Malrotation: Management of Disorders of Gut Rotation for the General Surgeon.

In this article, we aim to provide the general surgeon with a clinical blueprint to navigate disorders of gut rotation. We emphasize that bilious emesis in a newborn is malrotation with volvulus until proven otherwise. Although an upper GI series can establish the diagnosis, surgical intervention should not be delayed until the child is ill-appearing. Following detorsion, the key steps are to broaden the mesentery, fully Kocherize the duodenum, and mobilize the cecum. If nonviable bowel is encountered, the principles of damage control can be applied to children. Every effort should be made to preserve bowel length.

Serrated Polyposis Syndrome in a Young Adolescent Patient.

Serrated polyps are pathological neoplastic lesions in the colon with subtle gross morphology leading to underreporting during colonoscopy. While detection rates are increasing in average-risk adult screening colonoscopy, the rate of detection during pediatric colonoscopy is unknown. Serrated polyposis syndrome is characterized by the presence of multiple serrated polyps in the colon and an increased risk of developing colorectal cancer. Cancer prevention relies on early recognition, endoscopic clearance of all polyps > 5 mm, and continued interval surveillance or prophylactic colectomy. We report the diagnosis and management of serrated polyposis syndrome in a young adolescent patient and highlight the subtle features of serrated polyps that may go unrecognized leading to underreporting in childhood.

Hindgut Duplication in an Infant with Omphalocele-Exstrophy-Imperforate Anus-Spinal Defects (OEIS) Complex.

Introduction The congenital anomaly of omphalocele, cloacal exstrophy, imperforate anus, and spinal abnormalities (OEIS complex) is rare but well recognized. Hindgut duplications are also uncommon and are not known to be associated with OEIS. We describe a neonate with OEIS who was found to have fully duplicated blind-ending hindguts. Case Report A premature infant boy with OEIS underwent first-stage closure on day of life 6, which included excision of the omphalocele sac, separation of the cecal plate and bladder halves, tubularization of the cecal plate, hindgut rescue with end colostomy, and joining of the bladder halves. Cecal plate inspection revealed two hindgut structures that descended distally, one descended midline into the pelvis along the sacrum and the second laterally along the left border of the sacrum. Both lumens connected to the cecal plate and had separate mesenteries. In an effort to maximize the colonic mucosal surface area, the hindgut segments were unified through a side-to-side anastomosis, creating a larger caliber hindgut. The cecal plate was tubularized and an end colostomy was created. Bowel function returned and he was discharged home on full enteral feeds. Discussion This case represents a cooccurrence of two extremely rare and complex congenital anomalies. The decision to unify the distinct hindguts into a single lumen was made in an effort to combine the goals of management for both OEIS and alimentary duplications. The hindgut is abnormal in OEIS and should be assessed carefully during repair.

Quality of dictated feedback associated with SIMPL operative assessments of pediatric surgical trainees.

BACKGROUND: SIMPL is a workplace-based operative performance assessment tool which allows for dictated feedback (DF). To better understand the value of DF, we sought to characterize the type and quality of DF generated during SIMPL evaluations. METHODS: Thematic analysis of DF from SIMPL assessments between June 2017 and December 2018 at a single pediatric surgery fellowship program was performed. Comments were categorized as specific, encouraging or corrective. Categories were combined to determine DF quality as effective, mediocre or ineffective. RESULTS: Of 781 SIMPL assessments (21 faculty, 5 trainees), 451 (57%) had DF. Most comments were encouraging (93%) and specific (65%). Only 21% were corrective, 17% had entrustment features, and 8% had an explicit learning plan. Feedback quality was deemed mediocre (45%), ineffective (33%) and effective (21%). CONCLUSION: SIMPL dictated feedback was mostly encouraging and specific. To improve quality, feedback should incorporate learning plans as well as corrective and entrustment features.

A Surgical View of Anatomy: Perspectives from Students and Instructors.

As curricular emphasis on anatomy in undergraduate medical education continues to evolve, new approaches to anatomical education are urgently needed to prepare medical students for residency. A surgical anatomy class was designed for third- and fourth-year medical students to explore important anatomical relationships by performing realistic surgical procedures on anatomical donors. Under the guidance of both surgeons and anatomists, students in this month-long elective course explored key anatomical relationships through performing surgical approaches, with the secondary benefit of practicing basic surgical techniques. Procedures, such as left nephrectomy, first rib resection for thoracic outlet syndrome, and carotid endarterectomy, were adapted from those used clinically by multiple surgical subspecialties. This viewpoint commentary highlights perspectives from students and instructors that suggest the value of a surgical approach to anatomical education for medical students preparing for procedure-oriented residencies, with the goals of: (1) describing the elective at the authors' institution, (2) promoting similar efforts across different institutions, and (3) encouraging future qualitative and quantitative studies of similar pedagogic efforts.

Safety of delayed surgical repair of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) complex in infants with significant comorbidities.

PURPOSE: Management of infants with OEIS complex is challenging and not standardized. Expeditious surgery after birth has been recommended to limit soilage of the urinary tract and optimize intestinal function. However, clinical instability secondary to comorbidities is common in this population and early operation carries risk. We sought to define the risk/benefit profile of delaying repair. METHODS: All newborn patients with OEIS managed by our institution between Sep 2017 and Oct 2019 were reviewed. Comorbidities were evaluated, including cardiopulmonary pathologies and associated malformations. RESULTS: Ten patients with OEIS were managed. Patients underwent early (2 patients, repair at 0-2 days) or delayed (6 patients, repair at 6-87 days) first-stage exstrophy repair. Two patients died prior to repair (progressive respiratory failure, severe genetic anomalies). Repairs were delayed secondary to cardiac conditions, neurosurgical interventions, medical disease, and/or delayed transfer. Delayed repair patients had longer lengths of stay and use of parenteral nutrition. No patients experienced urinary tract infections prior to repair. CONCLUSIONS: Delaying first-stage exstrophy repair to allow physiologic optimization is safe. All repaired patients were discharged home, without parenteral nutrition or supplemental oxygen.

Assessment of Operative Autonomy and Readiness for Independent Practice Among Pediatric Surgery Fellows.

PURPOSE: The purpose of this study was to evaluate the implementation of the System for Improving and Measuring Procedural Learning (SIMPL) "app" in a pediatric surgery training program. SIMPL is a novel workplace-based operative performance assessment platform which allows case by case assessment of trainees using three scales (autonomy, performance, and case complexity) and takes seconds to complete. METHODS: A pediatric surgery-specific procedural taxonomy for SIMPL was developed from the ACGME procedural library. SIMPL was piloted and implemented in a single pediatric surgery training program. A descriptive analysis of the operative assessments was undertaken. RESULTS: In the year following SIMPL implementation, 565 (39% of cases) assessments were completed by 21 faculty for 3 fellows for 148 unique procedures within a median of 8.5 h. Recorded feedback accompanied 61% of assessments. 2nd year fellows were more likely to be deemed autonomous (81% ratings) and practice ready (84%) vs. 1st year fellows (50%; p < 0.001 both), with improvements over time. CONCLUSION: Within pediatric surgery, this is the first implementation of a workplace-based operative performance assessment "app". With its ease of use, SIMPL drastically increased the volume of operative evaluations and diversified the mix of cases evaluated while SIMPL ratings demonstrated expected and graduated performance improvements over time. TYPE OF STUDY: Education. LEVEL OF EVIDENCE: N/A.

Technique and perioperative management of left pneumonectomy in neonatal piglets.

BACKGROUND: Although commonly performed in adult swine, unilateral pneumonectomy in piglets requires significant modifications in the surgical approach and perioperative care because of their smaller size and limited physiological reserve. METHODS: Nineteen neonatal piglets underwent a left pneumonectomy. They were allowed 5-7 d of preoperative acclimation and nutritional optimization. Preoperative weight gain and laboratory values were obtained before the time of surgery. A "ventro-cranial" approach is adopted where components of the pulmonary hilum were sequentially identified and ligated, starting from the most ventral and cranial structure, the superior pulmonary vein. The principle of gentle ventilation was followed throughout the entire operation. RESULTS: The median age of the piglets at the time of surgery was 12 (10-12) d. The median preoperative weight gain and albumin level were 20% (16-26%) and 2.3 (2.1-2.4) g/dL, respectively. The median operative time was 59 (50-70) min. Five of the first nine piglets died from complications, two from poor preoperative nutritional optimization (both with <10% weight gain and 2 g/dL for albumin), one from an intubation complication, one from intra-operative bleeding, and one in the postoperative period from a ruptured bulla. No mortality occurred for the next 10 cases. CONCLUSIONS: Successful outcomes for unilateral pneumonectomy in piglets require special attention to preoperative nutritional optimization, gentle ventilation, and meticulous surgical dissection. Preoperative weight gain and albumin levels should be used to identify appropriate surgical candidates. The "ventro-cranial" approach allows for a technically straightforward completion of the procedure.

The effort and outcomes of the Pediatric Surgery match process: Are we interviewing too many?

PURPOSE: Increasing numbers of programs participating in the pediatric surgery match has resulted in economic and logistical issues for candidates, General Surgery residencies, and Pediatric Surgery training programs (PSTP). We sought to determine the ideal number of interviews conducted by programs based on resultant rank order lists (ROL) of matched candidates. METHODS: PSTPs received 4 online surveys regarding interview practices (2011-2012, 2014), and matched candidate ROL (2008-2010, 2012, 2014). Program directors (PD) also provided estimates regarding minimum candidate interview numbers necessary for an effective match (2011-2012, 2014). Kruskal-Wallis equality-of-populations rank tests compared ROL and interview numbers conducted. Quartile regression predicted ROL based on the interview numbers. Wilcoxon signed rank-sum tests compared the interview numbers to the minimal interview number using a matched pair. p Values<0.05 were significant. RESULTS: Survey response rates ranged from 85-100%. Median ROL of matched candidates (2-3.5) did not differ between programs (p=0.09) and the lowest matched ROL for any year was 10-12. Interview numbers did not affect the final candidate ROL (p=0.22). While PDs thought the minimum median interview number should be 20, the number actually conducted was significantly higher (p<0.001). CONCLUSION: These data suggest that PSTPs interview excessive numbers of candidates. Programs and applicants should evaluate mechanisms to reduce interviews to limit costs and effort associated with the match.

Passing the baton: The pediatric surgical perspective on transition.

BACKGROUND/PURPOSE: Transition of care from pediatric to adult providers is garnering intense national interest in pediatrics but is largely driven by nonsurgeons. We sought to describe the preferences of pediatric surgeons and their current transition practice patterns for various populations and wondered whether gender, experience, or practice setting affected surgeons' perspectives. METHODS: All American Pediatric Surgical Association (APSA) members were surveyed electronically. Responses were voluntary and confidential. RESULTS: Survey response rate was 22%. Most surgeons (95%) treated patients up to 22 years of age, and many cared for older patients (23-26 years, 49%; 27-30 years, 25%; 31-40 years, 18%; >40 years, 13%). Developmentally delayed adults comprised a significant portion of this population (54%). Congenital conditions such as Hirschsprung disease (25%), anorectal malformations (22%) and cystic fibrosis (22%) were most commonly reported. About half (52%) of respondents reported institutional limits for age. Lack of qualified adult surgeons was felt to be the greatest barrier to transition (p<0.001). Experience, gender and practice type did not significantly affect opinions of transition. CONCLUSIONS: Pediatric surgeons care for adult patients with a variety of surgical conditions, particularly those with developmental delay and congenital anomalies. The perception of a deficit of qualified adult providers warrants further exploration. Understanding the barriers to surgical transition represents a critical step in improving the quality and appropriateness of care transitions.

Predictive value of the Pediatric Ulcerative Colitis Activity Index in the surgical management of ulcerative colitis.

PURPOSE: The primary purpose of this study was to investigate the relationship between Pediatric Ulcerative Colitis Activity Index (PUCAI) and operative management. We also specifically evaluated those patients receiving tacrolimus for their disease. METHODS: A retrospective review (1/06-1/11) identified ulcerative colitis patients (≤21 years old) undergoing restorative proctocolectomy with rectal mucosectomy and ileal pouch-anal anastomosis. Main outcomes included pre-operative PUCAI, combined versus staged procedure, and postoperative complications. Patients receiving tacrolimus within 3 months of surgical intervention were identified. PUCAI at tacrolimus induction and medication side effects were also noted. RESULTS: Sixty patients were identified. Forty-two (70%) underwent combined and 18 (30%) had staged procedures. Pre-operative PUCAI was lower for combined versus staged patients (p = < 0.001). Furthermore, a higher pre-operative PUCAI strongly correlated with the likelihood of undergoing a staged procedure (p < 0.001). Forty-four patients (73%) received tacrolimus. Significant improvement in their PUCAI was noted from induction to pre-operative evaluation (p < 0.001). Minor and reversible side effects occurred in 46% of patients receiving tacrolimus, but complication rates were not significantly different. CONCLUSIONS: There is a very strong correlation between the PUCAI and the likelihood of undergoing a staged procedure. A significant improvement in PUCAI occurs following preoperative tacrolimus therapy.

Comparison of laparoscopic-assisted and open total proctocolectomy and ileal pouch anal anastomosis in children and adolescents.

BACKGROUND: Laparoscopic techniques have been applied to restorative proctocolectomy since the early 2000's. We have employed a technique for laparoscopic-assisted total proctocolectomy (TPC) and ileal pouch anal anastomosis (IPAA) for the treatment of children with ulcerative colitis (UC). METHODS: We retrospectively reviewed 68 laparoscopic-assisted TPCs and 39 open TPCs performed at our institution for UC between January 1997 and February 2011. Case duration, postoperative length of stay, and complications of the two groups were compared, and multivariable analysis was applied. RESULTS: The two groups were comparable with respect to gender, age, and postoperative length of stay. Total abdominal colectomy (TAC) duration was significantly longer in the laparoscopic-assisted group (P < .001). Complications were similar in the laparoscopic and open group, although small bowel obstruction (SBO) was significantly less frequent in the laparoscopic group (log-rank test = 8.88, P = .003). Kaplan-Meier estimated freedom from SBO at 1 year follow-up is 99% for patients treated laparoscopically (95% CI: 98%-100%) and 76% for those undergoing an open surgical approach (95% CI: 64%-88%). CONCLUSIONS: The significantly lower SBO rate, low complication rates, and equivalent length of stay favor use of the laparoscopic-assisted approach for TPC and IPAA in children.

Technique of laparoscopic-assisted total proctocolectomy and ileal pouch anal anastomosis in children and adolescents: a single center's 8-year experience.

BACKGROUND: Laparoscopic techniques have been applied to restorative proctocolectomy since the early 2000s. We have employed a technique for laparoscopic-assisted total proctocolectomy (TPC) and ileal pouch anal anastomosis (IPAA) for the treatment of children with ulcerative colitis (UC). METHODS: A total of 68 children underwent laparoscopic-assisted TPCs at our institution from June 2003 to February 2011. The patients ranged from 5 to 22 years of age. We progressively modified our surgical approach in positioning, equipment use, and operative technique. RESULTS: A total of 68 children underwent laparoscopic-assisted TPC within the 8-year time period. Two major complications occurred (2/68=3%) consisting of 1 duodenal injury and 1 small bowel injury. There were 6 minor complications (6/68=9%), including 5 stoma revisions and 1 port site hernia. There were no superficial wound infections. There were no reported cases of pouch fistulas, anastomotic breakdown, or neurovascular injuries. CONCLUSIONS: Our surgical technique is safe and effective in treating children with UC.

Risk adjustment for neonatal surgery: a method for comparison of in-hospital mortality.

OBJECTIVE: To develop a risk-adjustment method for evaluation of in-hospital mortality after noncardiac neonatal surgery regardless of gestational age. METHODS: Infants ≤ 30 days old undergoing noncardiac surgical procedures were identified by using the Kids' Inpatient Database (KID) 2000 + 2003. Neonates were included regardless of gestational age. International Classification of Disease, Ninth Revision, Clinical Modification codes were used to assign procedures to 1 of 4 previously derived risk categories. Prematurity and other clinical variables were assessed in logistic regression analysis. The final multivariable model was validated in 3 independent data sets: KID 2006, Pediatric Health Information System (PHIS) 2001-2003, and PHIS 2006-2008. The model was applied to generate standardized mortality ratios for institutions within PHIS 2006-2008. RESULTS: Among 18437 eligible cases in KID 2000 + 2003, 15278 (83%) had 1 of 66 procedure codes assigned to a risk category and were eligible for analysis. In-hospital mortality for premature infants was 10.5% compared with 2.0% for full-term neonates. In addition to risk category, the clinical variables improving prediction of in-hospital death were prematurity, serious respiratory conditions, necrotizing enterocolitis, neonatal sepsis, and congenital heart disease. Area under the receiver-operator characteristic curve for the final model was 0.90. The model also showed excellent discrimination in the 3 validation data sets (0.90, 0.89, and 0.89). Within 41 institutions in PHIS, standardized mortality ratios ranged from 0.37 to 1.91. CONCLUSIONS: This validated method provides a tool for risk adjustment of neonates undergoing noncardiac surgery to allow comparative analyses of in-hospital mortality.

Combined en bloc liver-double kidney transplantation in an infant with IVC thrombosis.

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.

Prospective study of health-related quality of life and restorative proctocolectomy in children.

PURPOSE: Although restorative proctocolectomy has become the standard surgical treatment for ulcerative colitis and familial adenomatous polyposis, there are no prospective studies in children of the impact of this intervention on health-related quality of life. METHODS: A prospective study of health-related quality of life in children with ulcerative colitis or familial adenomatous polyposis undergoing restorative proctocolectomy was performed. Patients and their parents who agreed to participate completed standardized health-related quality-of-life surveys (Medical Outcomes Study Short Form-36 and Child Health Questionnaire Parent Form) within one month before colectomy and approximately one year after completion of their surgery. RESULTS: Of the 60 patients who agreed to participate, 44 completed surveys at the appropriate time periods allowing comparison. The parents of 28 of these subjects also completed paired surveys. Before colectomy, patients with ulcerative colitis had substantially lower health-related quality-of-life scores, which were also mirrored in parental surveys. Following surgery patients with ulcerative colitis had significant improvement in 7 of 8 Medical Outcomes Study Short Form-36 patient subscales and all 6 corresponding Child Health Questionnaire Parent Form parental subscales of health-related quality of life. Patients with familial adenomatous polyposis showed improvement in the bodily pain subscale alone, whereas their corresponding parental surveys only showed improvement in the mental health subscale. CONCLUSION: There are clearly significant adverse affects on health-related quality of life in children with ulcerative colitis that dramatically improved following restorative proctocolectomy.

A risk adjustment method for newborns undergoing noncardiac surgery.

OBJECTIVE: To develop a risk adjustment method for in-hospital mortality in newborns undergoing noncardiac surgery. SUMMARY OF BACKGROUND DATA: Understanding variation in outcomes is critical to guide quality improvement. Reliable outcome assessments need risk adjustment to allow comparisons. METHODS: Infants or=20 cases in the data set were placed into 4 risk categories by in-hospital mortality rates. Clinical variables were added to the model to better predict mortality; areas under the receiver-operator characteristic (ROC) curves were compared. The final model was validated in the KID 2003 database. RESULTS: Among 6103 eligible cases in the KID 2000, 5117 (83.8%) could be assigned to a risk category. Mortality rates were 0.2% in risk category 1, 2.5% in category 2, 6.4% in 3, and 18.4% in 4. The odds of mortality increased in each risk category relative to category 1 (P < 0.001 for each). In multivariable models adjusting for risk category, the clinical variables most predictive of in-hospital death were serious respiratory conditions and necrotizing enterocolitis. The area under the ROC curve for the full model including clinical risk factors was 0.92 in the KID 2000. The model was validated using data for KID 2003 and showed excellent discrimination (ROC = 0.90). CONCLUSION: This validated method provides a means of risk adjustment in groups of newborns undergoing noncardiac surgery, and should allow for comparative analyses of in-hospital mortality.

Restorative proctocolectomy and ileal pouch-anal anastomosis in children.

PURPOSE: This study was designed to evaluate the results of restorative proctocolectomy with distal rectal mucosectomy and ileal pouch-anal anastomosis in children. METHODS: This study is a retrospective review of 100 consecutively referred children (<18 years old) who underwent reconstruction with a J-pouch of ileum and preservation of the transitional anorectal epithelium by the same two-surgeon team. Temporary diverting ileostomy was used. The main outcome measures were daytime and nocturnal fecal continence, bowel movements per day, and complications including pouchitis, ileoanal stricture, or postoperative small-bowel obstruction. RESULTS: Average age of the 100 children (48 males/52 females) was 13.2 years (range, 2.95-17.99). All 25 children with familial adenomatous polyposis had proctocolectomy and reconstruction performed simultaneously. Of 75 children with ulcerative colitis, 50 (67%) had their colectomy followed by reconstruction after an interval ranging from 2 months to 4.4 years. Median postoperative follow-up was 2.6 years. Daytime fecal continence was achieved in 98 children, although 4 reported rare accidents. Nighttime continence was achieved in 93 children, of whom 14 reported rare accidents. The average frequency of bowel movements was 5.43/day (+/-2.22). Only one child with polyposis had pouchitis. Of 75 children with ulcerative colitis, 35 had symptoms consistent with pouchitis; of these 35 children, 10 required prolonged treatment. The most frequent postoperative complication was ileoanal stricture requiring operative dilatation and/or anoplasty (18 children). Bowel obstruction requiring surgery occurred in 18 children. One child eventually required pouchectomy for probable Crohn's disease. CONCLUSIONS: Excellent results can be achieved with restorative proctocolectomy in children with respect to fecal continence and stool frequency. However, with ulcerative colitis, a substantial risk of pouchitis remains.

Mesenteric lipoblastoma presenting as a segmental volvulus.

Mesenteric lipoblastoma is a rare tumor and, its presentation as a bowel obstruction with possible midgut volvulus has only been reported once before. A 7-year-old girl presented with nausea and vomiting but a benign abdominal examination. Upper gastrointestinal contrast study demonstrated possible malrotation with midgut volvulus. During emergency laparotomy, segmental small bowel volvulus secondary to a large mesenteric lipoblastoma was found. The lipoblastoma was resected with a segment of small bowel. Resection margins were negative for tumor, and the patient is doing well with no evidence of recurrence.