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Many patients with anorectal malformations (ARM) need a bowel management program (BMP) to manage lifelong problems of fecal incontinence or severe constipation. We aimed to evaluate the sustainability of the results in such a program.A single-institution retrospective review was performed in children with ARM who attended our BMP (2015-2019). Standardized definitions and validated tools were used to assess fecal continence (Baylor Continence Scale), constipation (Cleveland Constipation Scoring System), urinary symptoms (Vancouver Symptoms Score), and the Pediatric Quality of Life (PedsQL) and health-related quality of life (HRQOL) at the start of BMP and 1-year after completion of the program.222 patients with ARM at a median age of 6.7 (IQR, 4.9-10.1) years were identified. All (100%) soiled at intake with 149 (67.1%) patients being treated with rectal or antegrade enemas and 73 (32.9%) with oral laxatives. At 1 year 150 (70.4%) were clean, 72.7% were on enemas and 27.3% were on laxatives (p = 0.08). 109 out of 148 (73.6%) patients were clean on enemas. A further 41 out of 66 (62.1%) patients were continent on laxatives with voluntary bowel movements and clean. In the group that was clean, there was improvement in Baylor Continence Scale (25 vs. 13.0, p 0.000000002), Vancouver (11 vs. 6, p = 0.0110) scores, and clinically relevant improvement in the total PedsQL HRQL (78-85) and the PedsQL HRQL physical function (86-92) and psychosocial domain (77-82). There was no improvement in Cleveland (10 vs. 9, p = 0.31) score.An intensive BMP offers significant benefits in the treatment of fecal incontinence in ARM. It appears to also improve urinary incontinence and urinary voiding as well as the patient's quality of life. These changes are sustainable over at least one year.

Aim of the Study : For the past four decades, routine daily postoperative anal dilation by parents has been the standard treatment following a primary posterior sagittal anorectoplasty (PSARP). However, the clinical benefit of this practice has never been formally investigated. It is known that dilations can have a significant negative psychological impact on patients and families, and therefore, we aimed to study if routine dilations after a PSARP are necessary. Methods : A prospective, single institution randomized controlled clinical trial was conducted on patients with anorectal malformations (ARM) at our institution between 2017 and 2019. Patients were randomized to either a dilation or non-dilation group following their PSARP. Inclusion criteria included age less than 24 months and all patients undergoing primary repair of their ARM (except for cloaca). Patient characteristics, type of ARM, presence of colostomy, postoperative stricture, need for a skin level revision (Heineke-Mikulicz anoplasty (HMA)), and need for redo PSARP were recorded. The primary outcome of the trial was stricture formation. The secondary outcome included strictures requiring interventions. A p-value of less than 0.05 was considered statistically significant. Institutional approval was obtained for this study and informed consents were obtained from all the patients. Results : 49 patients were included in our study. 5 (21%) in the dilation group and 8 (32%) in the non-dilation group developed strictures (p=0.21). Of these, 3 (13%) patients in the dilation group required HMA, and 4 (16%) patients in the non-dilation group required HMA (p=0.72). 4 patients required a redo operation for strictures: 2 in the dilation arm (these patients despite the plan to do dilations, chose not to do them consistently) and 2 in the non-dilation arm (p=0.59). Conclusion : Routine dilations after PSARP do not significantly reduce stricture formation. Based on these results, non-dilation is a viable alternative, and HM anoplasty remains a good back-up plan if a stricture develops. Level of Evidence : Level I

Purpose A complication of the surgical management of anorectal malformations (ARMs) is a retained remnant of the original fistula (ROOF) formerly called a posterior urethral diverticulum (PUD). A ROOF may have multiple presentations or may be incidentally discovered during the work-up of ARM after previous surgery. We sought to define the entity and the surgical indications for excision of a ROOF when found. Methods We performed a retrospective cohort study of all male patients who presented to our center following previous repair for ARM at another institution, who came for evaluation of problems with urinary and/or fecal continence, from 2014 to 2017. Charts were reviewed for symptoms, original type of malformation, preoperative imaging, treatment, and postoperative follow-up. Results Of 180 referred male patients, 16 had a ROOF. 14 underwent surgical repair to address this and for other redo indications, and 2 did not require intervention. 13 patients had an additional reason for a redo such as anal mislocation or rectal prolapse. Indications for ROOF excision were urinary symptoms (e.g. UTI, dribbling, passage of mucous via urethra, stone formation), to make a smoother posterior urethra for intermittent catheterization, or for prophylactic reasons. Patients were repaired at an average age of 4.2 years, using a PSARP only approach with excision of the ROOF for all except one patient who needed a laparotomy due to abdominal extension of the ROOF. No patient needed a colostomy. The original ARM repairs of the patients were PSARP (9), laparoscopic assisted (4) and abdominoperineal pullthrough (3). Preoperative evaluation included pelvic MRI, VCUG, and cystoscopy. The ROOF was visualized on 14 of 16 MRIs, 10 of 14 VCUGs, and 14 of 15 cystoscopies. Urinary symptoms associated with a ROOF and ease of catheterization were improved in all repaired cases. Conclusion Patients not doing well from a urinary or bowel standpoint post ARM pull-through need a complete evaluation which should include a check for a ROOF. Both modalities MRI and cystoscopy are needed as a ROOF can be missed on either alone. A VCUG was not reliable in identifying a ROOF. Excision is needed in patients to improve urinary symptoms associated with these lesions and to minimize the small but theoretical oncologic risk present in a ROOF. Level of evidence Level III.

Background Despite variability at presentation, presacral masses in patients with and without anorectal malformations (ARM) appear histologically similar. The purpose of this study was to identify differences in oncologic outcomes between these two groups. Methods A retrospective review was performed utilizing our institutional cancer and colorectal and pelvic reconstruction databases for patients with presacral masses and sacrococcygeal teratomas between 1990 and 2017. Data captured included age at surgical resection, type of ARM, tumor location within the pelvis, tumor histopathology, tumor size, adjuvant chemotherapy, recurrence, and follow-up. Results Forty-six patients comprised our cohort, of whom 12 had an ARM. The median age was older at resection for those with an ARM (1.4 years; range 1 day to 29.4 years) compared to those without an ARM (9 days; range 0 days to 6.9 years) (p = 0.01). The mean tumor size was 2.5 cm in patients with an ARM compared to 6.0 cm in patients without an ARM (p = 0.036). All patients with ARM had exclusively intrapelvic tumors, and histopathology included mature teratoma (8), yolk sac tumor (1), lipoma (1), and unknown (2). Tumor location for patients with sacral and presacral masses without ARM included exclusively extrapelvic (10), primarily extrapelvic with large intrapelvic component (7), primarily intrapelvic with extrapelvic component (1), exclusively intrapelvic (8), and unknown (8). Histopathology for patients with presacral masses without ARM included mature teratoma (20), immature teratoma (7), yolk sac tumor (3), ganglioneuroma (1), neuroblastoma (1), benign epithelial cyst (1), and unknown (1). Tumor recurrence rate was similar between patients with ARM (n = 3, 25%) and those without an ARM (n = 5, 15%) (p = 0.41). The 5-year event free survival was 65% (95% CI: 25%–87%) in the group with ARM and 81% (95% CI: 60%–92%) in the group without ARM (p = 0.44). Conclusion Sacral and presacral masses in patients with ARM are resected at a later age and are more likely to be intrapelvic. They appear histologically similar and have similar rates of recurrence and malignancy when compared to patients without ARM. Level of Evidence III Type of Study Retrospective comparative study.

Introduction There is a lack of an agreed Hirschsprung-associated enterocolitis (HAEC) definition. In 2009, a HAEC score was proposed for the diagnosis of HAEC episodes. Our aim was to apply the HAEC score on HAEC episodes to determine its diagnostic efficiency and whether it correlated to its severity. Methods Retrospective study of patients with HAEC admitted between 2000 and 2016. Episodes of HAEC were identified and the HAEC score was calculated. A cut-off of ≥ 10 according to Pastor et al and ≥ 4 according to Frykman et al were used. A Pearson's correlation coefficient was performed for outcome variable: length-of-stay (LOS). Results Note that 21/93 (22.6%) patients with Hirschsprung's disease presented 50 HAEC episodes with a median of 2 (1–5) episodes during an 8.3-year (2–15.6) follow-up. The most common symptoms were foul-smelling (86% [43/50]) and explosive (60% [30/50]) diarrhea. Physical findings showed a distended abdomen (76% [38/50]) and fever (60% [30/50]) with dilated bowel (82% [41/50]) and rectosigmoid cut-off (80% [40/50]) identified on X-rays. Only 34% (17/50) showed abnormal laboratory findings. Patients were admitted with a median LOS of 7 days (1–28). A HAEC score of 9 (2–16) was found, and only 50% (25/50) of patients met the initial criteria (score of 10 points). However, the use of a 4-point cut-off would have allowed the diagnosis of 98% (49/50) of the patients. A positive linear correlation was found for LOS and HAEC score (r = 0.3, p = 0.014). Conclusion There is no standardized definition for HAEC. The initial HAEC score cut-off is restrictive and might fail to identify milder episodes. The positive correlation with LOS and thus HAEC severity might aid in patient information and anticipation of treatment.

Introduction Acquired skin-level strictures following posterior sagittal anorectoplasty (PSARP) and some rare cases of congenital anal stenosis can be managed using a Heineke–Mikulicz like anoplasty (HMA). We hypothesized that this procedure was an effective, safe, and durable outpatient procedure in select patients. Methods We retrospectively reviewed all patients who underwent HMA for skin level strictures following PSARP or for certain congenital anal stenoses from 2014 to 2017. Results Twenty-eight patients (19 males, 9 females) with a mean age of 5.8 years (range 0.5–24.4) underwent HMA. Twenty-six had a prior PSARP, of which 18 were redo, and 8 were primary procedures. Two patients had congenital skin level anal stenosis. The mean follow up was 1.0 years (range 0.4–2.9). The average preprocedure anal size was Hegar 8, which after HMA increased 8 Hegar sizes to 16 (95% CI 7–9, p Conclusion HMA is a safe procedure for skin-level anal strictures following PSARP (primary and redo) and can also be used in some rare cases of congenital anal stenosis. Long-term follow up to determine the restricture rate is ongoing. A plan to do an HMA if a stricture develops may offer an alternative to routine anal dilations, particularly after a redo PSARP in an older child. Type of study Case series. Level of evidence Level IV.

Patients with neurogenic bladder frequently also have bowel dysfunction and a simultaneous urologic and colorectal reconstruction is possible. We present our experience with combined reconstructive procedures using robot-assisted laparoscopy, and demonstrate the utility of a minimally invasive approach that considers both the bowel and bladder management of these patients.We retrospectively reviewed all patients who underwent combined bowel and urologic reconstruction at our institution since the start of our multidisciplinary robotic program.Seven patients were identified in our cohort with a mean age of 6.4 years (3.8-10.1 years). Six patients had myelomeningocele and 1 had caudal regression. Malone appendicostomies were placed in all 7 patients. A split appendix technique was used as a conduit in 5 patients, in situ appendix in 1, and neoappendicostomy with cecal flap in 1. Six patients had a Mitrofanoff appendiceal conduit created, while 1 patient had a sigmoid colovesicostomy for urinary diversion. Five patients required bladder neck repair. One patient had stenosis of the Mitrofanoff and one patient had an anastomotic leak of the sigmoid anastomosis. The average operating time was 526 minutes (313-724 minutes). The median length of stay (LOS) was 5 days (4-7 days), excluding one outlier who suffered an anastomotic leak and had an extended LOS (50 days). All patients who underwent continent bladder reconstruction are dry on their current catheterizing regimen, 6/7 are clean with antegrade flushes.Patients with neurogenic bladder often have coexisting bowel dysfunction, which provides an opportunity to reconstruct both organ systems simultaneously and achieve social urinary and bowel continence. Before committing to any intervention, the surgeon should consider both the urologic and gastrointestinal needs of the patient, and perform the needed procedures simultaneously. We describe a number of combined operations aimed at bowel and bladder management that can be performed safely using robot-assisted laparoscopy.

Purpose Interactive courses play an important role in meeting the educational needs of pediatric surgical trainees. We investigated the impact of a multimodal pediatric colorectal and pelvic reconstruction course on pediatric surgery trainees. Methods A retrospective evaluation was performed of pre- and post-course surveys for an annual colorectal and pelvic reconstruction course over 3 consecutive years (2017–2019). The course included didactic and case-based content, interactive questions, video, and live case demonstration, and a hands-on lab. Pre- and post-course surveys were distributed to participants. Comfort with operative/case procedures was scored on a 5-point Likert scale (1 uncomfortable, 5 very comfortable). The primary outcome was improved confidence and content knowledge for pediatric colorectal surgical conditions. Results 165 pediatric surgical fellow participants with a 70 responses (42.4% response rate) comprised the cohort. Participants had limited advanced pediatric colorectal experience. At the time of the course, participants reported a median of 5 [ 3 , 10 ] Hirschsprung pull-throughs, 6 [ 3 , 10 ] anorectal malformation, and 1 [0,1] cloaca cases. Participants transitioned from discomfort to feeling comfortable with pediatric colorectal operative set-up and case management (pre-course 2 [ 2 , 3 ] and post-course 4 [ 4 , 5 ] p Conclusion Pediatric surgery trainees report limited exposure to advanced pediatric colorectal and pelvic reconstruction cases and management during their pediatric surgical fellowship training but report improved content knowledge- and technical understanding of complex pediatric disorders upon completion of a dedicated course. The course is an important adjunct to the experience gained in pediatric surgery fellowship for achieving competency in managing patients with Hirschsprung disease, anorectal malformation, and cloacal reconstructions.

Chronic idiopathic constipation, also known as functional constipation, is defined as difficult and infrequent defecation without an identifiable organic cause. Medical management with laxatives is effective for the majority of constipated children. However there is a subset of patients who may need evaluation by a surgeon. As constipation progresses, it can lead to fecal retention and rectal and sigmoid distension, which impairs normal colorectal motility. Surgical interventions are influenced by the results of: a rectal biopsy, transit studies, the presence of megacolon/megarectum on contrast enema, the degree of soiling/incontinence, anorectal manometry findings, and colonic motility evaluation. In this review, we describe the different surgical options available (intestinal diversion, antegrade enemas, sacral nerve stimulation, colonic resections, and Botulinum toxin injection) and provide guidance on how to choose the best procedure for a given patient.

Patients with anorectal malformations (ARM) commonly have associated urologic anomalies. Few large studies exist to accurately characterize the incidence or associations between severity of malformation and urologic diagnosis. The purpose of our study was to determine the incidence of urologic diagnoses in a large cohort of children with ARM and evaluate for any correlation between severity of ARM and the incidence and number of associated urologic diagnoses.A retrospective review was performed of patients with ARM treated at our pediatric colorectal center. All patients underwent protocolized urologic screening. ARM subtypes were ordered with increasing severity as follows in males: perineal, bulbar, prostatic and bladder neck fistulae. Females were similarly categorized as perineal, vestibular and vaginal fistulae followed by cloaca with3 cm common channel and cloaca with3 cm common channel. The following urologic diagnoses were assessed to determine whether a correlation existed with the severity of the ARM subtype: hydronephrosis, vesicoureteral reflux (VUR), solitary kidney, renal ascent anomalies (ectopic or pelvic), renal fusion anomalies (horseshoe or cross fused kidney), duplex kidney, hypospadias and undescended testicle. ARM subtypes were defined by distal rectal anatomy.A total of 712 patients were included in our study with a mean age of 4 years and of whom 45% were male. The overall rate of urologic anomalies was greater in males than females (65% vs 56% p0.026). In both sexes, the rate of urologic anomalies increased with increasing severity of ARM subtype (p0.00010) finding that males with bladder neck fistula and females with cloacal malformations, particularly with long common channels, being the highest incidence. In males and females, the rate of hydronephrosis increased as the complexity of ARM increased and this correlated significantly (p0.0001 vs p0.0003 respectively). Similarly, the incidence of VUR also increased as complexity of ARM increased in both males and females (p = 0.01 and p0.0001 respectively). The remaining urologic diagnoses were not significantly correlated with severity of ARM.Urologic anomalies occur at a high rate in children with ARM and appear to increase in frequency with increasing complexity of ARM subtype. These findings stress the importance of proper ARM screening and proactive collaboration with a clinician with expertise in pediatric urology early in the management of such children to improve early recognition of urologic diagnoses.Level III.

Your colleague asks for advice about a 1-month-old term baby boy who has presented with distention and failure to thrive. He is otherwise well, and having excluded an anorectal malformation, washouts were initially successful. A rectal biopsy was obtained and the results have caused a fair amount of discussion; although no ganglion cells were seen, the size of the nerve fibers was described as “normal,” all less than 40 μm. Since the baby was doing well, the diagnosis of Hirschsprung disease seemed unclear.

Published health-care costs related to constipation in children in the USA are estimated at $3.9 billion/year. We sought to assess the effect of a bowel management program (BMP) on health-care utilization and costs.At two collaborating centers, BMP involves an outpatient week during which a treatment plan is implemented and objective assessment of stool burden is performed with daily radiography. We reviewed all patients with severe functional constipation who participated in the program from March 2011 to June 2015 in center 1 and from April 2014 to April 2016 in center 2. ED visits, hospital admissions, and constipation-related morbidities (abdominal pain, fecal impaction, urinary retention, urinary tract infections) 12 months before and 12 months after completion of the BMP were recorded.One hundred eighty-four patients were included (center 1 = 96, center 2 = 88). Sixty-three (34.2%) patients had at least one unplanned visit to the ED before treatment. ED visits decreased to 23 (12.5%) or by 64% (p 0.0005). Unplanned hospital admissions decreased from 65 to 28, i.e., a 56.9% reduction (p 0.0005).In children with severe functional constipation, a structured BMP decreases unplanned visits to the ED, hospital admissions, and costs for constipation-related health care.3.

Introduction Reconstructive techniques for cloaca, anorectal malformations (ARM), and Hirschsprung disease (HD) may require intestinal flaps on vascular pedicles for vaginal reconstruction and/or colonic pull-throughs. Visual assessment of tissue perfusion is typically the only modality used. We investigated the utility of intraoperative indocyanine green fluorescence angiography (ICG-FA) and hypothesized that it would be more accurate than the surgeon's eye. Materials and Methods Thirteen consecutive patients undergoing cloacal reconstruction (9), HD (3), and ARM repair (1) underwent ICG-FA laser SPY imaging to assess colonic, rectal, vaginal, and neovaginal tissue perfusion following intraoperative visual clinical assessment. Operative findings were correlated with healing at 6 weeks, 3 months, and 1 year postoperatively. Results ICG-FA resulted in a change in the operative plan in 4 of the 13 (31%) cases. In three cases, ICG-FA resulted in the distal bowel being transected at a level (>10 cm) higher than originally planned, and in one case the distal bowel was discarded, and the colostomy used for pull-through. Conclusion ICG-FA correctly identified patients who might have developed a complication from poor tissue perfusion. Employing this technology to assess rectal or neovaginal pull-throughs in cloacal reconstructions, complex HD, and ARM cases may be a valuable technology.

Aim Hepatoblastoma is the most frequent hepatic tumor in children, and its initial presentation will affect treatment and prognosis. The aim of this study is to evaluate long-term results of liver transplantation in children with unresectable hepatoblastoma. Patients and Methods This is a retrospective review of patients with hepatoblastoma who underwent liver transplantation, analyzing risk factors, tumor presentation, treatment, and long-term survival to identify prognostic factors. Results Thirty-one patients underwent liver transplantation in the context of unresectable hepatoblastoma, mainly males (67%) and with risk factors such as prematurity (12.9%), maternal smoking (6.5%), and familial adenomatous polyposis (3.2%). Most frequent presentation was multifocal PRETEXT III (51.6%) and PRETEXT IV (45.2%), with metastasis at diagnosis in 12.9% and vascular involvement in 54.8%.Twenty-one patients received a living-donor (67.7%) and 10 a cadaveric graft (32.2%), at 31.7 months of age (5–125). Most transplants were primary, and only two were performed as rescue therapy after an attempt of surgical resection of the tumor.Overall survival 1 and 5 years after transplantation were 93.3% ± 4.6% and 86.4% ± 6.3%, respectively. We could not find any statistically significant differences between risk factors, tumor presentation, type of graft, or type of transplant. Conclusion Liver transplantation has increased hepatoblastoma survival in unresectable tumors. Probably due to these good results, we have not been able to find significant prognostic factors in this cohort.

Introduction Previous research in children with Hirschsprung's disease (HD) and Down's syndrome (DS) has focused on colorectal outcomes. We set out to review urinary outcomes in this patient group. Materials and Methods The medical records of all patients aged five years and older with HD were reviewed, and patients and caregivers filled out the Vancouver Symptom Score at intake, which is designed and validated to diagnose dysfunctional elimination syndrome. Results A total of 104 patients with HD were included in this study. Of these, 16 (15%) patients had DS. There were no significant differences in the prevalence of enterocolitis or colorectal symptoms between patients with or without DS. Five of 88 (6%) patients without DS and 7 of 16 (44%) (p = 0.00001) with DS reported having urinary accidents. Patients with HD and DS scored higher on the Vancouver score (9 vs. 17.5; p = 0.007), indicating more severe urinary symptoms. Patients who also reported fecal accidents scored significantly higher on the Vancouver (12 vs. 9; n = 61; p = 0.016), indicating more problems. Conclusion Patients with DS appear to be a unique subset of HD patients who have a higher prevalence of urinary symptoms after surgery. In the postoperative care of patients with HD and DS, a strong focus should be placed on postoperative urinary care in addition to their bowel care. This could significantly ease care and contribute to the quality of life of the parents and the patient.

Resumen: Introducción y objetivos: El tratamiento quirúrgico estándar del tumor testicular es la orquiectomía, sin embargo, se podría recurrir a la cirugía conservadora en casos seleccionados, basándonos en la edad del paciente, marcadores tumorales, tamaño tumoral y hallazgos histopatológicos. Nuestro objetivo es dar a conocer cuáles son las variables que tener en cuenta para indicar una cirugía conservadora como tratamiento de una masa testicular palpable y no palpable encontrada como hallazgo incidental. Material y métodos: Estudio retrospectivo en 22 pacientes menores de 18 años, diagnosticados de tumor testicular entre 2000 y 2014. Revisamos el motivo de consulta, antecedentes, ecografía, estudio histopatológico, marcadores tumorales (BHCG, AFP), actitud terapéutica y evolución. Resultados: De los 22 pacientes (10 prepuberales), el 82% presentaron masa palpable y el 18% fueron hallazgos incidentales. Dos presentaban criptorquidia. La BHCG estaba aumentada en el 27% y la AFP en el 45%. Se realizaron 18 orquiectomías y 4 tumorectomías. La histología fue en un 72% de células germinales, 14 orquiectomías y 2 tumorectomías (2 teratomas); y en un 27% de tumores de células no germinales, en 4 orquiectomías y 2 tumorectomías (2 tumores de células de Leyding). Seis pacientes recibieron quimioterapia postoperatoria (tumores mixtos). La mediana del tamaño de la tumoración fue de un cm (0,4-1,5) en las tumorectomías y de 2,5 cm (0,5-14) en las orquiectomías. El seguimiento fue de 5 años (1-15). Un paciente falleció por enfermedad metastásica. No hubo recidiva local en la evolución de las tumorectomías. Conclusiones: Ponemos de manifiesto una tendencia al cambio en nuestra actitud terapéutica. Planteamos una cirugía conservadora mediante tumorectomía en los pacientes que cumplan con los criterios de benignidad de la masa testicular (pequeño tamaño y marcadores tumorales negativos). Abstract: Introduction and objectives: Although standard surgical treatment of a testicular tumour is orchiectomy, use can be made of testis-sparing surgery in selected cases, based on tumour markers, tumour size, and histopathological findings. Our objective is to become acquainted with the indications of testis-sparing surgery as a treatment for the incidental finding of a palpable and non-palpable testicular mass. Material and methods: A retrospective study was conducted on 22 patients younger than 18 years diagnosed with a testicular tumour between 2000 and 2014. An assessment was made of the condition, the history, ultrasound, histopathology, tumour markers (BHCG, AFP), therapeutic approach, and outcome. Results: Of the 22 patients (10 prepubertal age) studied, 82% had palpable mass, and 18% were incidental findings. Two had cryptorchidism. The BHCG was increased in 27% and AFP in 45% of cases. There were 18 tumorectomies and 4 orchiectomies performed. The histopathology found 72% germ cell, 14 orchiectomy, and 2 tumorectomies (2 teratomas), with 27% non-germ cell tumours in 4 orchiectomies and 2 tumorectomies (2 cells of Leydig). Six patients received post-surgical chemotherapy (mixed tumours). The median tumour size was 1 (0.4-1.5) cm in tumorectomies, and 2.5 (0.5-14) cm in orchiectomies. The mean follow-up was 5 (1-15) years. One patient died due to metastatic disease. There was no local recurrence in the follow up of the tumorectomies. Conclusions: A change in the trend of our therapeutic approach is demonstrated. We propose that testis-sparing surgery is indicated in prepubertal patients who meet the benignity criteria of the testicular mass (small size and negative tumour markers).

Introduction and objectives: Although standard surgical treatment of a testicular tumour is orchiectomy, use can be made of testis-sparing surgery in selected cases, based on tumour markers, tumour size, and histopathological findings. Our objective is to become acquainted with the indications of testis-sparing surgery as a treatment for the incidental finding of a palpable and non-palpable testicular mass. Material and methods: A retrospective study was conducted on 22 patients younger than 18 years diagnosed with a testicular tumour between 2000 and 2014. An assessment was made of the condition, the history, ultrasound, histopathology, tumour markers (BHCG, AFP), therapeutic approach, and outcome. Results: Of the 22 patients (10 prepubertal age) studied, 82% had palpable mass, and 18% were incidental findings. Two had cryptorchidism. The BHCG was increased in 27% and AFP in 45% of cases. There were 18 tumorectomies and 4 orchiectomies performed. The histopathology found 72% germ cell, 14 orchiectomy, and 2 tumorectomies (2 teratomas), with 27% non-germ cell tumours in 4 orchiectomies and 2 tumorectomies (2 cells of Leydig). Six patients received post-surgical chemotherapy (mixed tumours). The median tumour size was 1 (0.4–1.5) cm in tumorectomies, and 2.5 (0.5–14) cm in orchiectomies. The mean follow-up was 5 (1–15) years. One patient died due to metastatic disease. There was no local recurrence in the follow up of the tumorectomies. Conclusions: A change in the trend of our therapeutic approach is demonstrated. We propose that testis-sparing surgery is indicated in prepubertal patients who meet the benignity criteria of the testicular mass (small size and negative tumour markers). Resumen: Introducción y objetivos: El tratamiento quirúrgico estándar del tumor testicular es la orquiectomía, sin embargo, se podría recurrir a la cirugía conservadora en casos seleccionados, basándonos en la edad del paciente, marcadores tumorales, tamaño tumoral y hallazgos histopatológicos. Nuestro objetivo es dar a conocer cuáles son las variables que tener en cuenta para indicar una cirugía conservadora como tratamiento de una masa testicular palpable y no palpable encontrada como hallazgo incidental. Material y métodos: Estudio retrospectivo en 22 pacientes menores de 18 años, diagnosticados de tumor testicular entre 2000 y 2014. Revisamos el motivo de consulta, antecedentes, ecografía, estudio histopatológico, marcadores tumorales (BHCG, AFP), actitud terapéutica y evolución. Resultados: De los 22 pacientes (10 prepuberales), el 82% presentaron masa palpable y el 18% fueron hallazgos incidentales. Dos presentaban criptorquidia. La BHCG estaba aumentada en el 27% y la AFP en el 45%. Se realizaron 18 orquiectomías y 4 tumorectomías. La histología fue en un 72% de células germinales, 14 orquiectomías y 2 tumorectomías (2 teratomas); y en un 27% de tumores de células no germinales, en 4 orquiectomías y 2 tumorectomías (2 tumores de células de Leyding). Seis pacientes recibieron quimioterapia postoperatoria (tumores mixtos). La mediana del tamaño de la tumoración fue de un cm (0,4-1,5) en las tumorectomías y de 2,5 cm (0,5-14) en las orquiectomías. El seguimiento fue de 5 años (1-15). Un paciente falleció por enfermedad metastásica. No hubo recidiva local en la evolución de las tumorectomías. Conclusiones: Ponemos de manifiesto una tendencia al cambio en nuestra actitud terapéutica. Planteamos una cirugía conservadora mediante tumorectomía en los pacientes que cumplan con los criterios de benignidad de la masa testicular (pequeño tamaño y marcadores tumorales negativos).

Introduction Minimally invasive repair for pectus excavatum (MIRPE) is controversial in extremely severe cases of pectus excavatum (PE) and an open repair is usually favored. Our aim is to describe a case of a patient with an extremely severe PE that underwent a minimally invasive approach. Case report An 8-year-old girl with severe sternum depression was assessed. She had a history of exercise intolerance, nocturnal dyspnea, fatigue, and shortness of breath. Chest computed tomography showed that sternum depression was posterior to the anterior vertebral column; therefore, Haller and correction index could not be measured. Spirometry indicated an obstructive ventilation pattern (forced expiratory volume in 1 second = 74.4%), and echocardiogram revealed a dilated inferior vena cava, mitral valve prolapse with normal ventricular function. After multidisciplinary committee evaluation, a MIRPE approach was performed.All symptoms had disappeared at the 3-month postoperative follow-up; the desired sternum shape was achieved and normalization of cardiopulmonary function was observed. The Nuss bars were removed after a 2-year period. After 18-month follow-up, the patient can carry out normal exercise and is content with the cosmetic result. Conclusion Nuss procedure is feasible in our 8-year-old patient. In this case, both the Haller and correction index were not useful to assess the severity of PE. Therefore, under these circumstances, other radiologic parameters have to be taken into consideration for patient evaluation.

Introduction Gastrointestinal (GI) operations represent a significant proportion of the surgical site infection (SSI) burden in pediatric patients, resulting in significant morbidity. We have previously demonstrated that a GI bundle decreases SSI rates, length of stay (LOS), and hospital charges. Following this success, we hypothesized that by targeting the preoperative antibiotics for stoma closures based on organisms found in infected wounds, we could further decrease SSI rates. Methods As part of a broad quality improvement effort to reduce SSI rates, we reviewed the responsible pathogens and their sensitivities as well as the preoperative antibiotic used, and found that 15% of wound infections were caused by enterococcus. Based on this information, starting in April 2017, we changed the prior preoperative antibiotic cefoxitin to ampicillin-sulbactam, which more accurately targeted the prevalent pathogens from April 2017 to October 2018. Results The baseline SSI rate for all stoma takedown patients was 21.4% (25 of 119). After bundle implementation, this decreased to 7.9% (17 of 221; p = 0.03) over a period of 2.5 years. Then, after changing the preoperative antibiotics, our rate of SSI decreased further to 2.2% (1 of 44; p = 0.039) over a period of 1.5 years. Conclusion Significant reduction of SSI in GI surgery can be accomplished with several prevention strategies (our GI bundle). Then a change of the preoperative antibiotic choice, chosen based on causative wound infection organisms, may further decrease SSI rates. We recommend an institution specific analysis of wound infections and modification of preoperative antibiotics if the responsible organisms are resistant to the original antibiotic choice. Type of study Retrospective cohort study. Level of evidence Level III.

A subset of patients with cloacal malformations requires vaginal replacement during their primary reconstruction, increasing the surgical complexity. Identifying factors which predict the need for vaginal replacement would facilitate operative planning.We retrospectively reviewed patients who underwent primary cloacal reconstruction at our Center (2014-2018) and assessed the length of the common channel, urethra, and vagina. The presence of hydrocolpos at birth, Müllerian anomalies, sacral ratio, and tethered cord were also assessed between patients who did and did not require vaginal replacement.50 patients were identified. 17/50 patients (34%) underwent a total urogenital mobilization (TUM), and none required vaginal replacement. 33/50 (66%) patients underwent a urogenital separation. 19/33 (58%) required vaginal replacement. This group had a shorter vagina (4.2 cm vs 6.6 cm, p 0.01). There was no difference in urethral or common channel length, number of cervices, sacral ratio, presence of a vaginal septum, hydrocolpos, or tethered cord between those who did and those who did not require vaginal replacement.Urethral and common channel lengths were used to successfully determine the operative plan (TUM or urogenital separation) to reconstruct cloacal malformations. The need for urogenital separation and a shorter vaginal length were predictive of the need for vaginal replacement.Level IV.Case series with no comparison groups.

Introduction Total colonic Hirschsprung's disease (TCHD) presents a postoperative challenge due to multiple stools and perineal rash. We propose a protocol developed by pediatric surgeons and ostomy nurses to help prevent and treat hypermotility and severe perineal rash, especially in younger children who are not toilet trained. Materials and Methods We retrospectively reviewed our TCHD patients' charts from 2014 to 2017. All patients received a prescribed protocol for the treatment of hypermotility and perineal rash. We describe patients who underwent their pull through before and after the age of urine toilet training, and assessed the number of bowel movements, the perineal skin status, and growth. Results We treated 25 patients. Out of 25, 9 patients received a straight ileoanal pull through before the age of 18 months. Nine of 25 patients presented for a second opinion and had redo pull through. The remaining seven presented for bowel management after having a pull through at another institution. All these were treated following the hypermotility protocol. In total, 19 of 25 patients were not toilet trained. The mean number of bowel movements in all groups was 4 (3–5). All had a resolution of perineal rash and liquid stools after 3 months. Eleven of the 25 patients presented with failure to thrive. Two older patients experienced severe proctalgia requiring replacement of the ileostomy. Conclusion TCHD patients who underwent definitive pull through had nine high incidence of multiple stool, perineal rash, and low growth. With the implementation of bowel management care to slow the stools and a perineal skin protocol to treat the skin, we believe that these symptoms can be minimized even in patients who are not toilet trained. Since the implementation of this protocol, we have changed our practice to perform the pull through in such patients between the age of 6 and 18 months.

Recto-urethral fistulae are the most common finding in males born with an anorectal malformation (ARM). A high pressure distal colostogram is an important tool in visualizing the fistula, although the precise level at which the fistula communicates with the urethra may be difficult to interpret and is not reported in a uniform manner. This anatomy affects the surgical plan; determining the likelihood that the rectum is reachable via a posterior sagittal incision or better approached through the abdomen via laparoscopy or laparotomy helps counsel families and stratifies diagnoses for outcomes work. Herein we present a figure used at our Center to assist with correlating findings on the distal colostogram with the anatomic level of the recto-urethral fistula in males with anorectal malformations.

This chapter describes the surgical procedure for incision and drainage of Bartholin gland duct cyst or abscess as performed by open approach. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: bimanual examination, vertical incision, placement of Word catheter, or marsupialization.

This chapter describes the surgical and nonsurgical management of ovarian, isolated fallopian tube, or adnexal torsion, via open and laparoscopic approach. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: abdominal access, anatomic assessment, reduction of torsion, ovarian or paratubal cystectomy, and suture pexy of the ligament as needed.

This chapter describes the endoscopic/surgical procedure for ovarian cystectomy for benign ovarian masses as performed by the following approaches: open and laparoscopic. The text includes an introduction that outlines the indications, risks, alternatives, essential steps, needed equipment, and variations in technique for the procedure in question. This is followed by a template operative dictation, which provides the reader with an operative report, such as is found in a patient chart or electronic medical record. A description is provided of the following critical concepts for this procedure: positioning, abdominal access, identification and dissection of ovarian lesion, resection and specimen delivery, and abdominal closure.

Introduction Repair of anorectal malformations (ARMs), primarily or with a reoperation, may be performed in certain circumstances without a diverting stoma. Postoperatively, the passage of bulky stool can cause wound dehiscence and anastomotic disruption. To avoid this, some surgeons keep patients NPO (nothing by mouth) for a prolonged period. Here, we report the results of a change to our routine from NPO for 7 days to clear fluids or breast milk. Materials and Methods After primary or redo ARM surgery, patients given clear liquids were compared to those who were kept strictly NPO. Age, indication for surgery, incision type, use of a peripherally inserted central catheter (PICC) line, and wound complications were recorded. Results There were 52 patients, including 15 primary and 37 redo cases. Group 1 comprised 11 female and 15 male patients. The mean age at surgery was 4.9 years (standard deviation [SD]: 2.3). There were 8 primary cases and 18 redo cases. Twelve (46.6%) received a PICC line. The average start of clear liquids was on day 5.3 (SD: 2.2) after examination of the wound, and the diet advanced as tolerated. The first stool passage was recorded on average on day 2.3 (SD: 1.3). Four minor wound complications and no major wound complications occurred.Group 2 comprised 14 females and 12 male patients. The mean age at surgery was 3.5 (SD: 2.4) years. There were 7 primary and 19 redo cases. One (3.8%) patient required a PICC line. A clear liquid diet was started within 24 hours after surgery. A regular diet was started on average on day 5.8 (SD: 1.3). The first stool passage was recorded on an average of day 1.6 (SD: 0.9). Three minor wound complications occurred; however, there was no significant difference between the two groups (SD: 0.71). One major wound complication occurred. However, there was no significant difference in major wound complications between the groups (SD: 0.33). Conclusion No increase in wound problems was noted in children receiving clear liquids or breast milk compared with the strict NPO group, and PICC line use was reduced. We believe this change in practice simplifies postoperative care without increasing the risk of wound complications.

Background The authors of this article became aware of significant differences in the management of two females with a rectovestibular fistula and associated vaginal agenesis. In one patient, a sigmoid colovaginoplasty was performed at the time of the posterior sagittal anorectoplasty (PSARP), and the other underwent repair of the rectovestibular fistula with a primary PSARP, but the surgeons elected to delay the timing of vaginal reconstruction. We decided to review the literature, to establish if recommendations could be made to optimize the management of these children based on current evidence. Methods A literature review was conducted to determine the management and long-term outcomes in patients with an anorectal malformation and associated vaginal atresia. Specific gynecological outcomes assessed were menstrual egress and adequacy of the vaginal replacement for penile–vaginal intercourse. Results Eighty-eight cases were included in the review. Age at diagnosis had a bimodal distribution: 0 to 5 years, 56%; >10 years, 37%. Vaginal atresia was recognized before the operation in 45 patients. Types of vaginal atresia encountered were: (a) distal vaginal atresia (n = 17), (b) vaginal agenesis with absent Mullerian development (n = 47), (c) vaginal agenesis with variable Mullerian development (n = 17), and (d) cervico-vaginal agenesis (n = 7). Types of vaginal replacement used were sigmoid colovaginoplasty (n = 26), distal rectal fistula as neovagina (n = 30), terminal ileum (n = 5), vaginal pull-through (n = 9), and others/unknown (n = 8). Two patients followed a perineal dilatation program and nine patients await reconstruction. Median follow-up was 18 months (6 weeks to 31 years). Long-term menstrual outcomes were reported in 18 (21%) patients. Sexual function was reported in 10 (11%) patients. Three pregnancies were reported but none resulted in live births. Conclusion Vaginal atresia is often missed in association with ARMs. Management should be in collaboration with pediatric gynecologists. Due to a lack of long-term outcome data, no definite conclusion can be drawn for the best technique, tissue, or timing of vaginal replacement. The opportunity to perform vaginal replacement in conjunction with the rectal repair may be worth considering because of a shallower pelvis, nonscarred tissue planes, and the excellent surgical exposure.

Aim of the Study mTOR inhibitors are showing promising results in the management of vascular anomalies. Although current controlled trials remain to be completed, many individual experiences are being published. We present our series of children with complex vascular anomalies treated with sirolimus. Patients and Methods A retrospective review of 41 patients treated with sirolimus between January 2011 and December 2015 was performed: 15% (n = 6) had vascular tumors (4 kaposiform hemangioendotheliomas, 1 PTEN) and 85% (n = 35) had malformations (13 generalized lymphatic anomalies/Gorham–Stout diseases [GSD], 1 kaposiform lymphangiomatosis [KLA], 11 large lymphatic malformations (LMs) in critical areas, 2 lymphedemas, 4 venous malformations, and 4 aggressive arteriovenous malformations [AVM]). Several variables were collected: type of vascular anomaly, duration of treatment, dosage, response, and secondary effects. Results There was a female predominance (1.4:1). All patients received sirolimus, at initial dosage of 0.8 mg/m2/12 hour. Overall successful response rate was 80.4% of cases, presenting improvement in radiologic imaging and reduction of symptoms, at a median time of 10 weeks. Patients showing no response included four AVMs, one GSD, one LM, one KLA, and one unknown tumor. Sirolimus was well tolerated, even in neonates, with insignificant side effects. No patients had complete resolution and no patients worsened on therapy. Thirty patients remain under treatment at the present moment. Conclusion Sirolimus has become a new therapeutic option for patients with vascular anomalies that do not respond to other treatments. Unfortunately, important questions as what is the most appropriate dosage and for how long should the patient be treated remain unanswered. An international registry followed by customized controlled trials is mandatory to clarify the future of this therapy.

Introduction Central hepatectomy or mesohepatectomy (MH) is a complex surgical technique rarely used in children. It is indicated in central tumors to preserve functioning liver mass avoiding an extended right hepatectomy. The purpose of this article is to analyze our experience with this technique. Methods We reviewed five patients who underwent MH in the period from 2008 to 2014. Diagnoses were hepatoblastoma PRETEXT III (two cases), hepatic embryonal sarcoma (one case), focal nodular hyperplasia (one case), and vascular tumor with rapid growth in a newborn causing an acute liver failure, compartment syndrome, and multiple organ failure (one case). In all cases, the tumor was centrally located, including the segment IVb, with large displacement of the hepatic pedicle in two cases. Results MH was standard in three cases and under total vascular exclusion in two cases. All children are alive with a mean follow-up of 38 (6–70) months. None of the children required reoperation because of bleeding. One child developed a biliary fistula in the cutting area that closed spontaneously. The newborn with the vascular tumor required the placement of a Gore-Tex patch (W. L. Gore & Associates, Inc, Flagstaff, Arizona, United States) to relieve the compartment syndrome. He subsequently underwent partial embolization of the tumor and MH under vascular exclusion. Conclusions In selected patients, MH is an alternative to trisegmentectomy and should be available in advanced pediatric hepatobiliary units.

Cloacal exstrophy is the most severe type of anorectal malformations that belongs to the bladder–exstrophy–epispadias complex of genitourinary malformations. Interestingly, its variant, the covered cloacal exstrophy, is often missed. The clinical findings of this variant may include an imperforate anus, low lying umbilicus, thick pubic bone, and pubic diastasis but with an intact abdominal wall. We present an interesting case of covered cloacal exstrophy with a side-by-side duplicated bladder and discuss important considerations for the time of colostomy creation in the newborn period.

Aim of the Study The goals of urinary reconstruction in urogenital sinus and cloacal repair include: (1) positioning of the bladder neck above the urogenital diaphragm to maximize future urinary continence, and (2) creating a visible urethra that can be catheterized if needed. A recent algorithm in cloacal reconstruction proposed a urethral length of 1.5 cm as the key determinant in deciding whether to perform a total urogenital mobilization or a urogenital separation, the hypothesis being that a 1.5 cm length urethra is needed for the patient to remain dry. We wondered if the normal female urethral length correlated with this empiric technical determinant. Methods We reviewed voiding cystourethrograms of healthy female patients between ages 6 and 36 months and measured the patient's urethral length. Results Ninety-one children were included. The mean urethral length for patients age 6–12 months was 2.50 cm, age 12–24 months was 2.31 cm, and age 24–36 months was 2.59 cm. There was no difference between the urethral length in the three groups (p = 0.38). Of 91 patients, 87 (96%) had a urethral length > 1.5 cm. Conclusion A urethra of at least 1.5 cm was present in the majority of normal control patients. We believe therefore that for urogenital sinus and cloacal repair, surgeons can extrapolate that patients need a 1.5 cm urethra at the end of the reconstruction. Additional follow-up is needed to determine if this urethral length as an independent factor maintains dryness in the long term after cloacal repair. Type of Study Case Series. Level of Evidence III.

Aim Extrahepatic portal vein obstruction (EHPVO) is a frequent cause of noncirrhotic portal hypertension in children. The aim of this study is to analyze long-term results after diversion surgery. Patients and Methods Retrospective review of EHPVO patients who underwent diversion surgery analyzing number of platelets, leukocytes, prothrombin activity, splenomegaly, and gastrointestinal bleeding 10 years after surgery. Results Thirty-three patients were evaluated, mostly males (64%) and presenting with gastrointestinal bleeding (46%). Mesoportal shunt (Rex) was performed in 19 patients, mesocaval in 7, distal splenorenal in 7, and proximal splenorenal in 3. While comparing mesoportal shunt to the other portosystemic shunts, an increase in platelets was found with every technique, but it was clearly higher in mesoportal shunt. The highest increase was 6 months after surgery (p = 0.0015) as well as prothrombin activity (p = 0.0003). Leukocytes level also increased without statistical significance. Spleen size (cm) and spleen size Z score (SSAZ) decreased significantly 6 months after mesoportal shunt (p = 0.0168). Before surgery, over 94% patients suffered gastrointestinal bleeding, which reduced significantly afterward with bleeding episodes in only four (12%) of them. Conclusion Diversion surgery in EHPVO, especially mesoportal shunt of Rex, improves hepatic function (prothrombin activity), reduces hypersplenism (platelets, leukocytes, and spleen size), and decreases gastrointestinal bleeding episodes.

Patients born with complex anorectal malformations often have associated Mullerian anomalies, which might affect fertility and obstetrical outcomes. Other vertebral-anorectal-tracheoesophageal-renal-limb associations, such as renal or cardiac anomalies, could also affect pregnancy intention, fertility rates, and recommendations about mode of delivery or obstetrical outcomes. Associated conditions present at birth, like hydrocolpos, could also potentially affect fertility. Depending on the complexity of the anomaly, primary reconstruction might include vaginoplasty, vaginal interposition, perineal body reconstruction, and extensive pelvic dissection. After the initial reconstruction, patients might have multiple additional surgeries for stoma reversal, bladder augmentation, and creation of conduits, all with potential for pelvic adhesions. Pregnancy intention, fertility rates, mode of delivery, and obstetrical outcomes data are limited in this patient population, making it challenging to counsel patients and their families. We sought to evaluate all available literature in an attempt to better counsel families. A PubMed literature search was undertaken to review this topic. Search terms of “cloaca,” “anorectal malformation,” “pregnancy,” “cloacal exstrophy,” “vaginal delivery,” and “cesarean section” were used and citation lists from all identified articles were checked to ensure that all possible articles were included in the review. We also outline comorbidities from the fetal period to adulthood that might affect reproductive health. Of the articles on anorectal and cloacal anomalies, 13 reports were identified that covered obstetrical outcomes. They were in patients with previous anorectal malformation, cloaca, and cloacal exstrophy repair. Twenty-four pregnancies were reported in 16 patients. Two ectopic pregnancies, 5 spontaneous miscarriages, 1 triplet pregnancy, and 16 singleton pregnancies were reported with a total of 19 live births. Regarding the method of conception, 15/18 pregnancies occurred spontaneously and 3/18 were via assisted reproductive technology with in vitro fertilization. There were 19 live births, of which at least 8 were preterm. Mullerian anatomy was reported in 8 of 13 articles. Only 2 patients underwent vaginal delivery (1 patient with repaired cloaca malformation had an operative vaginal delivery and 1 patient with repaired imperforate anus with rectovaginal fistula had a normal spontaneous vaginal delivery). The remaining patients all underwent a cesarean section. There were no reported cases of maternal mortality, and maternal morbidity was limited to recurrent urinary tract infections and worsening chronic kidney disease. There is a paucity of information regarding obstetrical outcomes in adult anorectal and cloaca patients. However, patients with previous cloacal repairs have achieved pregnancy spontaneously, as well as with in vitro fertilization. Patients with repaired cloacal malformations are at increased risk of preterm birth and cesarean delivery. Most patients with cloacal anomalies have an associated Mullerian anomaly and therefore have an increased risk of preterm labor. From our review we conclude that contraception should be offered to patients not desiring pregnancy, and cesarean section is likely the preferred mode of delivery. On the basis of this review, we recommend proactive data collection of all such patients to document outcomes and collaboration among providers and between centers devoted to this complex patient population.

Introduction We previously reported our surgical technique for functional constipation for patients who have failed medical management using a novel collaborative approach with gastroenterology input, pre-operative contrast enema, colonic manometry, and laxative protocol combined with a laparoscopic colonic resection with Malone appendicostomy. Now we report our intermediate outcomes. Methods Patients who failed bowel management program for functional constipation were reviewed from 3/2014–2/2017. Patients with Hirschsprung disease, anorectal malformation, tethered cord, spina bifida, Trisomy 21, cerebral palsy, mitochondrial disease, or prior colon resection were excluded. Results Of 31 patients (14 females; median age 12years, follow-up 10.3months) with functional constipation and failed medical management, 26 (84%) had preoperative colonic manometry which, in addition to the contrast enema, guided laparoscopic colon resection. Ten patients (32.3%) are clean with no flushes (1 takes no laxatives, 8 are on low dose laxatives only, and 1 patient was clean on laxatives but chose to switch back to flushes). Of the 21 patients that remain on antegrade flushes, 20 (95.2%) are clean, and one patient (4.8%) continues to soil. We define clean as no soiling and no abnormal stool burden on x-ray. Laxative trials are planned for all patients on an antegrade flush regimen. Conclusion Our intermediate results show that laparoscopic colon resection with Malone appendicostomy allows the majority of patients to be clean on antegrade flushes, and some to be on no or minimal laxatives. Type of study Retrospective review. Level of Evidence 3

Introduction Patients with anorectal malformations (ARM), Hirschsprung disease (HD), and colonic motility disorders often require care from specialists across a variety of fields, including colorectal surgery, urology, gynecology, and GI motility. We sought to describe the process of creating a collaborative process for the care of these complex patients. Methods We developed a model of a devoted center for these conditions that includes physicians, psychologists, social workers, nurses, and advanced practice nurses. Our weekly planning strategy includes a meeting with representatives of all specialties to review all patients prior to evaluation in our multidisciplinary clinic, followed by combined exams under anesthesia or surgical intervention as needed. Results There are 31 people working directly in the Center at present. From the Center's start in 2014 until 2017, 1258 patients were cared for from all 50 United States and 62 countries. 360 patients had an ARM (110 had a cloacal malformation, 11 had cloacal exstrophy), 223 presented with HD, 71 had a spinal malformation or injury causing neurogenic bowel, 321 had severe functional constipation or colonic dysmotility, and 162 had other diagnoses including familial polyposis, Crohn's disease, or ulcerative colitis. We have had 170 multidisciplinary meetings, 170 multispecialty outpatient, and 52 nurse practitioner clinics. In our bowel management program we have seen a total of 514 patients in 36 sessions. Conclusion This is the first report describing the design of a multidisciplinary team approach for patients with colorectal and complex pelvic malformations. We found that approaching these patients in a collaborative way allows for combined medical and surgical decisions with many providers simultaneously, facilitates therapy, and can potentially improve patient outcomes. We hope that this model will help establish new-devoted centers in other locations to encourage centralized care for these rare malformations. Level of evidence IV.

Aim of the study Many patients with an anorectal malformation (ARM) or pelvic anomaly have associated urologic or gynecologic problems. We hypothesized that our multidisciplinary center, which integrates pediatric colorectal, urologic, gynecologic and GI motility services, could impact a patient’s anesthetic exposures and hospital visits. Methods We tabulated during 2015 anesthetic/surgical events, endotracheal intubations, and clinic/hospital visits for all patients having a combined procedure. Main results 82 patients underwent 132 combined procedures (table 1). The median age at intervention was 3 years [0.2-17], and length of follow up was 25 months[7-31]. The number of procedures in patients who underwent combined surgery was lower as compared to if they had been done independently [1(1-5) vs 3(2-7)(p

The sacral ratio (SR) is a well-established tool to quantify sacral development in patients with anorectal malformations (ARM) and can be used as a predictor of fecal continence. We hypothesized that a lower SR correlated with the presence of urologic and renal malformations.We retrospectively reviewed the medical records of patients with ARM treated at our center from 2014 to 2016. We measured the lateral SR as a marker for sacral development and assessed the spine for the presence of tethered cord (TC). Urological and renal anomalies, including single kidney, hydronephrosis, hypospadias, vesicoureteral reflux (VUR), ectopic ureter, and penoscrotal transposition were assessed. Analysis of variance (ANOVA), t-tests, and multivariable linear regression were used to test for differences in SR with consideration of associated urologic malformations and tethered cord.283 patients with ARM were included for analysis (156 females). The median age was 39months (10-90). Among these, 178 (55.6%) had 1 or more urologic malformations, and 81 (25.3%) had a TC. Hydronephrosis, high-grade VUR (3-5), solitary kidney, and tethered cord were significantly associated with lower SR (p0.01). In multivariable regression models, the presence of urologic abnormalities remained significantly associated with lower a SR despite the presence or absence of TC (p0.001).SR is a potentially useful indicator of certain urologic anomalies including hydronephrosis, high grade VUR, and solitary kidney in patients with ARM. This association is independent of the presence of TC. A sacral ratio as a part of the VACTERL screening can help the surgeon identify which patients need closer urologic follow up.IV.

Introduction Congenital portosystemic shunt (CPSS) is a rare entity without insufficiency in treatment issues. The aim of this article is to show our experience in the heterogeneity of this condition. Material and Methods A retrospective study of 25 CPSS in the period 1995 to 2014 was conducted. Description of the morphology, clinical impact, and treatment is given. Results According to the imaging techniques (IT), the shunt was apparently intrahepatic in 14 patients, extrahepatic in 10 patients, and mixed in 1 patient. In 14 children, IT showed hepatic portal circulation. In total shunts in which radiological examination was performed, invasive radiological techniques were able to demonstrate intrahepatic portal vein. In other patients, it was not investigated as they are asymptomatic. A child presented multiorgan failure with fulminant hepatic failure at birth. The shunt was radiologically closed and clinical impairment reversed rapidly. He is now asymptomatic with no longer images of CPSS in ultrasound scan controls. Also, seven children are asymptomatic at this time and are monitored periodically. Seven children had prenatal diagnosis, in five the shunt closed spontaneously. Nine children were symptomatic in their evolution (hyperammonemia, regenerative nodules, cholestasis, gastrointestinal bleeding). Of these, in five we performed balloon test occlusion, tolerated in all patients, followed by radiological closure. In our experience, the advancement of interventional radiology techniques avoided surgery to close the shunt. Conclusions Morphologically, the CPSS is extremely heterogeneous, with multiple possible connections established. CPSS has multiple clinical presentations, from asymptomatic patients to acute liver failure. The therapeutic approach should be individualized and therefore held in overspecialized centers.

Introduction Most patients with a cloacal malformation have a Mullerian anomaly. We sought to examine our patients with a cloacal malformation to determine the proportion of them we felt we knew their Mullerian anatomy and which proportion we felt would most benefit from longitudinal assessment to define their anatomy, reproductive potential, and risk of outflow tract obstruction after puberty. We also compared the preoperative assessment of reproductive anatomy (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) and intra-operative abdominal findings (at the time of primary cloacal reconstruction or subsequent abdominal procedures) to see how these correlated with and which preoperative assessment tool was most predictive of intraoperative anatomy. We also sought to confirm what we expected to be a lack of ovarian pathology. Methods A single site retrospective chart review was performed on all patients with a cloacal anomaly seen between May 2014 and September 2017. Preoperative assessment (pelvic ultrasound, MRI, cloaca gram, and vaginoscopy) and operative reports (both primary reconstruction and later abdominal procedures) were reviewed to ascertain Mullerian and ovarian anatomy. Results 30 of 36 (83%) of patients had defined Mullerian anatomy after preoperative assessment (pelvic ultrasound, MRI, cloacagram, and vaginoscopy) with or without the addition intraoperative assessment of Mullerian structures obtained during laparoscopy or laparotomy. 19/30 (63%) had duplication of their Mullerian structures. 25/36 (69%) had intraoperative assessment of Mullerian anatomy during laparoscopy or laparotomy. In this group, preoperative assessment with pelvic ultrasound correlated in 4/8 patients (50%), MRI correlated in 3/4 patients (75%), cloacagram in 10/15 patients (67%), and vaginoscopy in 23/25 patients (92%). 14/36 (39%) patients were found to require longitudinal assessment to define anatomy, reproductive potential or risk of outflow tract obstruction after puberty. Patients with ovarian findings described at the time of laparoscopy or laparotomy had no evidence of ovarian pathology. Conclusions The majority of patients with cloaca in our series (83%) had their Mullerian anatomy defined by either preoperative assessment and/or findings at the time of laparoscopy or laparotomy. Duplication of the vagina and uterus was the most commonly described Mullerian anatomy (63%) in our series. Vaginoscopy appears to be superior to pelvic ultrasound, MRI, and cloacagram in predicting Mullerian anatomy. Fourteen of our 36 (39%) patients will require longitudinal assessment follow for reproductive potential and/or risk of outflow tract obstruction after puberty as their Mullerian anatomy is not known. There was no evidence of ovarian pathology in any cloaca patient. While we felt as though we could define Mullerian anatomy in most of our patients, any opportunity for intraoperative assessment of Mullerian anatomy should be utilized and therefore teams who are involved in the management of these patients must have a systematic and collaborative method established to ensure that Mullerian structures are thoroughly evaluated intra-operatively and documented in a standardized fashion. Type of study Retrospective Chart review. Level of Evidence III.

Introduction Health literacy is low in an estimated one-third of the US population. Little is known about the health literacy of caregivers of children with colorectal conditions. The objective of this study was to investigate whether a timed health literacy intervention could improve health literacy in this population. Methods We used the BRIEF Health Literacy screening (BHLS) tool on caregivers of children who came to our colorectal clinic. Health literacy was categorized as inadequate, marginal, or adequate. The number of caregivers with adequate health literacy was compared to the number of clinic visits and socioeconomic status. Results We included 233 caregivers. The average number of clinic visits was 3.5 over 1.2 years. At the first clinic visit, 70% (n = 98) of caregivers had “adequate” health literacy. Scores improved to 88% (p = 0.024) after the fourth visit. Socioeconomic factors were not associated with health literacy. Patients of caregivers with “adequate” health literacy visited our clinic 3.8 times, compared to 2.7 times for those with lower literacy (p = 0.006). Conclusion Emphasis on providing an education-based approach at each visit increased health literacy significantly. As expected, health literacy was lowest during the first visit, which we believe is the optimal time to implement educational interventions. Type of study Case Control/Retrospective Comparative Study. Level of evidence Level III.

Background and aim Senna is a stimulant laxative commonly used by pediatricians, pediatric gastroenterologists, and pediatric surgeons. Many clinicians avoid Senna for reasons such as tolerance or side effects but this has little scientific justification. We recently found several patients we were caring for developed perineal blistering during the course of Senna treatment. Because of this we chose to review the literature to identify side effects in children taking this medication as well as to analyze our Center's experience with Senna 's secondary effects. Methods We performed a literature review (MEDLINE, PUBMED) using the keywords of Senna , sen, sennosides and children, and pediatric and functional (idiopathic) constipation. We looked for articles with information regarding perineal blisters related to Senna as well as other secondary effects of Senna laxatives in children when used on a long-term basis. We also reviewed the charts of our patients who had previously taken Senna or are currently taking Senna , looking for adverse reactions. Results Eight articles in the literature reported perineal blisters after administration of Senna laxatives in 28 patients. Of those occurrences, 18 patients (64%) had accidental administration of Senna and 10 (36%) had Senna prescribed as a long term treatment. All of the blistering episodes were related to high dose, night-time accidents, or intense diarrhea with a long period of stool to skin contact. At our institution, from 2014 to 2017, we prescribed Senna and have recorded data to 640 patients. During the study period, 17 patients (2.2%) developed blisters during their treatment. Patients who developed blisters had higher doses 60mg/day; 60 [12–100] vs. 17.5 [1.7–150] (p All of the blistering episodes were related to night-time accidents, with a long period of stool to skin contact. 83 (13%) patients presented minor side effects such as abdominal cramping, vomiting or diarrhea which resolved once the type of laxatives were changed or enemas were started. The doses of Senna was not significantly different in these patients 15mg/day [4.4–150] vs. 17.5mg/day [1.5–150]. There were no other long-term side effects from Senna found in the pediatric literature for long-term treatment besides abdominal cramping or diarrhea during the first weeks of administration. We found no evidence of tolerance to Senna in our review. Conclusion There is a paucity of information in the literature regarding side effects of sennosides as a long-term therapy, and to our knowledge, this is the first review of Senna side effects in children. Senna induced dermatitis is rare, but may occur when patients need a higher dose. All of the cases described had a long period of exposure of the skin to stool. Besides the perineal rash with blisters, we could find no other described major side effect with Senna administration in the pediatric population or evidence of the frequently mentioned concern of the development of tolerance to Senna . Pediatric caregivers should advise families of the rare side effect of skin blistering and educate them to change the diaper frequently in children who are not toilet- trained to reduce stool to skin exposure. We can conclude from this review that Senna is a safe treatment option for constipation in children. Level of evidence IV.

Aim Short bowel syndrome (SBS) is the leading cause of intestinal failure (IF) in the pediatric population. Our aim was to review long-term outcome of ultrashort bowel syndrome (USBS) in an Intestinal Rehabilitation Unit (IRU). Patients and Methods Retrospective study of patients with USBS (defined as Results Out of 250 children, 30 referred to the IRU met inclusion criteria. Upon first assessment, patients had a median age of 3 (1–217) months and had undergone 3 (1–6) previous laparotomies that left 5 (0–9) cm of remnant small bowel. The main cause of USBS was neonatal midgut volvulus (50%). Follow-up was 28 (4–175) months. Advanced IF-associated liver disease (IFALD) was documented in 63%. None of the patients achieved digestive autonomy and was consequently considered for IT. One patient was excluded, five died before IT, and three are still on the waiting list. Six patients received an isolated IT, 6 a combined liver IT, and 18 a multivisceral graft. Digestive autonomy was achieved in 71% after 31 (14–715) days after IT and currently 62% are alive and off total PN. A significant drop in IFALD progression prior to IT was observed with the introduction of new lipid emulsions in 2010 (SMOF or Soy oil MCT (mid-chain triglycerides) Olive oil Fish oil). Conclusion A multidisciplinary IRU including an IT program offers a comprehensive approach for patients with IF and is crucial to improve survival rate of USBS. New PN lipid emulsions had an impact on IFALD progression and may eventually reduce overall mortality.

Introduction Severe tracheobronchial injuries (TBI) in children are usually traumatic or iatrogenic. However, they can also be caused by mediastinal infections that lead to critical situations. We herein report our experience in the treatment of these lesions. Methods A retrospective study was conducted for patients treated at our center from 2008 to 2014. TBI was diagnosed by imaging studies and bronchoscopy. Treatment was initially conservative (drainage of air and secretions, mechanical ventilation with minimal pressures, and an early extubation) with a limited use of surgical procedures whenever necessary. Results A total of 10 patients (7 males and 3 females) with a median age of 7.5 years (range, 3–17 years) suffered TBI. The mechanism was traumatic in six (three accidental and three iatrogenic) and mediastinal infection in four (three mycotic and one bacterial abscesses). All traumatic cases responded to conservative measures, except one iatrogenic lesion, which was surgically repaired. There were no complications or residual damages. Two patients with mediastinal infection presented with sudden cardiorespiratory arrest, one with hemoptysis caused by an arteriotracheal fistula and the other because of carinal rupture. Both died before any therapeutic measures could be taken. The other two patients were treated, one with previous extracorporeal membrane oxygenation support, underwent arterial embolization, but ultimately died, and the other one survived, but required esophagectomy and creation of a thoracostome for secondary wound closure of the bronchocutaneous fistula. Conclusion Conservative treatment with gentle respiratory support suffices in most traumatic cases of TBI. Infectious abscesses with involvement of adjacent structures sometimes require complex surgery and are life-threatening.

Introduction Ganglioneuroma (GN) is a benign, differentiated variety of neurogenic tumor. It is often asymptomatic and may be diagnosed by serendipity. Surgical removal is the treatment of choice. However, it has been suggested that postoperative complications and sequelae might outweigh the benefits of this approach. The purpose of the present study was to examine these issues in a large experience of neural tumors. Methods Patients treated between 1992 and 2012 were retrospectively reviewed. Modern imaging, measurement of catecholamine metabolite excretion and metaiodobenzylguanidine were used for workup. Surgical treatment aimed at complete resection. Complications and sequelae were recorded. Literature was searched for regrowth or malignant transformation of GN. Results Of 227 patients with neural tumors, 24 were GN patients (12 abdominal, 11 thoracic and 1 cervical with 8 dumbbell extensions). Six children were symptomatic (three with abdominal pain and mass, one with stridor or dysphonia, and one each with anisocoria and opsomyoclonus). However, 18 (75%) were asymptomatic and the diagnosis was incidental. Several tumors were large and involved more than one body space. There were no neurologic symptoms in eight cases with dumbbell extension. Complete resection was achieved in 20 children (83%) whereas gross residual was left in four. Postoperative complications were: Horner syndrome (3 patients), mild scoliosis (1 patient), adhesive bowel obstruction (1 patient) and acute urinary retention (1 patient). There was no evidence of either regrowth or malignant behavior in residual masses left in place after follow-up of 84 (1–194) months. Conclusions There were a limited number of general minor complications in this series that did not include cases of regrowth or malignant transformation. However, these unfavorable events were occasionally reported in the literature. Since diagnosis of GN cannot be ascertained before removal of the mass, this should remain the aim of the treatment, although limiting the chances of complications to a minimum even if incomplete resection is the price to pay. Nonoperative attitudes should not be recommended in all cases, but they are certainly justified in some.