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.     

Anomalies associated with anorectal malformations according to the Krickenbeck anatomic classification

Purpose

We evaluated the incidence of congenital anomalies associated with anorectal malformations (ARMs) in relation to the anatomic type of ARM as defined by the Krickenbeck classification.

Methods

We reviewed 99 children with ARM in our institution from 2002 to 2011. Data were collected on patient demographics, type of ARM, and associated congenital anomalies, which were categorized according to organ systems. Statistical analysis was performed for comparison between groups using ‘perineal fistula’ as the base group.

Results

There were 62 (63%) male patients. The majority had perineal fistulas (35, 35%). Seventy-seven (78%) had at least one associated malformation. The most frequent malformations seen were genitourinary (28, 28%) and spinal anomalies (26, 26%). Those with rectovesical fistula had the highest proportion of genitourinary malformations (Odds Ratio [OR], 41.3; 95% confidence interval [CI], 4.7–363.4). Those with cloaca (OR, 49.5; 95% CI, 3.4–718.9) and those with rectovestibular fistula (OR, 12.4; 95% CI, 2.3–65.6) were most likely to have major spinal abnormalities, with tethered cord seen in all groups. The rectovestibular group was also most likely to have other associated malformations (OR, 8.6; 95% CI, 2.2–32.8).

Conclusion

More than 75% of children with anorectal malformation have other associated malformations. Genitourinary anomalies are the most common. Major spinal anomalies are seen in all groups, affecting nearly half of those with rectovestibular fistula and those without fistula. The incidence of associated malformations in the rectovestibular group is higher than described in the literature. Thorough systematic evaluation of all infants with ARM should be done regardless of type of ARM.     

Continence after posterior sagittal anorectoplasty for anorectal malformations: comparison of different scores

Purpose

To evaluate bowel function in patients with anorectal malformations (ARM) comparing existing scoring systems.

Bowel symptoms in children with anorectal malformation — a follow-up with a gender and age perspective

Background

Gender specific outcome for children with anorectal malformations (ARM) is rarely reported although it is important for medical care and in parent counseling.

Purpose

To assess bowel function according to the Krickenbeck system in relation to ARM-subtype, gender and age.

Method

All children born with ARM in 1998–2008 and referred to two centers in two different countries were followed up. The bowel function in 50 girls and 71 boys, median age 8 years, was analyzed.

Results

Among those with a perineal fistula, incontinence occurred in 42% of the females and in 10% of the males (p = 0.005) whereas constipation occurred in 62% of the females and 35% of the males (p < 0.001). No bowel symptoms differed between the females with perineal and vestibular fistulas (p > 0.3 for every symptom). Sacral malformations were associated with incontinence only in males with rectourethral fistulas. Constipation among the males differed between the age groups: 58% versus 26% (p = 0.013). Bowel symptoms did not change with age among the females.

Conclusion

Gender differences in outcome for children with ARM must be considered. Males with perineal fistulas had less incontinence and constipation than the females with perineal fistulas. The females with perineal and vestibular fistulas had similar outcomes.     

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.     

Loop colostomies are safe in anorectal malformations

Aim of the study

Divided colostomy (DC) has been recommended in anorectal malformations (ARMs) with previously reported advantages of decreasing overflow into the distal limb and urinary tract infections (UTIs). Skin bridge loop colostomy (LC) is a technically easier alternative without an increase in these complications. We report our institutional experience of LC in ARM.

Spinal dysraphism with anorectal malformation: lumbosacral magnetic resonance imaging evaluation of 120 patients

Purpose

We evaluated the prevalence of spinal dysraphism (SD) in patients with anorectal malformation (ARM) by magnetic resonance imaging (MRI).

Methods

From January 2002 to March 2009, 120 patients with ARM who underwent anorectal reconstruction were evaluated for SD with sacral plain film, spinal ultrasonography (US), and lumbosacral MRI. We adopted Krickenbeck international classification of ARM.

Results

Spinal dysraphism was present in 41 (34.2%) of 120 patients with ARM, 3 (13.0%) of 23 patients with perineal fistula, 7 (29.2%) of 24 patients with vestibular fistula, 4 (36.4%) of 11 patients with rectovesical fistula, 18 (40.9%) of 44 patients with rectourethral fistula, and 9 (60.0%) of 15 patients with cloacal anomaly (P = .04). Among 41 patients having SD detected by MR, 26 patients (26/41; 63.4%) underwent detethering surgery for tethered spinal cord. The mean sacral ratio (SR) in patients who underwent detethering surgery (0.54 ± 0.19) was significantly lower than in patients who did not undergo detethering surgery (0.69 ± 0.13; P < .001). The optimal cutoff for the SR value predicting SD requiring detethering surgery was 0.605, with sensitivity of 65.4% and specificity of 77.7%.

Conclusions

Spinal dysraphism is common in patients with ARM, and its prevalence is higher in patients with complex ARM. Spinal anomalies can occur even with benign types of ARM and, therefore, that all patients should be screened. Magnetic resonance imaging is useful in detecting occult SD that may be missed by conventional radiologic evaluation, physical examination, and spinal US. We further recommend a lumbosacral MRI examination in those whose SR is lower than 0.6.     

Laparoscopy and its use in the repair of anorectal malformations

Introduction

Laparoscopy has been used for the treatment of anorectal malformations (ARMs) in an attempt to be less invasive and with the hope that it would result in a better functional outcome. There remains a significant debate about whether these expectations have been fulfilled.

Methods

Seventeen patients with ARM for whom laparoscopy was used were retrospectively reviewed. Six were operated on primarily by the authors, and 11 cases were referred after a laparoscopic repair performed elsewhere. In addition, a literature review was performed looking for evidence of less invasiveness and improved functional results in patients operated on laparoscopically.

Results

The diagnosis was imperforate anus with a rectobladder neck fistula in our 6 cases with the fistula ligated laparoscopically in each case. In 1 patient, the malformation was repaired entirely using laparoscopic technique. The other 5 patients had a laparoscopically assisted repair because we had to open the abdomen to taper a dilated rectum in 2, mobilize a very high rectum in 2, and take down a distal colostomy stoma in 1. Eleven patients were referred with a variety of problems after a laparoscopic repair done elsewhere for rectal stricture (5), rectal prolapse (4), recurrent rectourethral fistula (3), rectal mislocation (3), failed attempted repair leading to fecal incontinence (1), and a posterior urethral diverticulum (1). Our literature review included 47 references (involving 323 patients) published between 1998 and 2010. All studies showed that laparoscopic repair of ARMs is feasible. The review, however, did not provide evidence of less invasiveness or improved functional results.

Conclusions

Laparoscopy for ARM is a less invasive procedure when compared with those operations that would have previously required a laparotomy (rectobladder neck fistula). In cases of rectoprostatic fistulae, the laparoscopic approach is feasible and avoids a lengthy posterior sagittal incision. There is no evidence that the laparoscopic approach is a less invasive procedure for other types of ARMs. In cases of rectobulbar fistula, congenital anal stenosis, perineal fistula, ARM without fistula, the evidence suggests that it may be lead to more complications. There is no evidence in the literature demonstrating better functional results in cases of ARM operated on laparoscopically.     

Complete VACTERL evaluation is needed in newborns with rectoperineal fistula

Purpose

Given that a rectoperineal fistula is developmentally the most mature lesion in the spectrum of anorectal malformations, it is not clear whether it merits a complete VACTERL evaluation. We sought to determine if the same evaluation is required to rule out associated anomalies in newborns with rectoperineal fistula as those with more complex anorectal malformations.

Methods

We performed a retrospective review of the pediatric colorectal center database at our tertiary care children’s hospital from 2000 to 2012. Patients with anorectal malformations were categorized as rectoperineal fistula or “other” using the Krickenbeck classification. Records were reviewed to identify associated anomalies.

Results

308 patients (156 males) were treated at our institution during the time period (rectoperineal fistula = 102). Thirty-five (34%) patients with a perineal fistula had at least one associated anomaly. The most common anomalies were cardiac lesions (29% excluding PFO and PDA), genitourinary (20.6%), and malformations of the spine (15.7%). The overall occurrence of anomalies was lower than the “other” group.

Conclusion

Our review demonstrates that newborns with a rectoperineal fistula frequently have associated anomalies and should undergo an evaluation similar to more complex lesions. These findings illustrate the importance of a structured approach to the evaluation of even the most straightforward lesions.     

Megarectosigmoid in children with anorectal malformations: Long term outcome after surgical or conservative treatment

Purpose

Megarectosigmoid (MRS) is commonly seen in children with anorectal malformations (ARM) and contributes to the high incidence of constipation. Surgical resection has been advocated by some, whereas others propose intense bowel management as the treatment of choice. The aim of this study was to evaluate outcome of both bowel function and configuration after surgical or conservative treatment of MRS in ARM patients.

Materials and methods

The study included 79 patients with ARM, excluding perineal fistula, (48 boys, 31 girls) from 1986 to 2007. MRS was diagnosed at colostomy formation or contrast enema performed in the neonatal period. Early in the period, the majority of the patients were treated surgically, whereas in the late 1990s, a conservative approach with intensified bowel treatment was implemented. Contrast enema and bowel function investigations were performed repeatedly during follow-up.

Results

MRS, according to radiological criteria, was diagnosed in 26/79 (33%) of the ARM children. Bowel functional outcome was similar regardless of surgical or conservative treatment and comparable to function in ARM children with non-MRS. The radiological signs of rectal dilatation and elongation disappeared after surgical intervention, but normalisation of the rectosigmoidal configuration was also seen with age in the conservative group.

Conclusions

Bowel functional outcome in ARM children with MRS was similar after either surgical or conservative treatment during follow-up. The radiological signs of rectal dilatation and elongation disappeared also in the conservatively treated patients over time.     

Bowel perforation in newborn with anorectal malformation and no fistula at presentation

Purpose

Anorectal malformation (ARM) in newborns with no fistula at presentation resembles intestinal obstruction. The aim of this study is to study the factors associated with bowel perforation in this group of patients.

Methods

From 2000 to 2012, 106 newborns with ARM were managed in our hospital. Thirty neonates without fistula at presentation were included in this study. Demographic data and the incidence of bowel perforation were studied.

Results

Twenty-nine male and 1 female were included in the study. Five patients were born premature and six patients had low birth weight. Six patients had Down's syndrome and 12 patients had associated anomalies. Cross-table lateral x-ray in prone position was performed from 20 to 24 hours after birth. All operations were performed within 48 hours after birth. One neonate underwent primary anoplasty. Twenty-nine neonates underwent colostomy. Two males developed bowel perforation before surgery (at 33 and 36 hours after birth). Perforation was associated with low birth weight (p = 0.034) and was not associated with prematurity (p = 0.31), Down's syndrome (p = 0.634) or the presence of other associated anomalies (p = 0.687).

Conclusions

In newborns with ARM, bowel perforation can occur within 36 hours after birth. Forty-eight hours of waiting is too long as it risks perforation. In this study, a neonate with low birth weight was trended toward bowel perforation.     

Single-stage repair of vestibular and perineal fistulae without colostomy

Purpose

This retrospective study was undertaken to evaluate the feasibility of primary anorectoplasty without a covering colostomy using the anterior sagittal anorectoplasty (ASARP) or posterior sagittal anorectoplasty (PSARP) technique in patients having vestibular and perineal fistulae, its complications, results, and remote outcome in our institute.

Methods

From January 2000 to June 2007, patients with vestibular and perineal fistulae subjected to single-stage surgical correction at our institute were reviewed retrospectively from the data available in hospital records and follow-up complaints of patients and their parents in the outpatient department. Patients who had undergone a staged repair were excluded from the study. All patients were assessed for immediate and delayed complications including continence of the neorectum.

Results

From January 2000 to June 2007, 123 patients having vestibular (94) and perineal fistulae (29), age range from 28 days to 10 years, were subjected to primary repair either by the ASARP (34) or PSARP (89) technique. Follow-up period ranged from 3 months to 7 years. Mortality was nil. Constipation (25.68%) was the major long-term problem. Incontinence occurred in 1 patient (1.85%), who also had associated sacral agenesis. A total of 98.15% of patients were continent with stool frequency of 1 to 4 per day. Recurrence of fistula (0.81%), anal stenosis (6.76%), mucosal prolapse (2.70%), and anterior migration of the neoanus (1.35%) were the other major problems. Other minor problems like wound infection, superficial wound dehiscence, transient constipation, and diarrhea, etc, were successfully managed by local wound care, antibiotics, laxatives, enema, anal dilatation, and dietary changes.

Conclusion

Primary anorectoplasty either by PSARP or ASARP is feasible in vestibular and perineal fistulae without covering colostomy. Associated sacral agenesis/hypoplasia, redundant rectosigmoid or pouch colon, and wound infections with dehiscence are the major confounding factors affecting overall outcome. Better outcome in terms of continence can be achieved by careful surgical technique and follow-up along with proper toilet training. Complication rate was greater in cases of vestibular fistula than of perineal fistula, regardless of technique used. Some sort of laxatives and enema are often required. Dilatation of the neoanus for varying periods is also needed.     

Long-term results of anterior sagittal anorectoplasty for the treatment of vestibular fistula

Purpose

Vestibular fistula is the commonest anorectal malformation in the female child. This article reports the treatment and long-term follow-up of 1206 patients of vestibular fistula treated by anterior sagittal anorectoplasty (ASARP) in a single center for 38 years.

Material and Methods

All patients of vestibular fistula admitted and operated on at the Department of Pediatric Surgery, King George Medical University (Lucknow, UP India), from 1970 were included in the study; the age ranged from 2 days to 40 years. The diagnosis was made by clinical examination. We differentiated between anovestibular fistula (AVF) and rectovestibular fistula (RVF) in that the latter is a longer narrow fistula closely applied to the posterior wall of the vagina. Preoperative investigations included hemogram and blood glucose. Echocardiography was done in those patients showing a physical sign of cardiac anomaly. All patients were operated on in the lithotomy position by ASARP; this was done without colostomy in 1169 patients. In 6 patients, preliminary colostomy was done because of excessive perineal excoriation, and 31 others had colostomy done elsewhere. The striated muscle complex was delineated by electrostimulation, and anoplasty was performed after anchoring the rectum within the muscle complex. Washing of the perineum after passage of stools with application of povidone-iodine ointment constituted the local care. Intravenous antibiotics were administered for 48 hours and oral antibiotics (including metronidazole) for 5 days. The patient was discharged home by the fifth day.

Results

Follow-up ranges from 3 months to 19 years; uneventful postoperative recovery was seen in 1147 patients. They had normal growth and development, normal appearance of the perineum, and a normal quality of life. Complications were seen in 60 patients (5%) of which 42 had AVF and 18 had RVF. Eight patients had postoperative wound disruption that was minor in 4 and required colostomy in the other 4. Four patients had recurrence of vestibular fistula thus creating an iatrogenic perineal canal; this could be repaired by a second ASARP in 3 patients and required colostomy and PSARP in one child. Anal stenosis was seen in 11 patients; this was treated by dilatation alone in 6 and required posterior Y-V plasty in 5. The rate of complications in RVF was lower than AVF probably because of lesser number of patients; there was no difference in stooling pattern or continence between uncomplicated patient of RVF and AVF; however, fecal staining was seen in all patients undergoing revision surgery for complications.

Conclusions

This experience with ASARP showed a good result in 95% patients in a single-stage procedure. The technical ease and minimal preoperative and postoperative measures make ASARP the procedure of choice for vestibular fistula in females at all ages. Until sufficient experience is gained, it may be safer to operate on patients with RVF under cover of a protective colostomy.     

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.     

Vesicoureteral reflux and febrile urinary tract infections in anorectal malformations: A retrospective review

Background

Multiple studies document a correlation between anorectal malformations (ARMs) and vesicoureteral reflux (VUR), development of urinary tract infections (UTIs), and subsequent renal disease. We aimed to determine which patient characteristics are associated with VUR and UTI in this population.

Methods

A retrospective review of ARM patients at a free-standing children’s hospital from January 1996 to December 2011 was performed. Logistic regression was used to investigate the associations between VUR and UTI and ARM classification and co-morbid diagnoses.

Results

Of 190 patients, 41 (31%) received a diagnosis of VUR. Thirty-one of the 190 patients had at least one febrile UTI (16%). Of these, only 16 (51%) had a diagnosis of VUR. On multivariable logistic regression, the only patient variable associated with VUR was having an ectopic kidney (p = 0.026). Similarly, the presence of GU malformations was the closest variable associated with developing a UTI (p = 0.073).

Conclusions

In ARM patients, VUR as well as UTIs are associated with the presence of GU malformations. Thus, voiding cystourethrogram (VCUG) testing should be pursued when there are other caudal and GU abnormalities, regardless of fistula location. Antibiotic prophylaxis for UTI should be considered in children with ARM and any GU malformation, not only VUR.     

Long term outcomes of laparoscopic-assisted anorectoplasty: A comparison study with posterior sagittal anorectoplasty

Purpose

The aim of this study is to compare the long term outcomes between laparoscopic-assisted anorectoplasty (LAARP) and posterior sagittal anorectoplasty (PSARP) for children with rectobladderneck and rectoprostatic fistula anorectal malformations (ARM).

Methods

Thirty-two ARM children with rectobladderneck and rectoprostatic fistula who underwent LAARP between October 2001 and March 2012 were reviewed. The outcomes were compared with those of 34 ARM children who underwent PSARP between August 1992 and September 2001. The sacral ratio (SR), age at operation, operative time, postoperative hospital stay and complications were evaluated. Bowel functions were assessed using the Krickenbeck classification.

Results

The mean operative time of the LAARP was significantly shorter than that of PSARP group (1.62 ± 0.40 vs 2.13 ± 0.30 h). The postoperative hospital stay was significantly shorter in the LAARP group (5.8 ± 0.65 vs 8.4 ± 0.67 h). The wound infections (11.8% vs 0%) and recurrent fistula (11.8% vs 0%) were more common in PSARP patients. The overall morbidity rate of PSARP group was significantly higher than that of the LAARP group (35.3% vs 12.5%, p < 0.05). However, 7.5% of the LAARP patients developed rectal prolapse. Twenty-four of 32 patients were followed up for more than 3 years in LAARP group. The median follow up period was 7.5 years (range 4–11) in LAARP patients and 15.5 years (range 11–20) in PSARP patients. The rates of voluntary bowel movement, soiling (grade 1, 2 & 3) were similar in both groups. More patients from PSARP group developed grade 2 or 3 constipation (22.5% vs 0%, P < 0.01).

Conclusions

Compared to PSARP, LAARP is a less invasive procedure. The long term functional outcomes after LAARP were equivalent if not better than those of PSARP.     

Surgical Treatment of 31 Complex Traumatic Posterior Urethral Strictures Associated with Urethrorectal Fistulas

Background

Urethrorectal fistulas (URF) in patients with complex posterior urethral strictures are rare and difficult to repair surgically. There is no widely accepted standard approach described in the published literature.

Objective

The aim of this study was to describe the outcomes of various operative approaches for the repair of URFs in patients with complex posterior urethral strictures.

Design, setting, and participants

From January 1985 to December 2007, 31 patients (age: 6–61 yr; mean: 28.4) with URFs secondary to posterior urethral strictures were treated using a perineal or combined abdominal transpubic–perineal approach.

Interventions

A simple perineal approach was used in 4 patients; a transperineal inferior pubectomy approach was used in 18 patients; and a combined transpubic–perineal approach was used in 9 patients. A bulbospongiosus muscle and subcutaneous dartos pedicle flaps were interposed between the repaired rectum and urethra in 22 patients. The combined transpubic–perineal approach used either a gracilis muscle flap (one patient) or a rectus muscle flap (eight patients).

Measurements

Suprapubic catheterisation was used for bladder drainage, and a urethral silicone stent was left indwelling for 4 wk.

Results and limitations

One-stage repair was successful in 4 patients (100%) using the perineal approach, in 16 of 18 patients (88.9%) using the transperineal–inferior pubectomy approach, and in 7 of 9 patients (77.8%) using the transpubic–perineal approach. Recurrent urethral strictures developed in two cases; one patient required regular dilation, and the other patient was treated successfully with tubed perineoscrotal flap urethroplasty. Recurrent URFs developed in two additional patients.

Conclusions

Surgical approaches for the treatment of URFs associated with complex urethral strictures should be based on a number of considerations including the location of the URF, its aetiology, the length of the urethral strictures, and a history of previous unsuccessful repairs. These results demonstrate that the transperineal–inferior pubic approach may be appropriate as a first-line procedure.     

Anorectal malformations with good prognosis: Variables affecting the functional outcome

Backgroud/Purpose

The purpose of this study was to investigate the outcome of patients operated for anorectal malformations (ARMs) with good prognosis.

Methods

Thirty patients underwent clinical evaluation by Rintala score and anorectal manometry recording anal resting pressure (ARP), rectoanal inhibitory reflex (RAIR), and rectal volume (RV). The results were analysed with regard to sex, type of ARM, surgical timing of posterior sagittal anorectoplasty (PSARP), neurospinal cord dysraphism (ND), neonatal colostomy, and institution where they underwent surgery.

Results

6/30 (20%) presented ND despite normal sacrum. 17/30 (57%) patients had a normal Rintala score. ND and neonatal colostomy were significantly associated with a pathologic score (p = 0.0029 and p = 0.0016). Patients with ND had significantly lower ARP compared to patients with normal spine (23.5 ± 7.2 mmHg vs 32 ± 7.9 mmHg, p = 0.023). ARP was significantly lower in patients with neonatal colostomy compared to patients with primary repair (25.22 ± 10.24 mmHg vs 32.57 ± 6.68 mmHg, p = 0.026). RAIR was present in only 2/6 (33%) patients with ND, while in 21/24 (87.5%) without ND (p = 0.015) and in 4/9 (44%) patients with neonatal colostomy, while in 19/21 (90.5%) patients submitted to primary repair (p = 0.014).

Conclusions

Neurospinal cord dysraphism may be present despite normal sacral ratio. From a clinical point of view, patients with good prognosis ARMs are not completely comparable to healthy children. Neurospinal cord dysraphism and neonatal colostomy seem to worsen the clinical and manometric (ARP and RAIR) outcomes of these patients.     

Thoracoscopic repair of oesophageal atresia: Experience of 33 patients from two tertiary referral centres

Background

With advances in minimally invasive surgery, thoracoscopic repair of oesophageal atresia has become popular in many centres worldwide and indeed has been described as the pinnacle of neonatal surgery. Here, we report our experience in two tertiary referral centres.

Methods

Thoracoscopic technique was introduced in 2007. Thus, a retrospective review of all patients diagnosed with oesophageal atresia was carried out. Patients who had thoracoscopic repair were included, and those who had open repair due to co-morbidities were excluded. Patient demographics, operative data, complications, and associated anomalies were noted.

Results

A total of thirty-three patients underwent thoracoscopic repair during the time period. Thirty-one were successfully repaired thoracoscopically. Two patients had conversions due to intra-operative instability. The mean body weight of the neonates was 2.58 kg. The mean operative time was 146 min. Three patients suffered from minor anastomotic leaks, which healed on conservative management. Seven patients had anastomotic strictures, which responded successfully to endoscopic dilatation. Two patients died in the post-operative period due to pneumonia. One patient had a recurrent fistula 3 months after the primary repair, and he subsequently underwent a successful second repair.

Conclusions

In experienced hands, thoracoscopic repair of oesophageal atresia is at least as good as open surgery but with less surgical trauma. Standard of post-operative care contributes significantly to post-operative outcome. Thoracoscopic technique is now our preferred approach.