Complications following ileal pouch-anal anastomosis in pediatric ulcerative colitis.

Ileal pouch-anal anastomosis (IPAA) is the procedure of choice for reconstruction after total proctocolectomy in pediatric patients with ulcerative colitis. 30-60 % of patients undergoing IPAA will experience a postoperative complication. The primary objective of this article is to address the most common complications specific to IPAA in the pediatric population and provide an up-to-date review of their presentation, risk factors, workup, and management. We also share our preferred approaches to management and prevention of complications, where relevant. We intend to provide a concise review on the topic aimed at pediatric surgeons and healthcare providers involved in the care of this population with the goal of contributing to improved outcomes and patient quality of life.

Hirschsprung-Associated Enterocolitis at a Referral Institution: A Retrospective Review.

BACKGROUND: Hirschsprung-associated enterocolitis (HAEC) is the most common cause of morbidity and mortality amongst patients with Hirschsprung disease (HD); rectal Botulinum toxin (Botox) has been reported a possible prevention strategy. We aimed to evaluate our institution's historic cohort of HD patients, first to determine our incidence of HAEC and second to begin assessing the effect of Botox on HAEC incidence. METHODS: Patients with HD seen at our institution between 2005 and 2019 were reviewed. Incidence of HD and frequencies of HAEC and Botox injections were tallied. Associations between initial Botox treatment or transition zone and HAEC incidence were evaluated. RESULTS: We reviewed 221 patients; 200 were included for analysis. One hundred thirteen (56.5%) patients underwent primary pull-through at a median age of 24 days (IQR 91). Eighty-seven (43.5%) patients with initial ostomy had their intestinal continuity reestablished at a median of 318 days (IQR 595). Ninety-four (49.5%) experienced at least one episode of HAEC and 62 (66%) experienced multiple episodes of HAEC. Nineteen (9.6%) patients had total colonic HD and had an increased total incidence of HAEC compared to patients without total colonic HD (89% vs 44%, p < 0.001). Six (2.9%) patients received Botox injections at the time of pull-through or ostomy takedown; one experienced an episode of HAEC (versus 50.7% of the patients who were confirmed to have not received Botox injections at their surgery, p = 0.102). CONCLUSION: Further prospective study on Botox's effect on Hirschsprung-associated enterocolitis is required and is the next step in our investigation. LEVEL OF EVIDENCE: Level III.

High Infertility Rates and Pregnancy Complications in Female Physicians Indicate a Need for Culture Change.

OBJECTIVE: This survey study aims to determine the prevalence of pregnancy complications and infertility in female physicians in comparison to the general population. Risk factors, workplace environment, and education are also examined. BACKGROUND: Physicians undertake long training and have stressful work environments during optimal childbearing years. While growing literature indicates increased rates of pregnancy complications and infertility in female surgeons, the prevalence in female physicians of all specialties is unknown. METHODS: An anonymous, voluntary survey was distributed to female physicians via private physician social media groups. It queried pregnancy demographics and complications, infertility diagnosis and treatment, workplace environment, and prior education on these topics. Results were compared with general population data, between medical and surgical subspecialties, and between physicians who were and were not educated on the risks of delaying pregnancy. RESULTS: A total of 4533 female physicians completed the survey. Compared with the general population, female physicians were older at first pregnancy, more often underwent infertility evaluation and treatment, and had higher rates of miscarriage and preterm birth. During training, only 8% of those surveyed received education on the risks of delaying pregnancy. Those who were educated were significantly less likely to experience miscarriage or seek infertility evaluation or treatment. Compared with physicians in nonsurgical specialties, surgeons had fewer children, were older at first pregnancy, had more preterm births and fetal growth problems, and were more likely to be discouraged from starting a family during training and practice. CONCLUSIONS: Female physicians, particularly surgeons, have a significantly greater incidence of miscarriage, infertility, and pregnancy complications compared with the general population. The culture of medicine and surgery must continue to evolve to better support women with family planning during their training and careers.

Early postoperative feeding in single-stage repair of anorectal malformation with vestibular or perineal fistula is not associated with increased wound complications.

INTRODUCTION: The objective of this study is to assess the postoperative outcomes of single-stage repair of anorectal malformations with vestibular (VF) or perineal fistula (PF) and early initiation of postoperative feeding. METHODS: A retrospective review of patients undergoing single-stage repair of isolated low anorectal malformations (VF and PF) from 2017 to 2020 was conducted. All patients underwent an anterior anoplasty with complete mobilization of the rectal fistula, or posterior sagittal anorectoplasty (PSARP), without protective colostomy. The variables examined include age, timing of postoperative feeding initiation, length of stay (LOS), and complications. RESULTS: Nineteen patients with VF or PF underwent a single-stage repair. 12/19 (63%) patients were female. All 7 males and 9/12 females had a PF. The range of age at surgery was 2 days to 3 years with median age of 92 days [IQR 1,3: 9,193]. The median postoperative day for initiation of feeds was day 0 [IQR 1,3: 0,1] and median LOS was 1 day [IQR 1,3: 1,4.5]. 18/19 (95%) patients were evaluated in follow-up and there were no wound infections, wound dehiscences, or recurrent fistulas. Within 90 days postoperatively, no patients were seen in the emergency department for postoperative issues. Within 6 months, 2/19 (11%) patients required an unplanned return to the operating room for anal dilation. CONCLUSION: In single-stage repair of isolated low anorectal malformations, VF and PF, early initiation of postoperative feeding is safe, results in a short length of stay, and does not lead to increased wound complications. Early enteral feeding eliminates the need for parenteral nutrition and central venous access, and their associated complications. LEVEL OF EVIDENCE: Level IV.

Kidney donor profile index and post-transplant health care utilization: Implications for value of transplant care delivery.

BACKGROUND: Centers discard high kidney donor profile index (KDPI) allografts, potentially related to delayed graft function and prolonged hospital use by kidney transplant recipients (KTR). We sought to determine whether high KDPI KTRs have excess health care utilization. METHODS: We conducted a retrospective cohort study from a high-volume center analyzing KTRs from January 3, 2011 to April 12, 2015 (n = 652). We measured differences in hospital use, emergency visits, and outpatient visits within the first 90 days between low (≤85%) versus high KDPI (>85%) KTRs, as well as long-term graft function and patient survival. RESULTS: High (n = 107) and low KDPI (n = 545) KTRs had similar length of stay (median = 3 days, P = .66), and readmission rates at 7, 30, and 90 days after surgery (all, P > .05). High KDPI kidneys were not associated with excess utilization of the hospital, emergency services, outpatient transplant clinics, or ambulatory infusion visits on univariate or multivariate analysis (all, P > .05). Low KDPI KTRs had significantly better eGFR at 2 years (Low vs. High KDPI: 60.35 vs. 41.54 ml/min, P < .001), but similar 3-year patient and graft survival (both, P > .09). CONCLUSIONS: High and low KDPI KTRs demonstrated similar 90-day risk-adjusted health care utilization, which should encourage use of high KDPI kidneys.

Outcomes and Health Care Utilization After Early Hospital Dismissal in Kidney Transplantation: An Analysis of 1001 Consecutive Cases.

OBJECTIVE: To understand whether reduced lengths of stay after kidney transplantation were associated with excess health care utilization in the first 90 days or long-term graft and patient survival outcomes. BACKGROUND: Reducing length of stay after kidney transplant has an unknown effect on post-transplant health care utilization. We studied this association in a cohort of 1001 consecutive kidney transplants. METHODS: We retrospectively reviewed 2011-2015 data from a prospectively-maintained kidney transplant database from a single center. RESULTS: A total of 1001 patients underwent kidney transplant, and were dismissed from the hospital in 3 groups: Early [≤2 days] (19.8%), Normal [3-7 days] (79.4%) and Late [>7 days] (3.8%). 34.8% of patients had living donor transplants (Early 51%, Normal 31.4%, Late 18.4%, P < 0.001). Early patients had lower delayed graft function rates (Early 19.2%, Normal 32%, Late73.7%, P = 0.001). By the hospital dismissal group, there were no differences in readmissions or emergency room visits at 30 or 90 days. Glomerular filtration rate at 12 months and rates of biopsy-proven acute rejection were also similar between groups. The timing of hospital dismissal was not associated with the risk-adjusted likelihood of readmission. Early and Normal patients had similar graft and patient survival. Late dismissal patients, who had higher rates of cardiovascular complications, had significantly higher late mortality versus Normal dismissal patients in unadjusted and risk-adjusted models. CONCLUSION: Dismissing patients from the hospital 2 days after kidney transplant is safe, feasible, and improves value. It is not associated with excess health care utilization or worse short or long-term transplant outcomes.

Mentorship in pediatric surgery: A need for structure?

INTRODUCTION: Mentorship in surgical training is critical but differs greatly from the early apprenticeship model and often spans generations. This study evaluates the current state of and desire for structured mentorship in pediatric surgical training from the perspective of program directors (PDs) and trainees. METHODS: A survey addressing demographics, presence of or desire for structured mentorship, and proposed mentoring topics was emailed to pediatric surgery PDs (n = 58) and trainees completing fellowship in 2018-2020 (n = 72). RESULTS: The response rate was 38.5%. 50% of trainees were female versus 15% of PDs (p = 0.02). 19% of trainees reported having a structured mentorship program versus 26% of PDs (p = 0.72). The majority, 83%, of trainees felt a structured mentorship program is warranted versus 40% of PDs (p = 0.002). There were differing opinions between trainees and PDs regarding important components of a mentoring program. Trainees felt the following were more important: transition to practice, job negotiation, CV review, financial planning and performance review. PDs felt the following were more important: quality improvement projects and work/life balance. Both agreed academic development and job search were important. CONCLUSIONS: The majority of pediatric surgery trainees desire structured mentorship programs; however, few institutions have them. Training programs and program directors warrant a response to this gap. LEVEL OF EVIDENCE: IV.

A child presents with perianal symptoms - how often is this Crohn's disease?

BACKGROUND: The cumulative incidence and predictors of future diagnosis of Crohn's disease (CD) following presentation with perianal symptoms, such as anorectal abscess, fistula or fissure, is unknown. METHODS: A 5-year retrospective review of children presenting with perianal symptoms without prior CD diagnosis was performed. Institutional cumulative incidence of CD was calculated to determine the risk of CD presenting with perianal symptoms. RESULTS: 1140 children presented for evaluation of an anorectal abscess (n = 232), fistula (n = 49), or fissure (n = 859). Thirty-five were later diagnosed with CD, resulting in an incidence of 3%. Prognostic indicators of future CD diagnosis included increased age per every additional year (RR 1.19, 95% CI: 1.14-1.25, p < 0.001), male sex (RR 2.12, 95% CI 1.07-4.22, p = 0.024), or perianal fistula (RR 4.67, 95% CI 2.26-9.67, p = 0.022). Among those diagnosed with CD, 57% experienced and had a documented history of a CD-associated symptom prior to perianal symptom onset. Absence of symptoms resulted in delayed diagnosis (43 vs 3 days, p < 0.02). CONCLUSION: Of children presenting with a perianal symptom, three percent will eventually be diagnosed with CD. At highest risk (35%) were males aged 10 years or older with a perianal fistula; which should prompt expeditious workup.

Pleuroperitoneal (Denver) Shunt for Recurrent Nonmalignant Breast Seroma in a Metastatic Breast Cancer Patient.

Pleuroperitoneal (Denver) shunts have been used primarily for palliation of refractory malignant and chylous peritoneal and pleural collections.1-5 We used a pleuroperitoneal (Denver) shunt for a recurrent, nonmalignant breast seroma in the palliation of metastatic breast cancer as a novel use of this shunt.

Implementation of an Enhanced Recovery After Surgery (ERAS) Program is Associated with Improved Outcomes in Patients Undergoing Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy.

BACKGROUND: Cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC) has been associated with increased postoperative complications and a prolonged length of stay (LOS). We report on our experience following implementation of an Enhanced Recovery After Surgery (ERAS) program for CRS and HIPEC. METHODS: Patients were divided into pre- and post-ERAS groups. Modifications in the ERAS group included routine use of transversus abdominis plane blocks, intra- and postoperative fluid restriction, and minimizing the use of narcotics, drains, and nasogastric tubes. RESULTS: Of a total of 130 procedures, 49 (38%) were in the pre-ERAS group and 81 (62%) were in the ERAS group. Mean LOS was reduced from 10.3 ± 8.9 days to 6.9 ± 5.0 days (p = 0.007) and the rate of grade III/IV complications was reduced from 24 to 15% (p = 0.243) following ERAS implementation. The ERAS group received less intravenous fluid during hospitalization (19.2 ± 18.7 L vs. 32.8 ± 32.5 L, p = 0.003) and used less opioids than the pre-ERAS group (median of 159.7 mg of oral morphine equivalents vs. 272.6 mg). There were no significant changes in the rates of 30-day readmission or acute kidney injury between the two groups (p = non-significant). On multivariable analyses, ERAS was significantly associated with a reduction in LOS (- 2.89 days, 95% CI - 4.84 to - 0.94) and complication rates (odds ratio 0.22, 95% CI 0.08-0.57). CONCLUSIONS: Implementation of an ERAS program for CRS and HIPEC is associated with a reduction in overall intravenous fluids, postoperative narcotic use, complication rates, and LOS.

A Newly Described, Highly Prevalent Arterial Pedicle Perfuses Both Gracilis and Profunda Artery Perforator Flap Tissues: An Angiographic Study of the Medial Thigh.

BACKGROUND: Novel secondary flap options are paramount for patients who are not candidates for common reconstructive methods. The purpose of this study is to identify the prevalence of single arterial pedicle supplying both the gracilis muscle and medial thigh tissue carried in a profunda artery perforator (PAP) flap. Such a pedicle could allow the creation of a chimeric gracilis and PAP flap with a single-arterial anastomosis. METHODS: We conducted a retrospective review of 157 lower extremity computed tomography (CT) angiograms to assess the vasculature of the thigh soft tissues. Imaging evaluation was supervised by a board-certified musculoskeletal radiologist. RESULTS: Prevalence of a single-arterial pedicle to a gracilis and PAP flap in each patient was 59% (31% within the right leg and 28% in the left leg). Furthermore, 16% of patients had a common arterial pedicle in both lower extremities. CONCLUSION: Existence of a single-arterial pedicle to both the gracilis muscle and PAP flap tissues is frequently present in most patients in at least one lower extremity. This chimeric flap configuration could serve as a reconstructive avenue for patients, particularly those who have exhausted other more common flap options. Screening angiography is warranted in patients looking for this anatomic variation to establish its presence.

The Opioid Crisis and Surgeons: National Survey of Prescribing Patterns and the Influence of Motivators, Experience, and Gender.

BACKGROUND: Surgeons are the fifth largest prescribers of opioids in the US. Few studies exist to describe surgeon prescribing practices. METHODS: A survey was conducted of surgical providers at all ACGME-accredited surgical residency programs. Statistical comparisons between groups were made. RESULTS: A total of 114 providers from 21 states responded; 58% male, 57% residents. Only 8% reported being told they were over-prescribing opioids. Oxycodone ± acetaminophen was most commonly prescribed (49%). Median opioids prescribed exceeded guidelines for acute pain management for every procedure except laparoscopic appendectomy/cholecystectomy, lumpectomy and laparoscopic inguinal hernia repair. Attending surgeons more often gave no opioids after laparoscopic appendectomy/cholecystectomy (9% vs 0%; p=0.012), more likely reported patient attempts to return opioids to them (33% vs 16%; p=0.04), and less likely considered patients giving their opioids to someone else (39% vs. 74%; p<0.001). PGY 1-2 residents prescribe fewer opioids than advanced residents for simple mastectomy (p=0.04), exploratory laparotomy (p=0.05), and thoracotomy (p=0.03). CONCLUSIONS: Surgeons vary significantly in their opioid prescriptions, even for the same operation. There are few differences by gender but some important differences by experience.

Nuss procedure in the adult population for correction of pectus excavatum.

Minimally invasive repair of pectus excavatum has been successfully modified for use in adult patients. Many patients present in adulthood with progression of symptoms as the chest wall becomes less compliant with age. A thorough workup is completed including echocardiogram and chest CT to evaluate for anatomic abnormalities. Cardiopulmonary exercise testing is done to quantify the physiologic impact. Modifications of the original Nuss procedure required to allow for successful adult repair include the use of forced sternal elevation, the use of multiple bars, medial bar fixation, and interspace support to prevent bar rotation and migration. Occasionally, fractures may occur that require an open procedure and osteotomy or cartilage resection and hybrid approach incorporating the principals of intrathoracic support and osteotomy with bar stabilization.

Cardiopulmonary Function in Thoracic Wall Deformities: What Do We Really Know?

Patients with pectus excavatum (PE) frequently present with complaints of exercise intolerance and cardiopulmonary symptoms. There continues to be controversy regarding the physiologic benefits of repair. The aim of this review is to summarize and discuss recent data regarding the cardiopulmonary effects of PE deformity and the evidence for improvement obtained after surgical repair including (1) a greater efficiency of breathing (chest wall mechanics), (2) improvement in pulmonary restrictive deficits, (3) an increase in cardiac chamber size and output, with improved cardiac strain and strain rate, and (4) improvement in exercise capacity.

High Rates of Positive Severe Combined Immunodeficiency Screening Among Newborns with Severe Intestinal Failure.

PURPOSE: Severe combined immunodeficiency (SCID) screening by T-cell receptor excision circles (TREC) has been part of Massachusetts routine newborn screening since 2009. Tetratricopeptide repeat domain 7A gene (TTC7A) mutations responsible for hereditary multiple intestinal atresia with combined immunodeficiency (MIA-CID) were also recently identified. We reviewed newborn SCID screening among infants with intestinal failure and correlated results with patient characteristics and outcomes. METHODS: Records of infants with severe intestinal failure and available newborn screen results treated at a single center 2009-2016 were reviewed retrospectively. Patients with 1 or more positive SCID screens (<252 TREC copies/µL) were compared with those without positive screens. TREC copies/µL were compared with population norms. RESULTS: Of 70 included infants, 34% had newborn screens with TREC <252 copies/µL, compared with 0.3% of the general population; TREC levels for the cohort were lower than the general population (p<0.001). Of those with positive screens, 42% had prior or subsequent negative screening, 8% had no further workup, and 50% had flow cytometry showing: severe T-cell lymphopenia (absolute CD3+ <1500 cells/mcL) in 8, 3 of whom had TTC7A mutation-associated MIA-CID. Four had CD3+ >1500 cells/mcL. MIA-CID patients had the lowest serum citrulline in the cohort; 4 of the 8 patients with CD3+ <1500 cells/mcL on flow cytometry had newborn screening notable for severe hypocitrullinemia (<3 µM). CONCLUSION: Infants with intestinal failure have lower TREC copies/µL than the general population; one-third had levels concerning for SCID, and 11% were diagnosed with severe T-cell lymphopenia. The clinical implications and etiology of this phenomenon remain unknown, but may be related to hypocitrullinemia.

Morbidity and mortality among "big" babies who develop necrotizing enterocolitis: A prospective multicenter cohort analysis.

BACKGROUND: Necrotizing enterocolitis (NEC) is classically a disease of prematurity, with less reported regarding morbidity and mortality of this disease among other infants. METHODS: Data were prospectively collected from 2009 to 2015 at 252 Vermont Oxford Network member centers on neonates with birth weight>2500g admitted to a participating NICU within 28days of birth. RESULTS: Of 1629 neonates with NEC, gestational age was 37 (36, 39) weeks, and 45% had major congenital anomalies, most commonly gastrointestinal defects (20%), congenital heart defects (18%), and chromosomal anomalies (7%). For the 23% of infants who had surgery for NEC, mortality and length of stay were 23% and 63 (36, 94) days versus 8% and 34 (22, 61) days in medical NEC. Independent predictors of mortality were congenital heart defects (p<0.0001), chromosomal abnormalities (p<0.05), other congenital malformations (p<0.001), surgical NEC (p<0.0001), and sepsis (p<0.05). All of these in addition to gastrointestinal defects were independent predictors of increased length of stay. Nutritional morbidity at discharge included 6% receiving no enteral feeds and 27% who were <10th percentile weight-for-age. CONCLUSIONS: Major congenital anomalies are present in nearly half of >2500g birth weight infants diagnosed with necrotizing enterocolitis. Morbidity and mortality increase with sepsis, surgical disease, and congenital anomalies. TYPE OF STUDY: Prognosis Study. LEVEL OF EVIDENCE: Level II.

Ileostomy Prolapse in Children with Intestinal Dysmotility.

BACKGROUND: A relationship between intestinal motility and ileostomy prolapse has been suggested but not demonstrated objectively. AIMS: This study evaluated the association between ileostomy prolapse and intestinal dysmotility in children. METHODS: IRB-approved retrospective review of 163 patients with ileostomies (1998-2014) at a single institution. Patients were categorized as having clinical dysmotility as a primary diagnosis (n = 33), clinically suspected dysmotility based on underlying diagnosis (n = 60), or intestinal dysmotility unlikely (n = 70) at the time of ileostomy present. Intestinal manometry was categorized as normal (n = 13) or abnormal (n = 10). Primary outcome was pathologic stoma prolapse. Multivariate analysis using a logistic regression model and log-rank test to compare stoma prolapse rates over time between motility groups were used. RESULTS: Clinical diagnosis of dysmotility (p ≤ 0.001) and manometric findings of dysmotility (p = 0.024) were independently associated with stoma prolapse. Clinical dysmotility correlated with manometric findings (κ = 0.53). Prolapse occurred in 42% of patients with dysmotility, 34% of patients with suspected dysmotility, and 24% of patients with normal motility. One-year prolapse-free stoma "survival" was 45% for dysmotility, 72% for suspected dysmotility, and 85% for intestinal dysmotility unlikely groups (p = 0.006). CONCLUSIONS: Children with intestinal dysmotility are at great risk for stoma prolapse. Intestinal manometry could help identify these patients preoperatively.

Severe neurodevelopmental disability and healthcare needs among survivors of medical and surgical necrotizing enterocolitis: A prospective cohort study.

PURPOSE: This study characterizes neurodevelopmental outcomes and healthcare needs of extremely low birth weight (ELBW) survivors of necrotizing enterocolitis (NEC) compared to ELBW infants without NEC. METHODS: Data were collected prospectively on neonates born 22-27weeks' gestation or 401-1000g at 47 Vermont Oxford Network member centers from 1999 to 2012. Detailed neurodevelopmental evaluations were conducted at 18-24months corrected age. Information regarding rehospitalizations, postdischarge surgeries, and feeding was also collected. "Severe neurodevelopmental disability" was defined as: bilateral blindness, hearing impairment requiring amplification, inability to walk 10 steps with support, cerebral palsy, and/or Bayley Mental or Psychomotor Developmental Index <70. Diagnosis of NEC required both clinical and radiographic findings. RESULTS: There were 9063 children without NEC, 417 with medical NEC, and 449 with surgical NEC evaluated. Significantly higher rates of morbidity were observed among infants with a history of NEC. Those with surgical NEC were more frequently affected across all outcome measures at 18-24months corrected age: 38% demonstrated severe neurodevelopmental disability, nearly half underwent postdischarge operations, and a quarter required tube feeding at home. CONCLUSION: At 18-24months, extremely low birth weight survivors of necrotizing enterocolitis were at markedly increased risk (p<0.001) for severe neurodevelopmental disability, postdischarge surgery, and tube feeding. LEVEL OF EVIDENCE: II (prospective cohort study with <80% follow-up rate).