Primary laparoscopic repair of high imperforate anus in neonatal males

Purpose

The standard approach to males with high imperforate anus has been a staged procedure starting with a descending colostomy, then posterior sagittal anorectoplasty with colostomy closure after 3 months. Recently, a minimally invasive approach to the repair of high imperforate anus has been described in infants after colostomy. We describe 6 newborn males with high imperforate anus successfully repaired laparoscopically as a primary, single-stage procedure.

Methods

A retrospective chart review was performed on all patients with imperforate anus from October 2003 to October 2006.

Results

We evaluated 9 newborn males with high imperforate anus. Of these patients, 6 underwent primary laparoscopic repair on day 1 to day 2 of life. Of these 6 patients, 3 were found to have bladder neck fistulas, whereas the other 3 had prostatic urethra fistulas. All patients passed stool within the first 72 hours postoperatively. One patient has required a procedure for a mild rectal prolapse. Follow-up ranges from 2 to 30 months in the single-stage group.

Conclusion

Our early results using primary laparoscopic repair appear encouraging. Laparoscopy allows excellent visualization and assessment of the fistula and repair of high imperforate anus without need for colostomy. Long-term follow-up will be needed to assess outcomes and continence rates.     

Wound infection after colostomy closure for imperforate anus in children: utility of preoperative oral antibiotics

Background/Purpose

There is little published data on the efficacy of surgical infection prophylaxis in children. The purpose of this study was to assess wound infection rate in children undergoing colostomy closure for imperforate anus and evaluate the impact of bowel preparation and antibiotics.

Methods

Children younger than 18 years with imperforate anus who had a colostomy closure between January 1996 and December 2007 were identified. Data collected included demographics, bowel preparation, antibiotics, operative details, and postoperative infections. Comparison of mechanical bowel preparation and intravenous antibiotics with and without oral antibiotics was compared using χ² tests. Significance was defined as P < .05.

Results

A total of 118 patients were identified. Primary skin closure was done in 97%. Mechanical bowel preparation was used in 93%, intravenous antibiotics in 97%, and oral preoperative antibiotics in 52%. Wound infections occurred in 14% (n = 17). The addition of oral antibiotics to the standard regimen of mechanical bowel preparation with intravenous antibiotics did not alter infection rate (13% versus 17%, P = .64).

Conclusion

Wound infection in children undergoing elective colostomy closure for imperforate anus was 14%. Infection rate was not affected by use of oral antibiotics. Future studies may allow specific guideline development for infection prophylaxis in pediatric patients.     

Midterm follow-up study of high-type imperforate anus after laparoscopically assisted anorectoplasty

Purpose

The aim of this study was to evaluate postoperative anal function of high-type imperforate anus after laparoscopically assisted anorectoplasty (LAARP).

Methods

Between 2000 and 2002, LAARP was performed in 13 patients with high-type imperforate anus. Clinical data of the LAARP group were compared with the posterior sagittal anorectoplasty (PSARP) group (n = 7) operated before 2000. All patients were treated with initial colostomy in the newborn period. After anorectoplasty, daily glycerin enemas were given for postoperative bowel management. Anorectal function of these patients was evaluated using the Kelly score and manometry at the age of 3 to 5 years (LAARP) and 5 to 6 years (PSARP).

Results

Age at evaluation in the LAARP group was younger than that in the PSARP group (51 ± 10 vs 73 ± 12 months, P < .01). Kelly score was 3.8 ± 1.3 vs 3.4 ± 0.8, respectively. Resting pressure of internal sphincter was 31 ± 11 vs 33 ± 10 cm H₂O, respectively. Relaxation reflex of the internal anal sphincter was observed in 62% (8/13) and 29% (2/7), respectively.

Conclusions

Midterm follow-up study revealed that satisfactory fecal continence can be achieved in patients with high-type imperforate anus after LAARP. Laparoscopically assisted anorectoplasty may be a good alternative in this patient population. However, long-term follow-up is necessary to compare the benefits of LAARP against PSARP.     

Colonic manometry as predictor of cecostomy success in children with defecation disorders

Purpose

The aim of this study was to define the predictive value of colonic manometry and contrast enema before cecostomy placement in children with defecation disorders.

Methods

Medical records, contrast enema, and colonic manometry studies were reviewed for 32 children with defecation disorders who underwent cecostomy placement between 1999 and 2004. Diagnoses included idiopathic constipation (n = 13), Hirschsprung's disease (n = 2), cerebral palsy (n = 1), imperforate anus (n = 6), spinal abnormality (n = 6), and anal with spinal abnormality (n = 4). Contrast enemas were evaluated for the presence of anatomic abnormalities and the degree of colonic dilatation. Colonic manometry was considered normal when high-amplitude propagating contractions (HAPC) occurred from proximal to distal colon. Clinical success was defined as normal defecation frequency with no or occasional fecal incontinence.

Results

Colonic manometry was done on 32 and contrast enema on 24 patients before cecostomy. At follow-up, 25 patients (78%) fulfilled the success criteria. Absence of HAPC throughout the colon was related to unsuccessful outcome (P = .03). Colonic response with normal HAPC after bisacodyl administration was predictive of success (P = .03). Presence of colonic dilatation was not associated with colonic dysmotility.

Conclusion

Colonic manometry is helpful in predicting the outcome after cecostomy. Patients with generalized colonic dysmotility are less likely to benefit from use of antegrade enemas via cecostomy. Normal colonic response to bisacodyl predicts favorable outcome.     

A historical comparison of long-term behavioral and emotional outcomes in children and adolescents after invasive treatment for congenital heart disease

Background/Purpose

Children with congenital heart disease (ConHD) are known to be vulnerable to behavioral and emotional problems. In this study, a historical comparison is made between the level of behavioral and emotional problems in a sample of children and adolescents with ConHD treated recently vs a comparable historical sample operated upon before 1980 in the same institute. The hypothesis was that improvements in medical care would result in more favorable behavioral and emotional outcomes for children and adolescents with ConHD treated recently, that is, between 1990 and 1995, compared with same-aged patients operated on before 1980.

Methods

To assess behavioral and emotional problems, the Child Behavior Checklist (parent report) and Youth Self-Report were used. The historical samples (n = 98 and n = 123, respectively) and recent samples (n = 90 and n = 84, respectively) consisted of 4 diagnostic groups.

Results

Parents and patients from the recent sample with ConHD reported fairly similar levels of behavioral and emotional problems compared with parents and patients in the historical sample with ConHD.

Conclusion

Despite evident improvements in diagnostic and surgical techniques and medical treatment of ConHD over the past decades, virtually no changes were found in levels of problem behavior of the recent patient sample compared with the historical patient sample, who both underwent invasive treatment for ConHD.     

The morbidity of constipation in patients with anorectal malformations

Background

Constipation in anorectal malformations (ARM) is extremely common, particularly in the lower types. Failure to adequately treat it can lead to significant morbidity.

Methods

From our series of over 2000 patients with ARM, we reviewed 398 with good prognosis for bowel control and a tendency toward constipation; rectoperineal fistula (63), rectovestibular fistula (114), rectobulbar urethral fistula (104), imperforate anus with no fistula (46), rectal atresia or stenosis (9), and cloaca with a common channel below 3 cm (62). Those lost to follow-up, not yet toilet-trained (<3 years old), or with poor prognostic features were excluded. We compared morbidities in patients we operated on and managed primarily (group A, n = 268) to those managed at other institutions who suffered from constipation or incontinence and were referred to us for treatment (group B, n = 130). Those we managed primarily were subjected to an aggressive senna-based laxative program, started after their primary repair or after colostomy closure.

Results

Morbidities associated with constipation were higher in the referral group and included fecal impaction (7.8% vs 38.5%), overflow pseudoincontinence (4.9% vs 33.8%), and megacolon (14.6% vs 54.6%). A loop or transverse colostomy (4.9% vs 9.2%), stoma or anorectal stricture, or a stenotic fistula (2.2% vs 28.5%) were contributing factors. Adequate laxative treatment with, in certain cases, resection of a megarectosigmoid (2.6% vs 23.1%) enabled many pseudoincontinent children to achieve bowel control (reported previously). Unneeded colorectal biopsies (1.9% vs 16.2%), Hirschsprung's-type pullthroughs (0% vs 3.1%), and, in retrospect, unneeded antegrade continent enema procedures (0% vs 3.1%) were higher in Group B. Overall, 19.8% of Group A and 66.2% of Group B experienced constipation-related morbidities.

Conclusion

The morbidity of constipation in ARM includes fecal impaction, megacolon, incontinence, and performance of unneeded surgeries. Inadequate treatment, the type of the original colostomy, and postoperative anal or stomal stricture as well as stenotic fistulae were key contributing factors. Children with ARM and good prognosis for bowel control are at the greatest risk for severe constipation and its consequences. With recognition and aggressive, proactive treatment, we have found that these morbidities can be reduced.     

15-Year experience in the treatment of rectal prolapse in children

Background

Rectal prolapse is a common and usually self-limited condition in children. Several surgical techniques have been advocated for refractory prolapse. We reviewed our experience with treatment and the outcome of refractory rectal prolapse.

Methods

Retrospective review was conducted on patients undergoing surgery for rectal prolapse from January 1993 to March 2009. Patients with imperforate anus/cloacal abnormalities, Hirschsprung disease, spina bifida, or prior pull-through were excluded.

Results

Twenty patients underwent 23 procedures for rectal prolapse. There were 10 posterior sagittal rectopexies, 6 transabdominal rectopexies, 5 laparoscopic rectopexies, 1 hypertonic saline injection, and 1 anal cerclage. The mean duration of symptoms was 1.6 years (range, 1-10 years). The mean age at operation was 6.8 years (range, 4 months-19 years), with a 5:1 male predominance. There was no operative or perioperative mortality. Median length of follow-up was 7.2 months; 2 patients were lost to follow-up.The overall recurrence rate was 35%. All recurrences followed posterior sagittal rectopexies, which had a 70% recurrence rate. Four patients required reoperation, all done transabdominally (2 open and 2 laparoscopically). None of the 3 remaining patients with mild recurrences required reoperation.

Conclusions

A variety of options for management of refractory rectal prolapse in children exist. Laparoscopic rectopexy seems to be safe and a comparatively successful option in these children.     

Perforation of the colon in neonates

Purpose

Gastrointestinal perforation is a catastrophic condition in neonates, especially in premature neonates. Although perforation is commonly observed in the small intestine during the neonatal period, perforation of the colon is a rare condition. This study analyzed the clinical findings and results of perforation of the colon in neonates.

Methods

Between 1989 and 2004, 8 neonates were treated for spontaneous perforation of the colon at our institute. These patients were retrospectively reviewed.

Results

Gestational ages ranged from 36 to 41 weeks. Seven patients weighed above 2500 g, whereas one patient weighed 1800 g at birth. Perforations developed within 7 days after birth in 6 patients and before birth in two. Associated bowel diseases included rectosigmoid type of Hirschsprung's disease in two patients, immature ganglia in one, imperforate anus in one, colonic atresia in one, and necrotizing enterocolitis in one. An obvious cause was not identified in the remaining two. Six patients without definite anatomic obstructions, such as imperforate anus or colonic atresia, required evaluations for suspected Hirschsprung's disease. All 8 patients underwent colostomy and recovered from peritonitis. Seven survived, but one died of sudden infant death syndrome.

Conclusions

In this study, perforation of the colon during the neonatal period mostly occurred in term or near-term neonates and carried a good prognosis. During management, it was important to identify Hirschsprung's and its allied disorders as a cause of perforation.     

Mucosal nerve deficiency in chronic childhood constipation: a postmigration defect?

Purpose

Idiopathic chronic childhood constipation (ICCC) includes children who are severely constipated and who are resistant to behavioral or medical treatments. These children are distinguished from those with Hirschsprung's disease (HSCR) by the presence of enteric ganglia in rectal biopsy specimens. We investigated potential autonomic dysfunction by examining nerves in rectal mucosa.

Methods

Immunostaining, confocal microscopy, and nerve analysis were performed on formalin-fixed and on Zamboni-fixed rectal biopsy specimens from children who were severely constipated. A computer-assisted neuron tracing technique was used to determine mucosal nerve density in Zamboni-fixed biopsy sections.

Results

Nerves in Zamboni-fixed biopsy specimens were better stained than in formalin-fixed biopsy specimens. Regardless of fixation method, a deficiency of mucosal nerves was observed in ICCC when compared to children who are not constipated. Analysis of autotraced mucosal nerves confirmed the deficiency in ICCC biopsy specimens. Mucosal nerves were also severely deficient in patients with HSCR, even in transitional segments that contained ganglia.

Conclusions

Most patients with ICCC had decreased innervation of the rectal mucosa. Because mucosal nerves are critical for the peristaltic reflex, water secretion, and absorption, their deficiency can be related to patient constipation. Mucosal nerve density provides a pathologic basis for diagnosis of dysfunction in children who do not have HSCR but are chronically constipated. The study validates the neuron tracing method for objective evaluation of mucosal innervation.     

Bowel vaginoplasty in children: a retrospective review

Background/purpose

There are numerous congenital genitourinary conditions that result in vaginal agenesis or atresia. Reconstruction presents a challenge to the surgeon who wishes long-term functional and cosmetic results with low morbidity. Historically, reconstruction has involved the use of skin grafts and nonoperative methods with less than ideal results. The preference of the authors is to use intestinal segments for creating the neovagina and herein review their experience with this procedure.

Methods

The authors identified 10 children who underwent vaginal construction with intestinal segments from 1996 to the present. Patient charts were reviewed for etiology, procedure performed, operative and postoperative complications, and follow-up.

Results

Average age at surgery was 10.9 years (1 to 29 years). Of the 10 children, 3 required vaginal construction for cloacal extrophy, and 7 had vaginal atresia of various etiology. Sigmoid colon was utilized in 6 cases, ileum in 2, and, in 2 patients with high imperforate anus, their mucous fistula segment was used to create the vagina. Mean follow-up was 24 months. Complications included a right ureteral injury necessitating placement of an indwelling ureteral stent and a girl presenting with cyclic pelvic pain who required reanastomosis of the neovagina to the uterus for an obstructed cervical os.

Conclusions

Intestinal segments can be used successfully for creation of a neovagina with exceptional cosmetic results and an acceptable complication rate. In appropriate patients, the mucous fistula can be used to create the vagina obviating the need for a bowel anastomosis.     

Medical predictors for long-term behavioral and emotional outcomes in children and adolescents after invasive treatment of congenital heart disease

Background

The aim of the study was to test the following: (1) the predictive value of medical variables for long-term parent-reported behavioral and emotional problems in children and adolescents who underwent invasive treatment of congenital heart disease in infancy and (2) the relationship between parental psychological distress and parental reports on problems in children.

Methods

The Child Behavior Checklist was used to investigate to what extent behavioral and emotional problems in 7- to 17-year-old children with congenital heart disease can be predicted by the following: (1) medical history, (2) therapeutic intervention and direct postinterventional course, (3) long-term medical course, (4) present contact with physicians, and (5) present medical status. The General Health Questionnaire was used to assess parental distress (especially anxiety).

Results

Higher Child Behavior Checklist total problems scores were predicted by cardiac medication before therapeutic intervention. Palliative intervention (Rashkind procedure) before therapeutic intervention was associated with more favorable scores on total problems and externalizing. Long-term maternal distress was significantly related to parent-reported problems in children.

Conclusion

Long-term behavioral and emotional outcomes are only marginally predicted by medical variables. In counseling of children with congenital heart disease and their parents, attention should be paid to long-term maternal distress that has an influence on parent-reported problems in children.     

Anal ultraslow waves and high anal pressure in childhood: a clinical condition mimicking Hirschsprung disease

Purpose

Anal ultraslow waves (USWs) have been described in several clinical conditions closely related to chronic constipation associated with high anal pressure; however, USW-related clinical manifestations in childhood are poorly understood. The purpose of this study is to elucidate the clinical relevance of USWs in childhood.

Methods

Manometric recordings of 118 cases including 70 children with constipation and 16 patients with Hirschsprung disease were analyzed.

Results

Ultraslow waves were seen in 4 of 70 children with constipation. None of the controls or patients with Hirschsprung disease exhibited USWs. The 4 patients comprised 2 infants with marked abdominal distension mimicking Hirschsprung disease and 2 children (aged 4 and 8 years) with intractable constipation accompanying hemorrhoid or anal fissure. The manometric findings of the USW-positive patients showed a markedly high anal resting pressure and high frequency of slow waves compared to controls, patients with constipation not accompanied by USWs or patients with Hirschsprung disease.

Conclusion

Children with USWs exhibit symptoms mimicking Hirschsprung disease in infants and chronic intractable constipation in older children. In manometric studies of children, more attention should be paid not only to rectoanal reflex, but also USWs.     

Residual fistula after laparoscopically assisted anorectoplasty: is it a rare problem?

Purpose

Although various urologic complications have been reported after abdominoperineal pull-through and posterior sagittal anorectoplasty for the treatment of high-type imperforate anus, reports regarding complications after laparoscopically assisted anorectoplasty (LAARP) are surprisingly rare. Here, we discuss the potential complications of LAARP.

Methods

A retrospective study was conducted of 24 patients treated with LAARP from 2000 to 2006. The clinical and operative records were reviewed. Of the 24 patients, 18 were evaluated postoperatively by screening magnetic resonance imaging (MRI).

Results

The 24 participants are composed of patients with rectoprostatic urethral fistula (n = 15), rectal agenesis (n = 3), rectovesical fistula (n = 2), rectovaginal fistula (n = 2), and cloaca (n = 2). Defecatory function after LAARP was satisfactory. None of the patients had dysuria or urinary infection postoperatively. Cystic formations posterior to the urethra were demonstrated in 9 of the 18 cases examined by MRI. Postoperative voiding cystourethrography failed to demonstrate the lesion in 6 of 9 patients. The types of imperforate anus in this subgroup were rectoprostatic urethral fistula (n = 7), rectovesical fistula (n = 1), and rectal agenesis without fistula (n = 1). Average cyst diameter was 22 ± 19 mm. Two patients with large cysts (62 and 42 mm) underwent surgical resection.

Conclusions

Although satisfactory fecal continence could be achieved by LAARP, we experienced 2 cases with a large residual fistula that required surgical resection. In addition, screening MRI demonstrated the presence of cystic formations in 9 of 18 patients. We recommend that MRI be performed routinely during follow-up of patients treated with LAARP.     

Nuclear transit studies of patients with intractable chronic constipation reveal a subgroup with rapid proximal colonic transit

Aims/Background

Nuclear transit studies (NTS) allow us to follow transit through the stomach and the small and large intestines. We identified children with chronic constipation with rapid proximal colonic transit and characterized their clinical features.

Methods

We reviewed NTS from 1998 to 2009 to identify patients with chronic constipation and rapid proximal colonic transit, defined as greater than 25% of tracer beyond hepatic flexure at 6 hour and/or greater than 25% of tracer beyond end of descending colon at 24 hour. This was correlated with clinical symptoms and outcome from patient records.

Results

Five hundred twenty children with chronic constipation underwent investigation by NTS, and 64 (12%) were identified with rapid proximal colonic transit. The clinical history, symptoms, and outcome in 55 of 64 available for analysis frequently showed family history of allergy (10.9%) and symptoms associated with food allergy/intolerance: abdominal pain (80%), anal fissure (27.3%), and other allergic symptoms (43.6%). Eighteen children were treated with dietary exclusion, with resolution of symptoms in 9 (50%).

Conclusions

Some children with intractable chronic constipation have rapid proximal colonic transit, have symptoms consistent with possible food allergy/intolerance, and may respond to dietary exclusion. The NTS can identify these patients with rapid proximal transit that may be secondary to food intolerance.     

Functional outcome after operation for Hirschsprung disease—transanal vs transabdominal approach

Background

It has been hypothesized that the extensive transanal dissection in transanal endorectal pull-through (TEPT) for Hirschsprung disease (HD) can impair the anal sphincters in neonates and thereby cause incontinence. Theoretically, transabdominal endorectal pull-through might have less impact on the sphincters. The aim of this study was to compare functional outcome in HD patients operated with either TEPT or laparotomy-assisted endorectal pull-through (LEPT) with particular focus on soiling and fecal incontinence.

Patients and Methods

Anorectal function in 52 children older than 3 years is reported. The patients were operated for HD with either TEPT (n = 28) or LEPT (n = 24) and followed prospectively. Functional outcome was recorded by standardized interviews. The Krickenbeck criteria were used to classify voluntary bowel movements, soiling, and constipation.

Results

The median age at follow-up was 5.7 years (3.1-13.2) for TEPT and 10.1 years (7.7-16.2) for LEPT. Twenty-nine patients reported soiling at final follow-up. There was no difference in the rate of soiling between children operated with TEPT (54%) or LEPT (58%). Constipation was reported in 11 children (TEPT, 25%; LEPT, 17%).

Conclusions

The functional outcome and in particular the rate of soiling did not differ between patients operated with LEPT or TEPT.     

Topical mitomycin-C for the treatment of anal stricture

Background

Anal stricture is a well-known and feared consequence of anorectal surgery. Daily dilatations are often prescribed in the immediate postoperative period to avoid stricture of the anus. Nonetheless, stricture may still occur and, particularly in older children, may require multiple dilatations under anesthesia. Topical mitomycin-C has been found to be effective in the treatment of strictures at various anatomical locations. In this article, we review our experience with topical mitomycin-C as an adjunct to anal dilatation for children with anal stricture.

Materials and methods

Cases of children with anal stricture who were treated with a single application of topical mitomycin-C as an adjunct to anal dilatation between 2000 and 2008 were analyzed retrospectively. Anal diameter was measured with Hegar dilators. Cottonoid swabs soaked in mitomycin-C were placed on the anal mucosa for 5 minutes after dilatation. Treatment success was defined by sustained improvement in anal size, decrease in symptoms, parental satisfaction, and need for additional intervention.

Results

Ten children with anal stricture who underwent anal dilatation with application of topical mitomycin-C were identified. All children presented with severe constipation. Average increase in anal size after dilatation under sedation was 5.7 mm (±3.2 mm). Average improvement in anal diameter on first clinic visit after mitomycin-C application was 3.7 mm. On follow-up, only 1 child required repeated intervention for stricture after treatment with mitomycin-C. No complications were associated with the use of mitomycin-C.

Conclusions

All children treated with mitomycin-C showed early improvement in their anal size after dilatation under sedation. A single application of topical mitomycin-C allowed them to retain an increased anal diameter over time and avoid additional dilatations. Furthermore, the application of mitomycin-C in our population was straightforward and safe. Therefore, we advocate its use as an adjunct to anal dilatation under sedation in the treatment of severe anal stricture.     

Transanoproctoplasty: a 21-year review

Background

Transanal anorectoplasty was developed through the 1980s by the senior author (AB) as an alternative approach to posterior sagittal anorectoplasty for the management of imperforate anus. This study evaluates this surgical approach and its longer-term results.

Methods

Case notes of all patients treated from 1984 to 2005 were reviewed. Operative procedures, colostomy requirement, complications, and long-term outcome were recorded. Patients were grouped according to the status of the pelvic floor, the location of the rectal fistula, and the sacral ratio.

Results

A total of 245 patients (175 male, 70 female) underwent transanal anorectoplasty. The perineum was well formed in 208 patients (85%), moderately formed in 15 (6%), and poorly formed in 22 (9%) patients. Two hundred three patients (82.8%) had a visible perineal or vulval fistula, 24 (9.8%) had a prostatic urethral fistula, whereas 18 (7.4%) had a bulbourethral fistula. Overall, 6 patients (2.5%) had wound infection or breakdown. As continence is age related, 32 patients were excluded from the study. Of the remaining 212 patients, 182 are continent with no soiling or only minimal staining. Thirty patients born with a poor perineum are incontinent.

Conclusion

Transanal anorectoplasty is a safe procedure with limited morbidity. It is sphincter sparing and permits accurate placement of the rectum with its internal sphincter within the anal canal. The anus lies accurately placed at the center of the external anal sphincter muscle complex. Given a well-performed surgical intervention, eventual continence relates to the original anatomy and neurology of the pelvic floor. Transanal anorectoplasty should be regarded as an alternative rather than as a substitute to posterior sagittal anorectoplasty for reconstruction of most forms of imperforate anus.     

Resource utilization and its management in splenic trauma

Background

This study compared resource utilization and its management for splenic injury at 2 level-I trauma centers and a pediatric referral center with other facilities in a state currently developing a trauma system.

Methods

Management strategy, length of stay, and total charges for children were compared among the pediatric referral center, trauma centers, and other facilities. Adult management, length of stay, and total charges were compared between trauma centers and other facilities.

Results

Nonoperative management was more frequent in children at the pediatric referral center than trauma centers or other facilities and was more common in adults at trauma centers than at other facilities. Mean length of stay and total charges for children were significantly greater at the pediatric referral center and trauma centers than at other facilities and for adults at trauma centers than at other facilities. Facility type was associated with length of stay and total charges when injury type and severity were controlled.

Conclusions

Nonoperative management of splenic injury is more common at trauma centers, and splenic trauma management may be more costly at trauma centers.     

The influence of Down's syndrome on the management and outcome of children with Hirschsprung's disease

Background

Children with Down’s syndrome (DS) have a reportedly poorer outcome after treatment of Hirschsprung’s disease (HD) compared with control children. Because of overall improvements in their management, the authors hypothesized that the diagnosis of DS would not influence outcome after the management of HD.

Methods

Consecutive children with HD (1995 through 2002) were collected prospectively then divided retrospectively into those with DS and controls (C). Patients who underwent surgery at another institution and those with total colonic aganglionosis were excluded.

Results

Of 66 patients, 9 had DS. Mean age at diagnosis, gender, racial distribution, gestational age, and proximity to our center were similar between groups. Presenting symptoms, location of the transition zone, and type of initial operation were similar. Patients with DS had significantly more comorbidities than controls, which generated significantly greater treatment costs and a higher mortality rate. However, with an average of 22 months of follow-up, the overall outcome including postoperative complications, enterocolitis, and constipation was similar.

Conclusions

These data suggest that in contrast to earlier reports, DS has minimal influence on surgical outcome of patients with HD. Although the overall cost of treating patients with DS is greater, this mainly reflects the impact of managing comorbidities.     

Prevalence of Posttransplantation Lymphoproliferative Disease in Pediatric Liver Transplant Recipients

Objective

The objective of this study was to identify the incidence of posttransplantation lymphoproliferative disease (PTLD) among children within 1 year after liver transplantation.

Methods

This retrospective review analyzed information in medical charts of pediatric (younger than 18 years of age) recipients of liver transplants between September 2000 and December 2007.

Results

Seventy-one patients underwent a liver transplantation and 7 (9.85%) developed PTLD. Among this group, 6 children were girls and 1 was a boy. The median age at transplantation was 35.14 months. Indications that led the children to have their transplantation were 1 case of hemangioendothelioma, 1 case of autoimmune hepatic cirrhosis, 1 case of alpha-1-antitrypsin deficiency, and 4 cases of biliary atresia. The most frequent symptoms were splenomegaly, diarrhea, and fever. The median time from the first symptoms to the initial treatment was 9.7 days. The standard treatment was withdrawal of immunosuppression and close observation of tacrolimus levels and liver function tests associated with antiviral drugs and chemotherapy. Four among 7 children died; 3 children recovered. All 3 children who recovered has presented at the transplantation center within 5 days of initiation of symptoms (P = .033896).

Conclusion

Despite its rarity, when it occurs, PTLD shows a high mortality rate. Therefore, it is necessary to have interdisciplinary work between the medical team that performs the transplantation and those promoting the primary care to diagnose the disease early and treat it effectively.