadrant- the Dance sign-may be observed.

. When a child presents with acute abdominal pain and any of the signs and symptoms suggestive of the comorbidities listed above, include intussusception with a PLP in the differential. For instance, this diagnosis should be considered in a child with a history of lower extremity vasculitis who presents with cramping abdominal pain. Children with Henoch- Schnlein purpura are at increased risk for intussusception caused by bowel wall hematomas.

Dilated loops of bowel with air-fluid levels, suggestive of bowel obstruction, is a possible radiographic finding in any patient who has intussusception, regardless of the location. Plain abdominal films help identify bowel perforation and may therefore obviate the need for more extensive diagnostic studies, including abdominal ultrasonography, pneumatic or hydrostatic enema with attempts at reduction, and CT.

Abdominal ultrasonography can identify the intussusception via the pseudokidney appearance longitudinally and the doughnut or target sign transversely.

 In one retrospective study, ultrasonography correctly predicted the presence of a PLP in 23 of 35 patients known to have a PLP.

 Thus, ultrasonography may be helpful when a PLP is suspected. In the absence of a PLP, ultrasonography is often recommended as a primary study because it can elucidate the difference between an unsuccessful enema reduction and residual edema or a postreduction doughnut sign.

 Radiological reduction of an intussusception is accomplished by ultrasonographic or fluoroscopic guidance. The decision about whether to perform a pneumatic or hydrostatic enema is often debated; pneumatic is usually preferred over hydrostatic enema, and water contrast instead of barium is preferred for convenience and safety.

 CT may be used when the PLP is thought to represent lymphoma and staging of the cancer is necessary.

If radiological procedures fail to reduce the intussusception, as in this patient's case, exploratory laparotomy is needed to restore or excise the affected bowel. In patients with bowel perforation, which can occur secondary to radiological reduction, surgery is urgently necessary. 

A single recurrence can be reduced radiologically, whereas a second recurrence often requires surgery. The recurrence rate after radiological or surgical reduction is fairly low and about the same for both procedures. 

A PLP and its cause require surgical intervention.

 Recurrent intussusception can be caused by a PLP: operative correction is necessary. 

Fallat ME. Intussusception. In: Ashcraft KW,    Holcomb GW, Murphy JP, eds. Pediatric Surgery.    4th ed. New York: Elsevier; 2005:533-542.

    McCollough M, Sharieff GQ. Abdominal pain in    children. Pediatr Clin North Am. 2006;53:107-137.    

Orenstein J. Update on intussusception. Contemp    Pediatr. 2000;17:180-181,185-186,188-191.    

    Wyllie R. Ileus, adhesions, intussusception, and    closed-loop obstructions. In: Behrman RE, Kliegman    RM, Jenson HB, eds. Nelson Textbook of Pediatrics.    17th ed. Philadelphia: Saunders; 2004:1241-1243.    

    Navarro O, Dugougeat F, Kornecki A, et al.    The impact of imaging in the management of intussusception    owing to pathologic lead points in    children. A review of 43 cases. Pediatr Radiol. 2000;    30:594-603.

Ikegami R, Watanabe Y, Tainaka T. Myoepithelial    hamartoma causing small-bowel intussusception: a    case report and literature review. Pediatr Surg Int.    2006;22:387-389.    

Stolar CJ. Intussusception. In: O'Neill JA, Grosfeld    JL, Fonkalsrud EW, et al, eds. Principles of Pediatric    Surgery. 2nd ed. St Louis: Mosby; 2003:527-530.    

Leung AK, Wong AL, Kao CP. Photo essay: GI    obstructions in children, part 2. Consultant. 2002;42:    491-495.    

Ko HS, Schenk JP, Troger J, Rohrschneider WK.    Current radiological management of intussusception    in children. Eur Radiol. 2007 Feb 17; [Epub ahead    of print].    

A 4-month-old girl was admitted to a rural hospital with nonbilious vomiting and bloody stools that began the prior evening. The parents reported that the infant had periods of excessive irritability mixed with periods of calm. She had no fever, exposure to illness, or surgical history. 

Bonna Benjamin, MD

Articles

Subclavian Central Venous Catheter Fracture and Embolization

Intussusception With a Pathological Lead Point