Quality of life in children and adolescents with anorectal malformation

Purpose

Quality of life (QOL) is an important endpoint in health outcomes research, especially in pediatric chronic conditions. While some studies suggest that patients with ARM report a lower level of physical and psychosocial QOL, as compared to healthy peers, not all research shows this consistently. The aim of this study was to compare the QOL of Italian children and adolescents with ARM to the QOL of a large control sample of healthy peers.

Methods

Parents of 109 children with ARM and 336 schoolchildren, completed the Pediatric Quality of Life Inventory. Analysis of variances were used to compare the groups.

Results

Compared to healthy peers, children with ARM showed lower emotional functioning (F = 8,41; p = .004), social functioning (F = 4,9; p = .027) and school functioning (F = 14,7; p = .000). In the adolescent group, females had a worse QOL in the physical functioning domain (F = 5,01; p = .03) than males, independent of group membership.

Conclusions

While children with ARM show a more impaired QOL compared to their peers, this difference does not emerge in the adolescent group. Our results are consistent with previous findings which hypothesize that while patients with ARM face more difficulties during childhood, by adolescence they may have learned to overcome these difficulties.     

Sexual function in adults with anorectal malformation: psychosocial adaptation. German Network for Congenital Uro-REctal Malformations (CURE-Net)

Purpose

The aim of the German Network for Congenital Uro-REctal Malformations (CURE-Net) is to collect data of affected patients with anorectal malformation (ARM) to investigate molecular causes, clinical implications and psychosocial outcome. The current issue was to examine sexual function and to explore psychosocial adaptation in adults with ARM.

Methods

This qualitative study using narrative inquiry is part of a larger multi-center study of clinical queries and quality of life in patients with ARM. The guided interview focused on analysis of sexual function.

Results

55 adult patients with ARM (23 females, 32 males, median age 23 years, range from 18 to 56 years) were investigated via standardized case-report forms comprising interview, analysis of medical data and personal questionnaires. In the female patients, 8 (35 %) of them lived alone and 15 (65 %) had sexual intercourse. In the male patients, the majority of 20 (69 %) patients lived alone and 13 (45 %) had sexual intercourse. 6 of the females got pregnant, 5 got 2 or more children. 3 of the men induced 2 or more pregnancies and fathered children.

Conclusion

Besides reconstructing the ARM, another main goal is the preservation of sexual function. According to our data, there seems to be a close relationship between psychosocial development and sexual activity.     

Abnormal neural crest innervation in Sox10-Venus mice with all-trans retinoic acid-induced anorectal malformations

Background/purpose

Despite technical advances in the surgical/medical care of anorectal malformation (ARM), persistent unsatisfactory postoperative bowel habit has been attributed to histopathologic abnormalities of the distal rectum/pouch (DRP) and hypoplasia of anal sphincter muscles (ASM). We used Sox10-Venus mice with ARM induced by all-trans retinoic acid (ATRA) to investigate neural crest cell (NCC) innervation in the DRP and ASM.

Method

Pregnant Sox10-Venus mice were administered single doses of 50, 70, or 100 mg/kg of ATRA on embryonic day 8.5 (E8.5) then sacrificed on either E16.5 or E19.5. Bowel specimens comprising the anorectum were examined using fluorescence microscopy without immunohistochemical staining (FMIS). Anti-PGP9.5 was used to delineate ganglion cells and anti-SMA for smooth muscles.

Results

The appropriate dose of ATRA for inducing ARM was 50 mg/kg. Under FMIS, all ARM embryos (n = 5; all high type; 3 male:2 female) had less NCC innervation with thick Venus-positive nerve fibers in the DRP compared with normal embryos (n = 8); there was abnormal NCC innervation in the DRP and absent ASM in ARM mice.

Conclusion

We are the first to delineate abnormal enteric nervous system innervation in the DRP of ARM mice without using immunohistochemical staining techniques thus allowing specimens to be examined without any distortion.     

The influence of anorectal malformations on fertility: a systematic review

Purpose

This systematic review aims to give an overview of available evidence concerning the influence of anorectal malformations (ARM) on fertility.

Methods

We conducted a search in PubMed, EMBASE and Cochrane Library conformed to the PRISMA standards. All studies reporting on fertility and ARM were included.

Results

2,905 studies were identified. Based on title, abstract and full text, nine articles on 429 patients remained to answer the research question. Childbirth rate was the only reported outcome parameter to describe fertility. An overall childbirth rate of 27 % (range 0–57 %) was found. Mean age at time of study ranged from 23 to 35 years. There was no statistical significant difference in childbirth rate between female and male patients, based on seven studies (p = 0.45). Patients with a more complex type of ARM (imperforated anus without fistula, rectourethral bulbar and prostatic fistulas, rectobladderneck fistulas and cloacal malformations) had a lower childbirth rate compared to healthy controls, whereas in patients with a less complex ARM (rectoperineal or rectovestibular fistula) the childbirth rate was similar to healthy controls. Patients with a more complex type of ARM had a significant lower childbirth rate than patients with a less complex type of ARM (18 vs 47 %, respectively) (p = 0.0001). When further dividing these patients by gender, this difference was only seen in female patients (p = 0.04).

Conclusion

In patients with a more complex type of ARM a lower childbirth rate was found compared to healthy controls and patients with a less complex type of ARM. The latter was only seen in female patients. However, conclusions concerning fertility in ARM patients have to be taken with caution due to limited quality of the studies. Further investigation is recommended.     

Esophageal atresia in patients with anorectal malformations

Purpose

The presence of esophageal atresia (EA) in patients with an anorectal malformation (ARM) is well known. The purpose of this work is to find out the most common type of ARM associated to EA and the functional prognostic implication of this association, which has not been described in previous publications.

Methods

We reviewed our database for demographic, functional, and associated anomalies data in our patients with EA and ARM, and then compared them with those of our general series of ARM without esophageal atresia.

Results

Out of 1,995 ARM patients, 167 had a concomitant EA (8.3 %). Prostatic fistula was the most common type of defect in the male EA patients (45.9 %) and cloacas were on the female group (57.9 %). EA patients had worse bowel (47 vs. 67 %) and urinary control (56.6 vs. 79.4 %) when compared to the general series (GS). Functional prognosis was significantly worse in cloacas and in patients subjected to re-operations (p < 0.001). EA patients had a 0.52 average sacral ratio and in the GS was 0.65 (p < 0.001). EA patients had a significantly higher incidence of tethered cord (32.3 vs. 17.6 %), cardiac anomalies (32.3 vs. 22.5 %) including VSD (12.5 vs. 4.5 %), hydronephrosis (36.5 vs. 15.4 %), absent kidney (26.3 vs. 10.5 %), duodenal atresia (7.7 vs. 1.7 %), vertebral anomalies (28.1 vs. 14 %), extremity defects (11.3 vs. 3.1 %), tracheal anomalies (6.5 vs. 0.4 %), and developmental delay (5.9 vs. 1.4 %).

Conclusions

The presence of esophageal atresia in ARM patients has a significant, probably coincidental, impact on bowel and urinary control. This association is also related with worse types of ARM defects and with more severe associated anomalies. This association should increase the awareness on the provider in terms of what to expect on functional prognosis and a throughout search for associated anomalies.     

Impact of the Bowel Management Program on the quality of life in children with fecal incontinence

Purpose

Evaluate the impact of implementing Bowel Management Program (BMP) on quality of life (QoL) in children with fecal incontinence (FI).

Methods

Children 3–12 years referred to the Children’s Treatment Center of FI were included and categorized accordingto the tendency to constipation (dilated colon; group 1) or diarrhea (non-dilated colon; group 2). Evaluations were performed before and after 3 months of BMP implementation. Perceptions from parents and children about QoL were assessed by the instrument PedsQL4.0 and the severity of FI by the fecal incontinence index (FII) of Wexner scale.

Results

43 children (mean age of 7.7 ± 3.1) were studied. 32 (74.4 %) belonged to group 1 and 11 (25.6 %) to group 2. After 3 months of BMP, it was noticed a significant improvement of the FII (14.5 ± 3.7 versus 2.8 ± 2.5; p < 0.001) in both groups. QoL perception by the parents and children increased on PedsQL4.0 evaluation (p < 0.001). There was a medium inverse statistically significant correlation of the FII and the total PedsQL4.0 score among children and parents (r = ?0.47, p = 0.009 and r = ?0, 39, p = 0.03, respectively).

Conclusion

The BMP applied by a multidisciplinary dedicated team significantly improves the QoL of children with FI in the short-term period.

     

Coloboma and anorectal malformations: a rare association with important clinical implications

Purpose

In a patient with an anorectal malformation (ARM), the presence of a coloboma is commonly associated with other serious anomalies.

Methods

Our database and the world literature were reviewed searching for associated defects in patients with ARM and coloboma.

Results

Of 2,482 ARMs in our database, 11 had coloboma (0.4 %): 2 were females. No specific associated type of ARM was identified. Six patients were developmentally delayed. Eight had a cardiac anomaly (3 had TAPVR, 2 VSD, 3 ASD), five required a cardiac operation. Five had a gastrointestinal anomaly (3 malrotation, 1 biliary and 1 duodenal atresia). Six had eye and seven had ear anomalies. Five had a genetic abnormality. In the literature, 71 patients with ARM and coloboma were found: 65 % were females. 24 % died prematurely. 74 % were developmentally delayed. 70 % had a cardiac malformation (35 % had TAPVR, 38 % required an operation). 57 % had gastrointestinal anomalies (31 % malrotation, 31 % biliary atresia, 17 % Hirschsprung disease). Eye and ear anomalies were present in 80 and 97 % of patients, respectively. 81 % had a genetic abnormality.

Conclusions

An ocular inspection in patients born with ARM is crucial. The finding of a coloboma should increase awareness to evaluate for a developmental, cardiologic or gastrointestinal anomaly.     

Computed tomography measurements of the normal and the pathologic cochlea in children

Background

Radiological investigation is frequently undertaken to assess the aetiology of sensorineural hearing loss (SNHL).

Objective

To establish the CT measurements of the normal cochlea in children and to determine radiological criteria correlated with SNHL.

Materials and methods

A retrospective study of temporal bone CT performed in 159 children, age range from 3 days to 16 years between February 1999 and July 2004. A control group (n?=?88) comprised children without SNHL; the SNHL group comprised 71 children. The width of the second turn of the cochlea (CW), the cochlear height (CH), and the width of the bony canal for the cochlear nerve (WCN) were measured on a reference plane containing the modiolus, the posterior semicircular canal, the footplate, and the stapes arch.

Results

Width of the canal measurements ≤1.7 mm or ≥2.5 mm supported the diagnosis of SNHL with a specificity of 97% and 91%, respectively. Cochlear width was found to be significantly smaller in the SNHL group (5.61?±?0.51 mm) than in the control group (5.75?±?0.31 mm, P?<?0.02), a size <5.4 mm being highly suggestive of SNHL with a specificity of 90%. No significant variations of all measurements were found with age.

Conclusion

Appropriate measurements of WCN and CW are highly correlated with SNHL.     

Incidence and risk factors of post-phototherapy neonatal rebound hyperbilirubinemia

Background

To determine the incidence and risk factors of post-phototherapy rebound hyperbilirubinemia because data about bilirubin rebound in neonates are lacking and few studies have concerned this condition.

Methods

A prospective observational study was conducted on 500 neonates with indirect hyperbilirubinemia who were treated according to standard guidelines. Total serum bilirubin (TSB) was measured at 24–36 h after phototherapy; significant bilirubin rebound (SBR) is considered as increasing TSB that needs reinstitution of phototherapy.

Results

A total of 124 (24.9%) neonates developed SBR with TSB increased by 3.4 (2.4–11.2) mg/dL after stopping phototherapy. Multiple logistic regression model revealed the following significant risk factors for rebound: low birth weight (B = 1.3, P < ?0.001, OR 3.5), suspected sepsis (B = 2.5, P < ?0.001, OR 12.6), exposure to intensive phototherapy (B = ?0.83, P = ?0.03, OR 2.3), hemolysis (B = ?1.2, P < ?0.001, OR 3.1), high discharge bilirubin level (B = ?0.3, P = ?0.001, OR 1.3), and short duration of conventional phototherapy (B = ?? 1.2, P < ?0.001, OR 0.3).

Conclusions

SBR should be considered in neonates with hemolysis, low birth weight, suspected sepsis, short duration of conventional phototherapy, exposure to intensive phototherapy, and relatively high discharge TSB. These risk factors should be taken into account when planning post-phototherapy follow-up.

     

Prevalence and Risk Factors for Hepatitis C Virus, Hepatitis B Virus, and Human Immunodeficiency Virus in Transfused Children in Kinshasa

Objective

To determine seroprevalence of hepatitis C virus (HCV), human immunodeficiency virus (HIV), and hepatitis B virus (HBV) and associated factors among transfused children.

Methods

A multicenter cross-sectional study of transfused children aged between 18 mo and 13 y old was conducted in 4 hospitals in Kinshasa. Blood samples were collected for the detection of Hepatitis B surface antigen (HBsAg) and antibodies to HCV, HIV 1and 2.

Results

A total of 177 (47.7 %) boys and 194 (52.3 %) girls participated in the study. The median age was 59.5 mo (Interquartile range IQR?=?60.6). The prevalence rates of HCV, HBV, and HIV infection were 13.5 %, 1.6 %, and 1.3 %, respectively. Frequency of transfusion events were significantly associated with HCV (p?<?0.001) and HIV (p?<?0.05) infections.

Conclusions

HCV infection was by far more frequently identified than HBV and HIV infections among Congolese transfused children. Frequency of transfusion events was the only significant risk factor associated with HCV and HIV infections but not for HBV.     

Knowledge and Awareness about Benefits of Kangaroo Mother Care

Objective

To determine mothers’ prior knowledge of Kangaroo Mother Care (KMC) and awareness about benefits of KMC for preterm babies.

Methods

Mothers of a consecutive sample of 46 preterm babies, eligible for KMC admitted to a teaching hospital, from June through August 2009, were studied to determine the attitude and knowledge about KMC. A structured questionnaire was prepared. Mothers were asked questions to determine their baseline knowledge about KMC. Then each mother was explained about KMC and instructed to do KMC. After one hour of KMC, mothers were asked questions again to know their feelings and difficulties regarding KMC and feasibility of breast feeding during KMC.

Results

Most of the mothers could understand what was explained to them (97.8 %; 95 % CI 88.5–99.9 %) in a single session. Positive feelings like closeness to baby (93.5 %) and sense of goodness (97.8 %) were noted amongst mothers. Though statistically not significant, the proportion of mothers who felt it impracticable to give breast feeding while doing KMC was considerable (39.1 %; 95 % CI 25.1–54.6 %) compared to those who felt no difficulty in breast feeding (60.9 %; 95 % CI 45.4–74.9 %). Practicable duration of KMC is 1, 2 and 12 h as felt by 52 %, 19.6 % and 6.5 % of mothers respectively. All the mothers expressed their willingness to continue KMC at home.

Conclusions

Mothers can understand and implement KMC with simple and clear oral instructions in local language. Positive feelings arise in mothers even with 1 h of KMC. KMC of 24 h is not practicable to almost all of the mothers. There is a need for special emphasis on breast feeding the child while doing the KMC.     

Postoperative complications in adults with anorectal malformation: a need for transition. German Network for Congenital Uro-REctal Malformations (CURE-Net)

Purpose

The aim of the German Network for Congenital Uro-REctal malformations (CURE-Net) is to collect data of affected patients with anorectal malformation (ARM) to investigate molecular causes, clinical implications and psychosocial outcome. The current issue was to examine the transition to adulthood in adults with ARM and to explore condition-related needs and skills required.

Methods

This qualitative study is part of a larger multi-center study of clinical queries and quality of life in patients with ARM. The guided interview focused on the analysis of medical data and personal questionnaires.

Results

Interviews were completed with 55 (23 females, 32 males) participants, age ranging from 18 to 56 years. Twenty-one patients suffered from mucosal prolapse, 18 patients had had megasigmoid/megacolon. Relevant stenosis of the neo-anus occurred in 13 (42 %) males and 4 (18 %) females, permanent neurogenic bladder dysfunction in 10 (32 %) males and 4 (18 %) females, recurrent urinary tract infections in 10 (32 %) males and 13 (59 %) females, latex allergy in 10 (32 %) males and 7 (32 %) females. Thirty-seven (70 %) patients had to be reoperated. Forty-one (75 %) patients needed means of aftercare to achieve social continence.

Conclusion

The study wants to contribute to a better understanding of the challenges of transition for adults with ARM.     

Long-term functional outcome after untethering surgery for a tethered spinal cord in patients with anorectal malformations

Background

Anorectal malformation (ARM) is associated with a tethered spinal cord (TSC). Long-term functional outcome of untethering surgery for TSC in patients with ARM has not been well evaluated.

Methods

Patients aged 7 years and older who underwent repair of ARM and spinal magnetic resonance imaging from January 1995 to December 2008 were reviewed retrospectively. Untethering surgery was performed in all patients who were diagnosed with TSC, regardless of the presence or of neurological symptoms. Clinical symptoms reflecting anorectal, urinary, and lower limb function were compared between patients complicated with TSC (TSC group, n = 17) and those without TSC (non-TSC group, n = 14).

Results

The median age at functional evaluation was 11.7 and 12.9 years in the TSC and non-TSC groups, respectively (p = 0.52). Untethering surgery for TSC was performed at a median age of 1.3 years. Preoperative urinary and lower limb dysfunction, except for vesicoureteral reflux in the TSC group in one patient, was improved after surgical detethering. Current anorectal function was comparable between the groups.

Conclusions

Long-term functional outcome in patients with ARM and TSC undergoing untethering surgery is equivalent to that in those without TSC. Prophylactic surgical detethering for patients with ARM and TSC can be a treatment of choice to maximize neurological functional outcome.

     

Unexpected results of a nationwide, treatment-independent assessment of fecal incontinence in patients with anorectal anomalies

Purpose

To determine the anorectal function in patients with anorectal malformations (ARM) in order to facilitate patient counseling and follow-up.

Methods

Data were collected by the German network for urorectal malformations (CURE-Net) according to the International Krickenbeck consensus. Questionnaires on bowel function and a defecation protocol were completed by the families/patients. The clinical findings were assessed from the patients’ clinical records.

Results

Two hundred and ninety-seven patients with ARM were assessed, 175 patients gave complete data on continence, 52 of them were excluded due to mental retardation, age, and earlier type of pullthrough. Complete continence was found in 27 %, perineal fistula in 40 %, rectourethral/vesical in 10 %, vestibular in 24 %, cloaca in 0 %. Krickenbeck grade 1 soiling: 42 %, grade 2 and 3: 31 %. Forty-nine percent of the incontinent patients practiced bowel management, reaching continence in 19 %. The statement of constipation (67 %) was validated with the last clinical findings, showing coprostasis in 46 %, “Not suffering constipation” was confirmed in 61 % and falsified in 29 %.

Conclusion

ARM patients in Germany, as assessed by independent researchers, show a high rate of fecal incontinence and insufficiently treated constipation. Parents should be counseled accordingly and motivated to engage in consequent follow-up. Intensified efforts in the conservative treatment of constipation and fecal incontinence are crucial to improvement.     

Prevalence and Spectrum of Sexual Abuse Among Adolescents in Kerala,South India

Objective

To study the prevalence and spectrum of sexual abuse among adolescents in Kerala, South India.

Methods

A self- report survey was conducted among adolescents in the 15–19 y age group, studying in the plus one and plus two classes in selected schools.

Results

Of the 1614 respondents (688 boys and 926 girls), 36 % of boys and 35 % of girls had experienced sexual abuse at some point during their lifetime. Most instances were sexual advances while using public transport. Feelings of insecurity and isolation at home, of being disliked by parents and of being depressed were significantly more in adolescents who had experienced sexual abuse, compared to those who had not.

Conclusions

Sexual abuse is widely prevalent and both boys and girls are equally susceptible. There is a need to evolve strategies to protect children from sexual abuse and the programmes should address both boys and girls.     

Model-based iterative reconstruction in pediatric chest CT: assessment of image quality in a prospective study of children with cystic fibrosis

Background

The potential effects of ionizing radiation are of particular concern in children. The model-based iterative reconstruction VEO^TM is a technique commercialized to improve image quality and reduce noise compared with the filtered back-projection (FBP) method.

Objective

To evaluate the potential of VEO^TM on diagnostic image quality and dose reduction in pediatric chest CT examinations.

Materials and methods

Twenty children (mean 11.4 years) with cystic fibrosis underwent either a standard CT or a moderately reduced-dose CT plus a minimum-dose CT performed at 100 kVp. Reduced-dose CT examinations consisted of two consecutive acquisitions: one moderately reduced-dose CT with increased noise index (NI = 70) and one minimum-dose CT at CTDI_vol 0.14 mGy. Standard CTs were reconstructed using the FBP method while low-dose CTs were reconstructed using FBP and VEO. Two senior radiologists evaluated diagnostic image quality independently by scoring anatomical structures using a four-point scale (1 = excellent, 2 = clear, 3 = diminished, 4 = non-diagnostic). Standard deviation (SD) and signal-to-noise ratio (SNR) were also computed.

Results

At moderately reduced doses, VEO images had significantly lower SD (P?<?0.001) and higher SNR (P?<?0.05) in comparison to filtered back-projection images. Further improvements were obtained at minimum-dose CT. The best diagnostic image quality was obtained with VEO at minimum-dose CT for the small structures (subpleural vessels and lung fissures) (P?<?0.001). The potential for dose reduction was dependent on the diagnostic task because of the modification of the image texture produced by this reconstruction.

Conclusions

At minimum-dose CT, VEO enables important dose reduction depending on the clinical indication and makes visible certain small structures that were not perceptible with filtered back-projection.     

Physiological analysis of the effects of rikkunshito on acid and non-acid gastroesophageal reflux using pH-multichannel intraluminal impedance monitoring

Purpose

To clarify the effects of rikkunshito on acid reflux, non-acid reflux, and esophageal clearance in patients with gastroesophageal reflux disease (GERD).

Methods

We enrolled seven patients with vomiting and/or stridor (median 6 years; 1 month–17 years), with a percent total time of esophageal pH <4.0 (reflux index) over 4.0 %. Rikkunshito (TJ-43; Tsumura Co, Tokyo, Japan) was given in three divided doses before meals. We retrospectively investigated its efficacy using pH-multichannel intraluminal impedance before and 7 (6–10) days after starting treatment. Statistical analyses were conducted using Wilcoxon signed-rank test.

Results

In the pH analyses alone, the median number of acid reflux episodes >5 min (14 versus 10, p = 0.046) and median acid-clearance time (184 versus 134 s, p = 0.03) decreased significantly, although median decrease in reflux index did not reach significance (16.0 versus 17.9 %, p = 0.06). In the combined impedance and pH analyses, the median number (36 versus 36, p = 0.03) and median duration (1.9 versus 1.1 %, p = 0.046) of acid reflux decreased significantly; non-acid reflux and bolus clearance time did not change.

Conclusion

Rikkunshito effectively reduced acid reflux, but not esophageal clearance, in patients with GERD.     

Delayed diagnosis of anorectal malformations (ARM): causes and consequences in a resource-constrained environment

Purpose

Delay in diagnosis or referral of patients with an anorectal malformation (ARM) is associated with significant morbidity and mortality. We describe the frequency and consequences of delay in recognition or referral of patients with ARM. Our study aims to determine whether the source healthcare facility affects the timing of diagnosis and to identify modifiable factors that may expedite diagnosis and referral.

Methods

Retrospective study of patients referred to a pediatric surgical service with a newly diagnosed ARM between July 2002 and December 2010. Data retrieved included patient demographics, perinatal history, time of diagnosis, clinical findings, management, and morbidity and mortality. Delay was defined as diagnosis after 24 h of birth or transfer to the tertiary referral centre more than 24 h after diagnosis.

Results

273 patients (186 males, 87 females) were referred during the study period. Delays were seen in 158 (57.9 %) patients with morbidity in 58 (36.7 %, p < 0.001) and mortality in 9 (5.7 %, p = 0.008). Delay was more frequent amongst babies born in community health centres (CHCs) than in hospitals (90.5 vs. 55.1 %, p = 0.002). The median age at diagnosis was 3 days (range 2 days to 5 years).

Conclusion

There is a need for staff education, particularly in CHCs, concerning routine neonatal examination and normal perineal anatomy. Girls, in whom a decompressive fistula is often present, are at particular risk of diagnostic delay. Delay causes increased morbidity and mortality.

     

Embryological and clinical implications of the association between anorectal malformations and spinal dysraphisms

Purpose

To describe the association of anorectal malformation (ARM) and spinal dysraphism (SD) in terms of impact on the management of SD and embryogenetic implications.

Methods

Patients with SD associated with (A) or without (B) ARM were included. The two groups were further divided into operated on (A1/B1) or not (A2/B2) for SD. Groups A and B were compared for type of SD (embryogenetic classification) and prevalence of neurosurgery; Groups A1 vs. A2 for type of ARM (Wingspread classification); Groups A1 vs. B1 for age at neurosurgery, neurophysiology, and clinical symptoms.

Main results

One hundred twenty-one patients with SD, 83 with and 38 without ARM were consecutively treated (1999–2015). Group A was associated only with SDs developing after primary neurulation, corresponding to the period of cloacal septation and organogenesis (p = 0.0007). Untethering surgery was significantly less frequent in Group A (p < 0.0001 and p = 0.04, respectively). Higher ARMs were not associated with increased risk for neurosurgery. No other significant differences were detected.

Conclusions

In our series, ARMs were associated only with SD developing after primary neurulation, suggesting a single insult leading to both SD and the associated ARM. Neurosurgery is indicated less frequently in patients with ARM-associated SD, despite the similar preoperative clinical features.