Devin R. Halleran

High Rate of Major Morbidity after Surgical Excision for Pilonidal Disease

BACKGROUNDnRecurrent pilonidal disease has been reported to occur in up to 30% of patients after their initial infection. Surgical resection is often performed to prevent recurrence of disease, however, morbidity after surgical excision from incision complications and disease recurrence is common. The aim of this study was to quantify major morbidity after initial pilonidal excision.nnnPATIENTS AND METHODSnPatients with pilonidal disease who had initial excision procedures between 2011-2013 at hospitals reporting data to the Pediatric Health Information System (PHIS) were included. Predictors of the composite outcome of major surgical site complication or surgical re-excision within one year were evaluated using multivariable logistic regression models. Kaplan-Meier analysis was used to examine time to surgical re-excision.nnnRESULTSnOf the 1,932 patients included, 4.7% (nu2009=u2009138) had a major surgical site complication, 8.0% (nu2009=u2009154) had a surgical re-excision, and 8.7% experienced either event within one year of their initial excision. The majority of re-excisions for recurrent disease occurred during the first two years after the initial excision. Risk factors associated independently with a greater risk of the composite outcome included older age (odds ratio [OR] 1.04 [95% confidence interval {CI} 1.00-1.07), pu2009=u20090.03), male gender (OR 1.49 [95% CI 1.09-2.08), pu2009=u20090.01), and the presence of a complex chronic gastrointestinal condition (OR 4.33 [95% CI 1.96-9.59], pu2009<u20090.001).nnnCONCLUSIONSnSurgical excision of pilonidal disease is often complicated by site complications and nearly 1 of 10 patients develop recurrent disease requiring re-excision within two years after their initial excision. Future research into alternative therapies to treat pilonidal disease is warranted.

Laser Hair Depilation in the Treatment of Pilonidal Disease: A Systematic Review

BACKGROUNDnLaser hair depilation has shown promise in small series of patients with pilonidal sinus disease. The purpose of this study was to review the published literature on laser hair depilation in pilonidal disease to determine its effect on disease recurrence.nnnMETHODSnThe PubMed, Embase, and Cochrane Central Register of Controlled Trials databases were searched to identify all studies published through December 1, 2017 that examined the role of laser hair depilation in the treatment of pilonidal sinus disease. Study characteristics, including design, treatment regimen, number of patients evaluated, rate of recurrence, and duration of follow up were recorded.nnnRESULTSnThirty-five published studies were included. Of these, 28 studies were retrospective and seven were prospective. There were five comparative studies: two retrospective, one prospective observational, and two randomized controlled trials. The number of patients included in each study ranged from one to 86 patients and patients received between one and 11 laser treatments. The pilonidal disease recurrence rate after laser depilation ranged from 0% to 28% at a mean follow-up ranging from 6 months to 5 years across studies. Four of the five studies that included a comparative group demonstrated a decreased recurrence rate compared to the non-laser cohort.nnnCONCLUSIONnLaser hair depilation is a promising therapy in the management of pilonidal disease. However, the literature published to date is heterogeneous and has limited generalizability. Additional research is needed to determine the effectiveness of laser hair depilation to prevent pilonidal disease recurrence.

Recurrence of pilonidal disease: our best is not good enough

BACKGROUNDnRecurrence rates of pilonidal disease have been reported to be high as 30%. Patients with recurrent pilonidal disease often develop chronic wounds and draining sinuses that incur long-term morbidity, disability, and decreased quality of life. The aim of this study was to characterize rates of recurrence in patients with pilonidal disease treated by pediatric surgeons.nnnMETHODSnA single center retrospective review of patients with pilonidal disease evaluated by pediatric surgeons from 2010 to 2015 was performed. Recurrence of pilonidal disease was defined as an episode of active pilonidal disease that required medical or surgical intervention >30xa0days from the preceding treatment. Repeated events proportional hazards regression modeling was performed to identify factors associated with time to recurrence.nnnRESULTSnAmong 307 patients treated for pilonidal disease, nearly 50% were male, and the median age at initial evaluation was 16xa0years (IQR 15-17). Approximately 45% were obese (BMI ≥ 95th percentile). The initial treatment during the study period was surgical excision in two-thirds and incision and drainage and/or antibiotics in one-third. The overall recurrence rate was 33%, with the majority of recurrences (80%) occurring within the first year. On multivariable analysis, obese BMI was the only factor independently associated with time to disease recurrence.nnnCONCLUSIONSnPilonidal disease has a substantial recurrence rate even after surgical excision. Future studies investigating treatments that can prevent disease recurrence are needed.

Obstetrical outcomes in adult patients born with complex anorectal malformations and cloacal anomalies: a literature review

Patients born with complex anorectal malformations often have associated Müllerian anomalies, which might affect fertility and obstetrical outcomes. Other vertebral-anorectal-tracheoesophageal-renal-limbxa0associations, 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 inxa0vitro fertilization. There were 19 live births, of which at least 8 were preterm. Müllerian 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 inxa0vitro fertilization. Patients with repaired cloacal malformations are at increased risk of preterm birth and cesarean delivery. Most patients with cloacal anomalies have an associated Müllerian 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.

A descriptive model for a multidisciplinary unit for colorectal and pelvic malformations

INTRODUCTIONnPatients 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.nnnMETHODSnWe 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.nnnRESULTSnThere are 31 people working directly in the Center at present. From the Centers 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, Crohns 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.nnnCONCLUSIONnThis 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.nnnLEVEL OF EVIDENCEnIV.

The Mullerian Black Box: Predicting and defining Mullerian anatomy in patients with cloacal abnormalities and the need for longitudinal assessment

INTRODUCTIONnMost 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.nnnMETHODSnA 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.nnnRESULTSn30 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.nnnCONCLUSIONSnThe 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.nnnTYPE OF STUDYnRetrospective Chart review.nnnLEVEL OF EVIDENCEnIII.

Assessment of the Heineke–Mikulicz anoplasty for skin level postoperative anal strictures and congenital anal stenosis

INTRODUCTIONnAcquired 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.nnnMETHODSnWe retrospectively reviewed all patients who underwent HMA for skin level strictures following PSARP or for certain congenital anal stenoses from 2014 to 2017.nnnRESULTSnTwenty-eight patients (19 males, 9 females) with a mean age of 5.8u202fyears (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 1.0u202fyears (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, pu202f<u202f0.001). There were no operative complications. One patient restenosed and required a secondary procedure.nnnCONCLUSIONnHMA 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.nnnTYPE OF STUDYnCase series.nnnLEVEL OF EVIDENCEnLevel IV.

Screening practices and associated anomalies in infants with anorectal malformations: Results from the Midwest Pediatric Surgery Consortium

BACKGROUNDnThis study evaluates screening practices and the incidence of associated anomalies in infants with anorectal malformations (ARM).nnnMETHODSnWe performed a multi-institutional retrospective cohort study of children born between 2007 and 2011 who underwent surgery for ARM at 10 childrens hospitals. ARM type was classified based on the location of the distal rectum, and all screening studies were reviewed.nnnRESULTSnAmong 506 patients, the most common ARM subtypes were perineal fistula (40.7%), no fistula (11.5%), and vestibular fistula (10.1%). At least 1 screening test was performed in 96.6% of patients, and 11.3% of patients underwent all. The proportion of patients with ≥1 abnormal finding on any screening test varied by type of ARM (p<0.001). Screening rates varied from 15.2% for limb anomalies to 89.7% for renal anomalies. The most commonly identified anomalies by screening category were: spinal: tethered cord (20.6%); vertebral: sacral dysplasia/hemisacrum (17.8%); cardiac: patent foramen ovale (58.0%); renal: hydronephrosis (22.7%); limb: absent radius (7.9%).nnnCONCLUSIONnScreening practices and the incidence of associated anomalies varied by type of ARM. The rate of identifying at least one associated anomaly was high across all ARM subtypes. Screening for associated anomalies should be considered standard of care for all ARM patients.nnnTYPE OF STUDYnMulti-institutional retrospective cohort study.nnnLEVEL OF EVIDENCEnIII.

A comparison of Malone appendicostomy and cecostomy for antegrade access as adjuncts to a bowel management program for patients with functional constipation or fecal incontinence

BACKGROUNDnAppendicostomy and cecostomy are two approaches for antegrade enema access for children with severe constipation or fecal incontinence as adjuncts to a mechanical bowel management program. Each technique is associated with a unique set of complications. The purpose of our study was to report the rates of various complications associated with antegrade enema access techniques to help guide which option a clinician offers to their patients.nnnMETHODSnWe reviewed all patients in our Center who received an appendicostomy or cecostomy from 2014 to 2017 who were participants in our bowel management program.nnnRESULTSn204 patients underwent an antegrade access procedure (150 appendicostomies and 54 cecostomies). Skin-level leakage (3% vs. 22%) and wound infections (7% vs. 28%) occurred less frequently in patients with appendicostomy compared to cecostomy. Nineteen (13%) appendicostomies required revision for stenosis, 4 (3%) for mucosal prolapse, and 1 (1%) for leakage. The rates of stenosis (33 vs. 12%) and wound infection (13 vs. 6%) were higher in patients who received a neoappendicostomy compared to an in situ appendicostomy. Intervention was needed in 19 (35%) cecostomy patients, 15 (28%) for an inability to flush or a dislodged tube, and 5 for major complications including intraperitoneal spillage in 4 (7%) and 1 (2%) for a tube misplaced in the ileum, all occurring in patients with a percutaneously placed cecostomy. One appendicostomy (1%) patient required laparoscopic revision after the appendicostomy detached from the skin.nnnCONCLUSIONnPatients had a lower rate of minor and major complications after appendicostomy compared to cecostomy. The unique complication profile of each technique should be considered for patients needing these procedures as an adjunct to their care for constipation or fecal incontinence.nnnTYPE OF STUDYnRetrospective comparative study.nnnLEVEL OF EVIDENCEnLevel III.

Can sacral development as a marker for caudal regression help identify associated urologic anomalies in patients with anorectal malformation

BACKGROUND AND AIMnThe 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.nnnMETHODSnWe 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.nnnRESULTSn283 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 (p<0.01). In multivariable regression models, the presence of urologic abnormalities remained significantly associated with lower a SR despite the presence or absence of TC (p<0.001).nnnCONCLUSIONnSR 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.nnnLEVEL OF EVIDENCEnIV.