Sexual problems in male patients older than 20 years with anorectal malformations

Background/Purpose

The nervi erigentes in high- and intermediate-type male anorectal malformation (ARM) runs a relatively medial course and is vulnerable in sacro-perineal dissection. These types of ARM are also associated with a high frequency of sacral anomaly, and sexual problems may be expected. However, sexual function cannot be evaluated until after the individual passes puberty. Few reports have investigated the sexual status of pubescent males with ARM. The present study evaluated sexual problems in patients with high- and intermediate-type ARM.

Methods

Sexual problems such as erectile dysfunction and ejaculatory incompetence were evaluated in 17 of 23 men aged more than 20 years who underwent operation for high- or intermediate-type ARM between September 1974 and January 2005.

Results

Erection angle was normal in 9 patients (52.9%), mild in 6 patients (35.3%), and dysfunctional in 2 patients (11.8%). Ejaculatory function was normal in 10 patients (58.8%), with ejaculatory incompetence in 5 patients (29.4%) and retrograde ejaculation in 2 patients (11.8%). Either erectile or ejaculatory dysfunction was present in 5 patients (29.4%), whereas both were present in 2 patients (11.8%). Sexual problems were identified in 7 patients (41.2%), with sacral anomalies in 5 (71.4%) of these 7 patients.

Conclusions

Sexual problems such as erectile and ejaculatory dysfunction are common in patients with high- or intermediate-type ARM. Patients with sexual distress require persistent follow-up and continuous counselling to support their sexual problems.     

10-Year outcome of children born with anorectal malformation, treated by posterior sagittal anorectoplasty, assessed according to the Krickenbeck classification

Background

Anorectal malformations (ARMs) affect 1 in 4000 to 5000 births. The Krickenbeck conference developed a classification based on anatomical and functional criteria to better compare treatment outcome.

Aim

The aim of this study is to evaluate the functional outcome in patients 10 years following standardized surgical treatment of ARM related to the Krickenbeck classification. .

Methods

Anatomical anomalies were classified as above. Children and carers were followed closely in a multidisciplinary clinic. Data were collected using a functional outcome questionnaire for a minimum of 10 years after surgical reconstruction. Outcome measurements were related to the Krickenbeck classification.

Results

There were 53 children in the study group (29 male, 24 female). Krickenbeck anatomy: perineal fistula, 36%; vestibular fistula, 26%; rectourethral fistula, 36%; rectovesical fistula, 2%.All children were treated by posterior sagittal anorectoplasty.In children with perineal fistula, continence was achieved in 90%. Grade 2 constipation was noted in 21%. One child had a Malone antegrade continence enema (MACE) procedure.In children with vestibular fistula, continence was achieved in 57%. Grade 3 constipation was noted in 28%. One child had grade 1, and one child had grade 2 soiling. Two children had a MACE procedure.In children with rectourethral fistula, continence was achieved in 58%. One child had grade 3 soiling. Grade 3 constipation was found in 42% of children and grade 2 constipation in 1 child. A MACE procedure was performed in 36%.The only child with a bladder neck fistula had a MACE procedure for intractable soiling.

Conclusions

The outcome for patients with ARM is related to the severity of the anomaly. The uniform application of the Krickenbeck classification should allow rational comparison of treatment outcome.     

Laparoscopic treatment of rectovaginal fistulas. Feasibility, technical details, and functional results of a rare anorectal malformation

Introduction

Rectovaginal fistulas are a rare variety of anorectal malformations. Eight patients with this anomaly among 420 children with anorectal malformations were treated in our center. We used a laparoscopic approach in 5 of the children. The aim of this study is to define the feasibility and technical details of laparoscopy in the treatment of these patients as compared with those treated by posterior sagittal anorectoplasty (PSARP) and to evaluate the preliminary results.

Methods

Three patients were treated with a PSARP starting February 2000 (group 1 [GI]). The laparoscopic approach (laparoscopic treatment [LT]) was used in the next 5 girls starting in November 2002 (group 2 [G2]). All patients were operated on by the authors. In LT cases, we used 3 ports. The rectum was dissected laparoscopically, and the fistula was transected. An assisted anorectal pull-through was then performed using a minimal perineal incision. Clinical features, age at operation, associated anomalies, ratio/sacrum (sacral ratio [SR]), distal colostogram, operative findings, complications, urinary continence, voluntary bowel movements, constipation, soiling, and requirements of bowel management program were analyzed. Patients older than 3 years and 6 months and with more than 3 months after colostomy closure were included in the analysis of functional results.

Results

The mean age at the time of operation was 21.8 months. Urogenital anomalies were seen in 6 (75%) of the 8 girls. Two patients had SRs less than 0.6. Only 3 patients in each group were considered for evaluation of functional results. Mean age was 67 months in 3 (G1) and 64 months in 3 (G2) patients. All 6 patients were continent of urine. Only 1 in G1 had an SR less than 0.6. Voluntary bowel movements were present in 1 patient in G1 and 2 in G2. One patient in G1 had severe constipation and grade 2 soiling requiring bowel management program (SR <0.6), and 1 patient in G2 had occasional soiling.

Conclusions

Laparoscopy allowed an optimal view of the pelvis and helped to achieve a low dissection of the fistula. Although the number of patients in this study is few, functional results with LT seem to be comparable to the open PSARP approach when done by experienced pediatric surgeons. The laparoscopic approach may be considered as an option for the treatment of this rare anomaly.     

Long-term clinical outcome in patients with Hirschsprung's disease and associated Down's syndrome

Background/Purpose

Down's syndrome (DS) is the most common chromosomal abnormality associated with Hirschsprung's disease (HD). The purpose of this study was to review the long-term clinical outcome in patients with HD and associated DS.

Methods

Between 1975 and 2003, 39 (15%) of the 259 patients with HD had been associated with DS. Follow-up was carried out by means of examination of patient's records and personal/telephone interviews with the patient's parents or guardians.

Results

Twenty-six (67%) patients presented in the newborn period and 13 (33%) after the neonatal period. Twenty-eight (72%) patients had rectosigmoid HD, 10 long segment, and 1 total colonic aganglionosis. Thirty-two patients had other associated anomalies, 24 of these having cardiac anomalies. Definitive pull-through operation was performed in 33 patients. Parents of 1 child refused surgical intervention and parents of 2 children decided against pull-through operation after colostomy. Three children died before pull through. Thirteen patients had one or more episodes of enterocolitis after pull-through operation. At the time of follow-up (6 months to 28 years), 3 patients were found to have reverted to stoma because of poor bowel control or recurrent enterocolitis. Of the remaining 30 patients, 3 were lost to follow-up and 4 were too young to be assessed for bowel control. Assessment of bowel function in 23 patients revealed normal control in 8 (4 of these soiled for 6-17 years after definitive surgery), soiling in 8, and constipation requiring enemas or laxatives in 7.

Conclusions

The vast majority of patients with HD associated with DS continue to have disturbances of bowel function after definitive pull-through operation.     

Colonic motility and functional assessment of the patients with anorectal malformations according to Krickenbeck consensus

Background/Purpose

In this study, the patients operated on for anorectal malformations (ARM) were evaluated in terms of segmental (SCTT) and total colonic transit times (TCTT) and clinical status according to Krickenbeck consensus before and after treatments.

Methods

Forty-one patients with ARM (28 males/13 females) older than 3 years (median age, 7.7 years; range, 3-25) who had no therapy before were assessed for voluntary bowel movements (VBM), soiling (from 1 to 3), and constipation (from 1 to 3), retrospectively. Distribution of the patients were rectourethral fistula (17), perineal fistula (PF; 8), vestibular fistula (VF; 8), cloaca (3), rectovesical fistula (1), rectovaginal fistula (1), pouch colon with colovestibular fistula (1), no fistula (1), and unknown (1). The patients ingested daily 20 radiopaque markers for 3 days, followed by a single abdominal x-ray on days 4 and 7 if needed. The results were compared with the reference values in the literature.

Results

Mean follow-up period was 36 months (range, 1-108.5 months). All patients but 1 had soiling in different degrees. Twenty-one patients who had VBM were divided into group 1, with constipation (n = 9), and group 2, without constipation (n = 12). The other 19 patients who had no VBM were divided into group 3, with constipation (n = 14), and group 4, without constipation (n = 5). The longest TCTT and rectosigmoid SCTT were found in group 3 (69.5 and 35.2 hours, respectively). Group 1 had long SCTT in rectosigmoid but normal TCTT (27.8 and 47.4 hours, respectively). Groups 2 and 4 had normal SCTT and TCTT, and there was no significant difference between them. After the appropriate treatment, of the patients, 45% (18/40) had no soiling, and the soiling score decreased to grade 1 in 27.5% (11/40) and to grade 2 in 10% (4/40). Four had unchanged soiling score, and 3 were excluded from the study because of follow-up problems. Half of the patients in group 3 (4 VF, 2 rectourethral fistula, PF) gained VBM without soiling after laxative treatment. Only four of 23 patients had decreased constipation score (2 cloaca, PF, VF).

Conclusions

In this study, ARM patients complaining of constipation with or without VBM had prolonged SCTT in the rectosigmoid region. Percentage of the improvement in soiling scores was more conspicuous than that of constipation scores. The dismal figure observed at the first examination in the assessment of VBM was not associated with an unfavorable improvement with laxative treatment. So, it is suggested that assessment of VBM initially may be deceptive for clinical status.     

Is there a role for surgery beyond colonic aganglionosis and anorectal malformations in children with intractable constipation?

Purpose

The aim of this study was to assess benefit of surgery in the treatment of childhood constipation in children without aganglionosis or anorectal malformations.

Methods

Retrospective chart review and follow-up questionnaire of 19 children (10 girls, age 7.6 ± 3.9 years) who underwent surgery after colonic and anorectal manometry had documented abnormal motility. Children at the time of manometric evaluation had symptoms of intractable constipation a mean of 5.1 ± 2.5 years. Follow-up questionnaires were administered to caregivers a mean of 11.4 ± 9.3 months after surgical intervention.

Results

Proximal colonic abnormalities were seen in 2 patients, abnormalities involving the distal colon and rectosigmoid region in 13 patients, pancolonic abnormalities in 2 patients, and incomplete relaxation of the internal anal sphincter in 2 patients. Surgery led to increase in frequency of bowel movements per week (8.1 v 1.9; P < .005), decrease in soiling episodes per week (2.20 v 4.7; P < .01), and decrease in daily use of medications for constipation (0.8 v 2.3; P < .05). Adverse events included skin breakdown at site of ostomy (9%), fecal incontinence after pull-through procedure (13%), and persistent constipation (4.5%). Parents felt that the symptoms after surgery were completely resolved in 89% of patients.

Conclusions

Surgery may be beneficial in the management children with chronic intractable constipation and documented abnormalities in motility.     

Outcome of males with high anorectal malformations treated with laparoscopic-assisted anorectal pull-through: preliminary results of a comparative study with the open approach in a single institution

Objective

The aim of this study was to analyze the outcome of males with HARM treated with a laparoscopic-assisted anorectal pull-through compared with the open posterior sagittal approach in a single institution.

Methods

This study includes 32 patients: 17 (9 with a rectoprostatic fistula [RPF] and 8 with a rectovesical fistula [RVF]) who underwent laparoscopic-assisted anorectal pull-through from October 2001 onward and 15 (8 with an RPF and 7 with an RVF) treated by posterior sagittal approach before that date. Patients were reviewed retrospectively but were operated on by the authors and had longitudinal follow-up. Parameters analyzed included associated anomalies, sacral ratio (SR) index, age at surgery, operative time, complications, presence of voluntary bowel movements, constipation, and soiling. A good outcome was determined by absent or grade 1 soiling and a poor outcome result by soiling grades 2 and 3.

Results

Mean age at surgery was 22 and 37.5 months for patients with RPF and RVF, respectively, in the laparoscopic group and 29.2 and 25.7 months in the open group. Operative time was significantly shorter (P < .0036) for the laparoscopic RVF repair compared with the open approach. In patients with RPF, 50% in the laparoscopic (L) and 37.5% in the open (O) approach had an SR below 0.6. Fifty percent of all patients with RVF had an SR below 0.6, making groups comparable in terms of evaluating bowel function. Four patients were excluded in the analysis of functional results. Voluntary bowel movements with previous defecatory sensation were present in 83.l3% (5/6) in L vs 87.5% (7/8) in O patients with RPF and 62.5% (5/8) L vs 50% (3/6) in O patients with RVF. Grade 1 soiling was present in 50% (3/6) vs 62.5% (5/8) of patients with RPF and 37.5% (3/8) vs 16% (1/6) of patients with RVF in the L and O groups, respectively. Soiling grade 2 or 3 was present in 50% (3/6) vs 12.5% (1/8) of patients with RPF and 37.5% (3/8) vs 50% (3/6) of patients with RVF in the L and O groups, respectively. The risk of poor outcome was 61% in the group with SR lower than 0.6 vs 13% in the group with a higher ratio. By stratifying the groups according to type of surgery or anatomical type, these results were maintained.

Conclusion

The laparoscopic approach is a reasonable surgical option for the management of HARM. Laparoscopic approach was less time consuming in patients with RVF without impairing functional results.     

Redo surgery in Hirschsprung disease: what did we learn? Unicentric experience on 70 patients

Purpose

This article is aimed at describing a series of 70 patients who underwent a redo pull-through in the period between January 1991 and January 2007 and to compare them with a series of consecutive patients with Hirschsprung disease who underwent a single pull-through.

Methods

The notes were reviewed, and a comprehensive interview based on a disease-specific questionnaire for continence and quality of life was submitted to all eligible patients. We used a number of consecutive patients who underwent a single pull-through for Hirschsprung disease as control group.

Results

Seventy patients were included. Residual aganglionosis in pulled-through bowel or retained aganglionic rectum represented the indications to reoperation in most cases. Intestinal obstruction represented the leading symptom in more than 60% of patients. We adopted either the endorectal pull-through, the Duhamel, or the Swenson procedure. Thirty-six patients were assessed for long-term outcome. One third experienced complications. Long-term continence was satisfactory in 70%.Enterocolitis, soiling, and perineal excoriations were complained by 30%. Excellent to good perspectives were described by 94%, and excellent to good cosmetic results by 22%. The only significant difference with control group (109 patients) involved cosmetic appearance, whereas soiling, although more frequent, could not be considered significantly increased.

Conclusions

Patients who experience failure of a pull-through must go through multiple procedures to achieve definitive results. Although overall outcome does not significantly differ from that of a single effective pull-through, great efforts should be made to minimize complications and avoid the need for a redo, which requires experienced surgeons and highly committed families for a longer course of the disease.     

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.     

Is surgery necessary for asymptomatic tethered cord in anorectal malformation patients?

Background

Evidence supporting routine surgery for asymptomatic tethered cord in patients with anorectal malformation (ARM) is, at best, speculative. The authors therefore examined whether untethering is indicated for asymptomatic tethered cord in patients with ARM.

Methods

A retrospective analysis of all patients with ARM (n = 223) between 1992 and 2002 was conducted. During the same period, 435 patients had surgery for tethered cord.

Results

Tethered cord was detected radiologically in 22 (9.8%); 8 patients with a low conus, and 14 with a low conus with and thickened filum. Seven of 22 patients underwent untethering; 3 prophylatic (14%) and 4 for neuro/motor function deficits (18%). All 4 symptomatic patients had significant clinical improvement in their neuro/motor functions after surgery. However, bowel and urinary functions remained unchanged in all 7 patients with a mean follow-up of 6.4 years (range, 4 to 8 years). Fifteen patients with radiologically diagnosed tethered cord remain asymptomatic with a mean follow-up of 2.7 years (range, 8 months to 10 years).

Conclusions

Neuro/motor functions clearly improved with surgery in symptomatic patients. However, bowel and urinary functions remained unchanged after surgery. Only 4 ARM patients with tethered cord required surgery, whereas prophylactic surgery appears to have minimal benefit. Expectant conservative approach in the management of asymptomatic tethered cord patient appears to be safe.     

Increased heritability of certain types of anorectal malformations

Purpose

Various lines of evidence point to genetic causes for the diverse spectrum of anorectal malformations (ARMs); we therefore studied patterns of heritability in a large case series.

Methods

We searched our ARM database for all patients having family members with congenital anomalies. This group was analyzed to determine the type of ARM and the specific anomalies in affected family members.

Results

Thirty-nine of 1606 patients (2.4%) had a family member with a congenital anomaly. The associated non-ARM anomalies included sacral masses and gynecologic, hematologic, esophageal, duodenal, renal, and spinal anomalies. Of these, 24 patients (1.4%) had 1 or more family members with an ARM. Among females with a positive family history, 73% of patients had either a vestibular or perineal fistula, compared with only 36% in patients without a family history (P = .0004). Among males, 35% had perineal fistulas compared with only 10% of those without affected family members (P = .0051).

Conclusions

A positive family history in 1.4% is supportive of a strong genetic component to ARM. The risk of having an affected family member is significantly increased in the presence of a vestibular or perineal fistula. These new data allow for more informed counseling of families with an ARM and support the need for further genetic studies.     

Megarectum after surgery for anorectal malformations

Purpose

Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction.

Methods

Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence.

Results

There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies (P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies (P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology.

Conclusions

Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.     

Intestinal transplantation for short bowel syndrome secondary to gastroschisis

Background/Purpose

Gastroschisis is the most frequent cause of pediatric intestinal transplantation. This study reviews our experience of intestinal transplantation secondary to gastroschisis to elucidate those factors affecting the outcome of children with short bowel syndrome.

Methods

A retrospective review was performed for children who underwent intestinal transplantation for gastroschisis at the University of Miami between June 2003 and August 1994.

Results

Thirty-two transplants were performed in 28 children with gastroschisis during the study period. Associated intestinal anomalies were present in 22 infants (atresia [n = 14], volvulus [n = 3], and/or ischemia [n = 16]). Spontaneous prenatal closure of gastroschisis, a rare anomaly associated with bowel atresia and ischemia because of a very small abdominal defect, was seen in 9 patients. Most of the patients had a complicated course and required multiple abdominal surgeries before transplant. Fifteen (53.6%) patients are currently alive at a median follow-up of 23.5 months. Short-term survival rate has significantly improved in recent years.

Conclusions

Patients with complex gastroschisis and intestinal anomalies have a significant risk for progression to short bowel syndrome. Intestinal transplantation can be a lifesaving option and provides a satisfactory outcome for children with short bowel syndrome secondary to gastroschisis.     

Follow-up of anorectal anomalies: the Italian parents' and patients' perspective

Background

Several studies addressed the long-term follow-up of anorectal anomalies (ARM) in relation to clinical issues (eg, continence) and quality of life.However, most of these studies are based upon questionnaires designed by physicians and/or health-care professionals, which may be sources of bias.

Methods

To investigate whether parents of children (patients themselves or older children or adults) who were born with ARM had the perception that they received appropriate care and follow-up, a survey was carried out in Italy, in 2003, among families with children with ARM. A 20-item questionnaire was mailed to 425 patients and parents listed in the AIMAR (Italian association for anorectal malformation) database and was returned by 209 families.The questionnaire covered different aspects of ARM: type of malformations and surgery, associated anomalies, fecal and urinary continence, as well as aspects about information given to the parents and satisfaction of care and follow-up received.

Result

The patients and parents demonstrated a good understanding of distribution of malformations and their anatomical classification; nevertheless, 67% of responders had to travel outside their living area for surgery. Bowel management (BM) was commonly used among subjects; however, a significant percentage of patients using regular enemas were still soiling (58 patients were clean and 116 soiled).Urinary continence problems were mostly found in females with cloaca; nevertheless, 21 male patients reported occasional dribbling of difficult interpretation. Most subjects were provided with a good explanation about their or their child's malformation at time of reconstructive surgery, but the same level of information was missing about functional prognosis later in life when the need of an appropriate psychologic support was also felt.

Conclusions

Patients and parents born with ARM are generally satisfied with the information received and with the short-term postreconstructive follow-up care.At longer follow-up, although more than a quarter of patients are completely clean, there is a significant percentage of subjects who still soil while following a BM program. This is explained by the small number of nurses and BM specialists who are involved in the rehabilitation process and by the lack of appropriate information about functional prognosis that are conveyed to the parents. In this respect, psychologic support in bridging the gap between cure and care may be critical.     

Outcome after aortic valve replacement in octogenarians

Background

The advancing age of the population in the western world and improvements in surgical techniques and postoperative care have resulted in an increasing number of very elderly patients undergoing cardiac operations. Therefore, the aim of this study is to evaluate the surgical outcome in 115 octogenarians after aortic valve replacement.

Methods

We retrospectively identified 115 patients (47 men, 68 women) aged 82.3 ± 2.1 years (mean, 80 to 92 years) who underwent aortic valve replacement alone (71 patients, 62.1%) or in combination with coronary artery bypass grafting (44 patients, 37.9%), between January 1992 and April 2003. These patients had significant severe aortic stenosis with a mean valve area of 0.62 ± 0.15 cm² and a mean gradient of 88.62 ± 24.06 mm Hg.

Results

The in-hospital mortality rate was 8.5%. The late follow-up was 100% complete. Actuarial survival at 1 and 5 years was 86.4% and 69.4%, respectively. Predictors of late mortality were ejection fraction (p < 0.01), preoperative heart failure (p < 0.03), and the type of prosthesis (p < 0.03).

Conclusions

The outcome after aortic valve replacement in octogenarians is excellent; the operative risk is acceptable and the late survival rate is good. Therefore, cardiac surgery should not be withheld on the basis of age alone.     

Associated congenital anomalies in patients with anorectal malformations--a need for developing a uniform practical approach

Background/Purpose

The aim of this study was to compare the incidences of various associated anomalies among subjects with high and low anorectal malformation (ARM), study the coexistence of these anomalies, and compare their frequency with those quoted in the existing literature.

Methods

Ten years of data (from January 1993 through December 2002) on the 416 subjects from our center with anorectal malformations (ARM) were evaluated. The subjects with supra or translevator rectal pouch (radiologic or operative findings) were classified as high ARM and the rest as low ARM. The associated anomalies studied were vertebral, skeletal, renal, cardiac, vesicoureteric reflux (VUR), gastrointestinal, tracheoesophageal fistula, spinal, genital, and miscellaneous. For a detailed comparison, 4 subgroups were made: boys with high ARM, boys with low ARM, girls with high ARM, and girls with low ARM. Statistical methods were used for intergroup comparison.

Results

There were 68% male and 32% female subjects. Overall, 58% of subjects had high ARM (supralevator and translevator). Our attempt to compare the incidences of anomalies in our study population with those in other populations was defeated because of nonuniformity of classification and investigation of these anomalies in different studies. We found a frequent clubbing of anomalies affecting distantly developing anatomic regions in patients with ARM in many studies. The highest incidence of associated anomalies was found among boys with high ARM. The male subjects also had significantly more genital anomalies in association with low ARM and gastrointestinal tract anomalies in association with high ARM. On the other hand, girls had more urologic anomalies with high ARM and VUR with low ARM.

Conclusions

Except for a low incidence of spinal anomalies (8%) and of VUR (1.7%), the incidences of most associated anomalies in our study were comparable with those of the earlier studies. Additionally, the girls with high ARM were observed to have significantly more urologic anomalies compared with the boys with high ARM in contrast to the results of the earlier reports. The existence of anomalies in distantly developing anatomic regions in patients with ARM supports the possibility of a “generalized” insult during embryogenesis rather than a (“localized”) defect. It was observed that the boys with ARM are more likely to suffer morbidity because of frequent occurrence of multiple associated anomalies, and it may be worthwhile to evaluate of the role of sex chromosome in relation to ARM. The authors are also of the view that there is a need for more uniformity in classification of the anomalies and in their diagnostic approach because various reported studies have differed so widely on these aspects that any interstudy comparison is difficult or not feasible.     

Long-term clinical outcome in patients with total colonic aganglionosis: a 31-year review

Background

The purpose of this study is to review the long-term clinical outcome in patients surgically treated for total colonic aganglionosis (TCA).

Methods

The hospital records of 58 patients with TCA from 2 centers were retrospectively analyzed. These patients were then followed up by personal or telephonic interviews using a standardized questionnaire.

Results

Of the 58 patients, 36 were male and 22 were female. Thirty-eight patients presented in the neonatal period. A total of 13 (22.4%) presented with enterocolitis, 43 (74.1%) with intestinal obstruction, and 2 (3.5%) with severe constipation. All patients had disease involving the entire colon up to at least the terminal ileum. Two patients had total intestinal aganglionosis and died after ileostomy. Various pull-through procedures performed in 56 patients included Soave in 16, Swenson in 5, and Duhamel in 35 patients.Postoperatively, 31 (55.4%) patients had enterocolitis. At long-term follow-up (2-31 years), 3 patients had opted for permanent ileostomies because of intractable incontinenece or recurrent enterocolitis. Eleven patients were too young to assess bowel function or unavailable for follow-up. Of the remaining 42 patients, 22 had normal bowel control and 20 were soiling. At 5 years, patients had an average of 5.2 bowel movements per day, which reduced to a mean of 3.4 per day at the age of 15 years.

Conclusion

Patients with TCA continue to have long-term problems with bowel control, although many improve with time.     

Laparoscopy-assisted surgery for prenatally diagnosed small bowel atresia: simple, safe, and virtually scar free

Purpose

The aim of this study was to describe a new technique for the surgical management of prenatally diagnosed small bowel atresia.

Methods

Under general anesthesia, a 5-mm trocar was inserted using an open technique through an intraumbilical incision. The proximal atretic bowel end was identified using laparoscopy and mobilized toward the umbilicus using an additional 3-mm trocar inserted in the left lower quadrant. The umbilical trocar then was removed, and a ring retractor was inserted into the trocar site and used to expand the wound to deliver both atretic bowel ends. The bowel was repaired and returned to the abdomen through the umbilical wound. The umbilical fascia and skin were closed conventionally.

Results

Three patients were reviewed. Two had minimal abdominal distension, and the atretic bowel ends could be identified easily; laparoscopy-assisted surgery was successful. The third case had significant dilatation, and laparotomy was required. Postoperatively, there was minimal abdominal scarring, and the umbilicus was normal in appearance.

Conclusions

Although this experience is limited to 3 patients, this technique is simple, safe, and virtually scar free and can be applied for the treatment of neonates with prenatally diagnosed small bowel atresia, especially if there is minimal abdominal distension at birth.     

Permanent diversion rates after neoadjuvant therapy and coloanal anastomosis for rectal cancer

Background

The aim of this study was to assess the rate of permanent diversion in patients undergoing coloanal anastomosis after neoadjuvant therapy for rectal cancer.

Methods

We performed a retrospective review of patients with rectal cancer who underwent a total mesorectal excision of a tumor within 9 cm of the anal verge.

Results

There were 201 patients who underwent resection with coloanal anastomosis, with a mean follow-up period of 51 months. The average tumor distance from the anal verge was 7 cm (range, 4-9 cm). Neoadjuvant therapy was administrated in 145 patients, 47 had no radiation, and 9 received radiation postoperatively. Thirty-two patients (16%) had long-term complications including incontinence, fistulas, and strictures. Twenty-five patients (12%) had recurrent disease, 16 of these were local recurrence. The total rate of permanent diversion was 29 (14%). Reasons for diversion included local recurrence in 12 patients (6%), complications in 10 patients (5%), and poor function in 7 patients (3%).

Conclusions

Poor bowel function, late complications, and local recurrence all contribute to permanent diversion after a coloanal anastomosis. Neoadjuvant therapy in conjunction with a total mesorectal excision and coloanal anastomosis leads to acceptably low permanent diversion rates in the vast majority of patients.     

Outcome of pediatric patients treated with extracorporeal life support after cardiac surgery

Background

Extracorporeal life support (ECLS) has been used for over two decades in select patients after cardiac surgery. We previously described factors associated with death in this population. We now review our recent experience to reassess factors related to mortality.

Methods

All pediatric patients who received ECLS support within 7 days after surgery between July 1995 and June 2001 were examined to describe clinical features associated with survival. We compared the results with our prior report to assess changes in practice and outcome.

Results

Seventy-four patients were followed. Fifty percent survived to discharge. Hospital survival was not significantly related to patient age, cannulation site, or indication. Thirty-five percent of patients required hemofiltration while on ECLS and were significantly less likely to survive (23% vs 65%). A multivariate analysis combining all children from our prior report with the present cohort revealed that patients who received hemofiltration were five times more likely to die (odds ratio 5.01, 95% confidence interval 2.11-11.88). Children with an adequate two-ventricular repair had lower risk of death (odds ratio 0.42, 95% confidence interval 0.19-0.91) after adjusting for patient age, study period, and hours elapsed before initiation of ECLS after surgery.

Conclusions

Patients with an adequate two-ventricle repair have significantly higher hospital survival, whereas those with single ventricle physiology or need for dialysis have decreased survival.