A prospective, longitudinal study of central venous catheter-related deep venous thrombosis in boys with hemophilia.

BACKGROUND: Central venous catheters (CVCs) are often inserted into boys with hemophilia to secure venous access for factor prophylaxis and immune tolerance induction therapy. Complications associated with CVCs include catheter-related infections, local hemorrhage, and mechanical failure. Less frequently reported is CVC-related deep venous thrombosis (DVT). We conducted a prospective study to determine the frequency and outcome of this complication. METHODS: All boys (n = 16) with congenital hemophilia A or B with a CVC in place who were registered in the pediatric comprehensive care program at the Hospital for Sick Children, Toronto, were included in the study. They were prospectively assessed by imaging studies and clinical examinations for CVC-related DVT at two time-points, 2 years apart. Each boy was evaluated for inherited hypercoagulability. RESULTS: Eleven (69%) of the 16 boys had radiological evidence of DVT at the first evaluation and 13/16 (81%) at the second evaluation. In two boys there was improvement in the venogram findings at the second evaluation. None of the CVC-related DVTs completely resolved. Median age at the time of initial insertion of a CVC was 1.0 years (range 0.02-6.7 years). Median duration of CVC placement was 6.4 years (range 3.3-15.5 years). Only 4/13 boys with DVTs had clinical evidence of upper venous system obstruction. Only one boy, who did not develop a DVT, had a low protein C level. CONCLUSIONS: CVC-related DVTs occur in the majority of boys with hemophilia who have CVCs inserted for a prolonged period of time. Annual screening with imaging is recommended for boys with CVCs in place for >/= 3 years. Consideration should be given to removing CVCs as soon as peripheral venous access is feasible.

Correlation between radiographic transition zone and level of aganglionosis in Hirschsprung's disease: Implications for surgical approach.

BACKGROUND/PURPOSE: The anticipated level of aganglionosis can influence the surgical approach to Hirschsprung's disease. The aim of this study was to determine the accuracy of the contrast enema in predicting this level. METHODS: Over a 6-year period (1995 through 2000), 88 patients with Hirschsprung's disease underwent surgical correction. Preoperative contrast enema findings were available for 75 of these patients and were compared with operative and pathology reports. Data were analyzed by chi(2). RESULTS: The contrast enema showed a transition zone suggestive of Hirschsprung's disease in 67 of 75 patients (89%). In 59 of 67 (88%), the pathologic and radiographic transition zones were concordant. Seven of the 8 patients with discordant studies had total colonic (n = 5) or long-segment (n = 2) disease. Contrast enema correctly predicted the level of aganglionosis in 55 of 62 (89%) patients with rectosigmoid disease but only 4 of 13 (31%) of those with long-segment or total colonic disease (P <.01). Of the patients with a radiographic transition zone in the rectosigmoid, 54 of 60 (90%) had a matching level of aganglionosis. CONCLUSIONS: In rectosigmoid Hirschsprung's disease, the location of the radiographic transition zone correlates accurately with the level of aganglionosis in 90% of cases. However, the small incidence of discordance between anticipated level of aganglionosis and operative findings should be recognized, particularly when planning a one-stage transanal pull-through.

The impact of imaging in the management of intussusception owing to pathologic lead points in children. A review of 43 cases.

OBJECTIVE: To review the imaging appearances, management and outcome of a large number of children with intussusception owing to pathologic lead points (PLP) in an attempt to define the role of various imaging modalities in this clinical setting. MATERIALS AND METHODS: Review of the records and imaging studies of 43 children with intussusception due to PLP diagnosed between 1986 and 1999. RESULTS: The commonest PLP found were Meckel diverticulum, polyps, Henoch-Schonlein purpura and cystic fibrosis. PLP were depicted on sonography in 23 (66%) of 35 patients, on computed tomography in 5 (71%) of 7, on air enema in 3 (11%) of 28, and on barium enema in 6 (40%) of 15. Air enema successfully reduced 60% of the intussusceptions. Nine children had recurrent intussusceptions. CONCLUSION: Sonography depicted two-thirds of PLP and provided a specific diagnosis in nearly one-third of our series. Our review does not provide sufficient data on how to continue the investigation of those patients in whom sonography does not depict a PLP but in whom there is a high index of suspicion for its presence. It remains a diagnostic challenge as to how to search for PLP in these patients, and other imaging modalities have to be requested according to each particular case.

Spontaneous reduction of intussusception: clinical spectrum, management and outcome.

BACKGROUND: To analyze the spectrum of clinical features, management and outcome of children with documented spontaneous reduction of intussusception (SROI). MATERIALS AND METHODS: Review of records of 50 children (33 boys, 17 girls; age range 11 days-15 years; mean age 4 years) with documented SROI, in whom intussusception was initially diagnosed by sonography (US) in 44, air enema in 2, and computed tomography in 4, in the 6-year period 1992-1998. RESULTS: Symptoms suggestive of intussusception were present in 21 (3 of whom had Henoch-Schönlein purpura and 4 had previous ileocolic intussusception reduced by air enema). Intussusception was an incidental finding in the other 29, in 28 of whom the finding was in the small bowel. Intussusception was limited to the small bowel in 43 and was ileocolic in 7. SROI was usually documented on US. Laparotomy performed in only 4 showed no evidence of intussusception or pathologic lead point. Outcome in all patients was favorable. CONCLUSIONS: SROI may present in symptomatic or asymptomatic children and occurs more commonly than previously reported. These intussusceptions are usually short-segment, small-bowel intussusceptions with no recognizable lead point. In asymptomatic patients, conservative observation is warranted. Intervention should be dictated by the clinical findings in symptomatic patients.

Unsuccessful air-enema reduction of intussusception: is a second attempt worthwhile?

Pneumatic reduction of idiopathic intussusception is successful in about 80% of cases, while 60% of the failures are reduced at surgery without resection. To determine whether delayed, repeated attempts at enema reduction of failures would reduce the need for operation in selected cases, over a 2-year period (1994-1996 inclusive), 17 infants with idiopathic intussusception underwent delayed repeat enemas 2-19 h following the first failed attempt at reduction. Clinical parameters and radiologic findings were evaluated with respect to outcome. Ten intussusceptions were successfully reduced after the second attempt in 9 and after the fourth attempt in 1. Seven children underwent a laparotomy, 5 because of failure of progressive reduction at air enema (AE). Two were taken to surgery early in the series, 1 because of perforation during a second attempt and 1 while awaiting a third reduction attempt. The 10 successful reductions all showed progressive movement of the intussusceptum on each AE; the 2 who perforated failed to show progressive reduction on their second AE. Because of these cases, the remaining 5 were referred to surgery because of failure of progressive reduction of the intussusceptum on the second attempt. At laparotomy, of the 7 unsuccessful reductions, 4 required resection and 3 had difficult manual reduction. The presence of vomiting, a mass, and/or bloody stools were not predictors of outcome. Failures had higher body temperatures (38.1 +/- 0.3 vs 37.4 +/- 0.1 degrees C, P = 0.07), heart rates (153.7 +/- 8 vs 136.9 +/- 2.1 min, P = 0.03), and longer duration of symptoms (36.8 +/- 4 vs 21.3 +/- 3.6 h; P = 0.01) than successes. Delayed repeat AEs may be safe and effective in selected cases of idiopathic intussusception, but should be considered only if significant movement of the intussusceptum is noted at each attempt. The ideal time for repeat AE reduction prior to surgery is not established, but 2-4 h appears appropriate. Pyrexia, tachycardia, and duration of symptoms greater than 36 h are relative contraindications to this course of management.

Colo-colic intussusception associated with pneumatosis cystoides intestinalis.

This paper describes pneumatosis cystoides intestinalis in association with colo-colic intussusception in a young teenager. The intussusception was easily reduced at barium enema. The recognition of the characteristic filling defects in the barium column facilitates a correct diagnosis. This association has only been reported previously in six adults.

Patterns of recurrence of intussusception in children: a 17-year review.

PURPOSE: Patterns of recurrence of intussusception (INT) were reviewed to determine whether changes in management have affected the rate and patterns of recurrence as well as long-term outcome in children with multiple (i. e., 2 or more) recurrences. MATERIALS AND METHODS: Review was done of 763 children with 876 intussusceptions, including (1) recurrence rate, (2) patterns of recurrence (number of and interval between recurrences), (3) reducibility, (4) pathologic lead points (PLP), (5) operative findings and (6) long-term follow-up in those with multiple recurrences. RESULTS: Above features (1)-(6) were the same in those managed with barium enema (1979-1985) and those managed with air enema (1985-1996). Overall recurrence rate was 9 %; 11 % with barium enema and 8 % with air enema. Sixty-nine patients had 113 recurrences: 47/69 (68 %) and 1 recurrence and 22/69 (32 %) had multiple recurrences. Multiple recurrences presented as isolated episodes or in clusters up to 8 years. Reducibility was 100 % for initial INT and 95 % for recurrent episodes; there were no perforations. Surgery, in 4 with irreducible recurrence, revealed no PLP. PLP were present in 5 (8 %): 2 (4 %) with 1 recurrence and 3 (14 %) with multiple recurrences. No pattern of recurrence was predictive for PLP. Long-term follow-up (up to 15 years) available in 11 with multiple recurrences revealed a favourable outcome. CONCLUSIONS: Rates and patterns of recurrence did not change with altered management. Because of the high reduction rate of recurrences, lack of perforation and favourable long-term follow-up, we recommend radiological reduction for recurrent INT. Multiple recurrences are not a contraindication. A careful search for PLP is mandatory. Surgery should be reserved for irreducible recurrences or for demonstrated PLP.

The value of sonography, CT and air enema for detection of complicated Meckel diverticulum in children with nonspecific clinical presentation.

BACKGROUND: Complicated Meckel diverticulum (MD) in children does not always present with painless rectal bleeding and its presentation can then produce a difficult diagnostic dilemma. In this clinical setting, sonography (US), CT or even air enema may be the first modality chosen to evaluate these children rather than the radionuclide Meckel scan (RNMS). PURPOSE: To assess the value of US, CT and air enema for detection of complicated MD. MATERIALS AND METHODS: Review of clinical, imaging, surgical and pathological findings in 64 children (55 males, 9 females) aged 4 days -14 years (mean = 3.7 years) with MD seen during an 8-year period, 1990-1997. RESULTS: (a) In 33 patients with rectal bleeding, MD was detected on RNMS in 32. Ten of these 32 had other imaging studies, all of which were negative. (b) The other 31 patients, with varied clinical presentations, did not undergo RNMS. In these 31 and the 1 with a negative RNMS, 14 (44 %) had imaging features highly suggestive for the diagnosis of MD on US in all 14, on CT in 1, and on air enema in 3. The radiological spectrum of the inflamed, hemorrhagic MD is illustrated. CONCLUSION: The inflamed, hemorrhagic and the inverted, intussuscepted MD have a spectrum of features recognizable on US, CT and air enema. Some of these appearances are specific, others are not. Knowledge of and recognition of these features will facilitate detection of complicated MD in larger numbers of children presenting with symptoms other than the classic history of painless rectal bleeding and also in those with normal RNMS.

The appearance of inverted Meckel diverticulum with intussusception on air enema.

This paper presents the appearances of inverted Meckel diverticulum with an irreducible intussusception on air enema in four children. The inverted Meckel diverticulum appeared as a bulbous (3) or triangular (1) filling defect in the air column projecting off the distal end of the soft tissue mass of the irreducible intussusceptum. The bulbous defect appears to be highly suggestive, and may be specific, for inverted Meckel diverticulum. Earlier recognition of the presence of the Meckel diverticulum as the lead point of the intussusception could have changed the management in two of the children.

The diagnosis of malrotation during air enema procedure.

This paper describes the correct diagnosis of cecal malposition, suggesting midgut malrotation, during air enema examination in seven patients. It is possible to diagnose cecal malposition by air enema, even in the presence of a reducible intussusception.

Intussusception. Issues and controversies related to diagnosis and reduction.

Diagnosis and management of intussusception remain controversial. The authors discuss the evolution of changes and advances in practice and procedures. They advocate sonographic diagnosis and air enema reduction; the abdominal radiograph is reserved for children with clinical evidence of peritonitis and suspected perforation, if clinical findings are unusual, or if the sonographic examination is equivocal.

Intussusception: ability of fluoroscopic images obtained during air enemas to depict lead points and other abnormalities.

PURPOSE: To evaluate the ability of fluoroscopic images obtained during air enemas to depict or exclude lead points of intussusceptions and other abnormalities that require surgical treatment in the absence of intussusception. MATERIALS AND METHODS: The clinical, radiologic, surgical, and pathologic findings were reviewed in 14 patients with lead points or other lesions. RESULTS: Fluoroscopic images failed to depict a lead point in 10 patients. The air enema easily reduced intussusceptions with benign lead points in seven patients. Fluoroscopic images depicted pathologic lead points in two patients and were normal in two patients with intussusception. Fluoroscopic images failed to depict an abnormality that required surgery in the absence of intussusception in two patients. CONCLUSION: Successful reduction of an intussusception does not always rule out a lead point. Other imaging studies, the patient's condition, and laparotomy may also be necessary to diagnose and treat lead points and other lesions.

Partially reduced intussusception: when are repeated delayed reduction attempts appropriate?

The imaging techniques for diagnosis and monitoring of reduction of intussusception by fluoroscopy and ultrasound continue to evolve. The common goal of all protocols is to reduce the intussusception by enema in as many patients as possible and avoid laparotomy with its potential morbidity. We report two infants in whom the initial attempt at reduction by air enema only achieved partial reduction, from the descending colon to the transverse colon in one, and from the splenic flexure to the caecum in the other. Both patients became asymptomatic and clinically stable. In light of the clinical stability, and in consultation with the surgeons, laparotomy was deferred. Sonography was used to confirm the persistence of the intussusception before repeat air enema several hours later. Following three further air enemas in each child, the intussusceptions were successfully reduced after 20 h and 24 h respectively. Both patients remained asymptomatic and did not require surgery. In infants with partially reduced intussusception we suggest that if the patient becomes asymptomatic and stable, surgery can be safely delayed to permit further attempts at enema reduction. Sonography has a valuable role in determining the persistence of the intussusception prior to repeat enema. Close cooperation with the surgeon and careful clinical monitoring of the patient are essential requirements for this proposal.

Perforation during attempted intussusception reduction in children--a comparison of perforation with barium and air.

This paper compares the effects on patients of perforation with barium and with air during attempted intussusception reduction by reviewing the clinical, radiological, surgical and pathological findings and sequelae in seven children who received barium and seven who received air. In both groups perforation occurred in infants under 6 months of age (with one exception) with a long duration of symptoms. All patients with barium enema required resection of bowel whereas only four with air enema required resection. Anesthetic times were longer in those patients with barium perforation in whom the intussusception did not move and there was a large leak. The patients with perforation due to air had a shorter hospital stay with decreased morbidity compared to those with perforation due to barium. Perforation occurred through areas of transmural necrosis in a minority of patients in each group. Perforations through normal bowel and shear injury (with air enema) indicate that increased pressure during the examination is an important factor in some patients. Because perforation with air is so much easier to deal with surgically and the children do better clinically, there is a tendency for some to consider perforation with air an inconsequential situation. However, a potential rare complication with this technique is tension pneumoperitoneum. Keeping this in mind, we continue to use air as the contrast of choice because the procedure in our hands is a safe, quicker and easier technique and we have achieved a substantial improvement in reduction rates. Overall reported perforation rates with air enema compare favourably with those due to barium enema.(ABSTRACT TRUNCATED AT 250 WORDS)

Intussusception: toward less surgery?

Since the 1950s, several large pediatric centers have used hydrostatic reduction with barium under fluoroscopic control as the treatment method of choice for ileocolic intussusception and have adopted rigid criteria for its management. One such rule has been that in order for an intussusception to be completely reduced, there must be adequate reflux of barium into the distal ileum. If this did not occur, it was assumed that the ileocolic intussusception had not been reduced, and the infant or child was taken straight to the operating room for laparotomy and surgical treatment. However, 10% of such intussusceptions were found to have reduced spontaneously. Needless to say, nonoperative management reduces morbidity and shortens hospitalization. From October 1985 through March 1991, 503 air contrast colon studies for suspected intussusception were performed on infants and children aged 2 days to 13 years (average, 16.8 months); 262 (52%) were normal, and 241 had an intussusception, 196 (81%) of which were reduced. The remaining 45 were operated on. In three patients (4 months to 2 years of age) the air enema reduced the intussusception from the colon without terminal ileum filling, but they all became asymptomatic immediately. For this reason they were not operated on; they were admitted and observed for 24 to 48 hours. Two of the three had recurrence of abdominal pain the next morning, but results of repeat air enemas were all normal (no intussusception observed, and normal terminal ileum filling).(ABSTRACT TRUNCATED AT 250 WORDS)

Pneumatic reduction of intussusception: 5-year experience.

Pneumatic reduction of 246 intussusceptions was attempted in 219 patients over a 5-year period. The mean age of the patients was 15.4 months. Successful reduction was achieved in 199 cases (80.9%). Bowel perforation occurred in seven cases (2.8%), requiring needle decompression of tension pneumoperitoneum in one case. Recurrence of intussusception occurred in 27 cases (11%). The mean fluoroscopy time was 3.5 minutes +/- 0.2 in successful reductions and 9.3 minutes +/- 0.9 in failed reductions (P less than .001). Logistic regression analysis helped identify four independent predictors of failure, as follows: (a) ileoileocolic intussusception (P less than .001), (b) long duration of symptoms (P less than .001), (c) rectal bleeding (P less than .01), and (d) failed reduction with barium at another institution (P less than .05). Predictors of bowel perforation were a younger age (P less than .05) and long duration of symptoms (P less than .05). Surgery was performed in 48 cases (19.5%), 16 of which required bowel resection. Transmural necrosis of bowel wall was found in nine specimens. The most important predictor of outcome in this series was a long duration of symptoms. Pneumatic reduction is a useful substitute for barium in the management of pediatric intussusception.

Lymphoid follicular pattern in the colon: an indicator of barium coating.

A lymphoid follicular pattern was noted more frequently when high-density (100% w/v) replaced low-density (58% w/v) barium in children undergoing double-contrast barium enema examinations. Therefore, a retrospective study was undertaken to compare the presence of follicles seen in 24 consecutive patients under 10 years old given low-density barium with that of 50 patients given high-density barium. The high-density studies revealed follicles in 90% of the children compared with 46% of the children having low-density studies. All follicles were less than 2 mm in diameter. An aphthoid appearance was present in nine of these 50 children. The most common indications for the high-density examination were rectal bleeding and abdominal pain. The final diagnosis was normal bowel in more than two thirds; no child under six years old given high-density barium had inflammatory bowel disease. There was no correlation between final clinical diagnosis and the presence, extent, or severity of the lymphoid follicular pattern.

Compensation filtration in paediatric double contrast barium enema examinations.

A prospective study of double contrast barium enema examinations performed on 42 children aged 2.5-19 years (mean age 12.3 years) showed that compensation filtration improved the radiographic quality in 32 (76%) of the examinations; it also reduced radiation dosage in all patients. In no examination was the use of a filter deleterious. Compensation filtration is recommended for all paediatric double contrast barium enema examinations to improve radiographic quality and reduce radiation dosage.

Value of the peroral pneumocolon in children.

This is the first study in children of the use of the peroral pneumocolon, which entails insufflation of air per rectum to examine the terminal ileum and cecum in conjunction with a conventional barium follow-through examination. Of 1188 conventional barium follow-through examinations performed during 2 1/2 years, results were ambiguous in 13 patients, who then underwent a peroral pneumocolon study. This study showed terminal ileitis in six patients, confirming the diagnosis of Crohn disease in five and Behçet syndrome in the other. In four patients peroral pneumocolon findings were normal. More extensive cecal or proximal ileal disease was shown in three patients by peroral pneumocolon, and fistulae suspected on conventional study were confidently excluded in two others. In all 13 the examination was well tolerated, and avoided further, more invasive procedures. The peroral pneumocolon is a useful procedure in children for these indications.