Clinical relevance of inflammation on rectal biopsy for Hirschsprung disease: An outcomes analysis.

OBJECTIVES: Inflammation on diagnostic rectal biopsy for children with suspected Hirschsprung disease (HSCR) is reported on pathology, and its significance is unknown. We describe the management and outcomes of a cohort with inflammation on rectal biopsy compared to those without. Specifically, to address the hypothesis that inflammation on diagnostic biopsy is associated with increased complication rates irrespective of intervention type and timing. METHODS: A single institution retrospective review of children with HSCR who underwent biopsy and endorectal pull-through (ERPT) from 2010 to 2020 was performed. The primary outcome was overall complications at 30-days following ERPT. Secondary outcomes included timing and type of operative intervention as well as postoperative enterocolitis diagnosed within 6-months of ERPT. RESULTS: Forty-nine children were identified; inflammation was present on diagnostic biopsy for 17 children. Those with inflammation were more likely to have clinical evidence of enterocolitis at the time of biopsy (p = 0.001) and were more likely to undergo leveling colostomy before ERPT (p = 0.01). Children with inflammation had a higher anastomotic leak rate (p = 0.04). Subgroup analysis of patients with inflammation undergoing primary ERPT versus leveling colostomy demonstrated no significant difference in outcomes following definitive ERPT. CONCLUSIONS: Our study suggests inflammation on diagnostic rectal biopsy for HSCR is associated with increased anastomotic leak rates. While additional prospective studies are indicated, attention to methods of mitigating inflammation and confirming its resolution before definitive pull-through may be of benefit for improving clinical outcomes in patients found with inflammation on diagnostic rectal biopsy.

Functional Outcomes of Patients Who Underwent Anorectal Malformation Repair Using MRI Guidance.

BACKGROUND: Despite the initiation of minimally invasive laparoscopic techniques, the majority of patients who undergo anorectal malformation repair still experience functional bowel issues in childhood, including constipation and fecal incontinence. In this study, we evaluate the functional outcomes of a procedure in which magnetic resonance imaging guidance is used during initial laparoscopic repair to better locate the epicenter of the sphincter muscle complex and pelvic floor with the goal of more accurate placement of the neoanus and improved functional outcomes. METHODS: A retrospective chart review evaluated demographic, operative, and outcome details for patients who underwent this procedure. A telephone survey was employed to determine levels of social continence using the validated Baylor Continence Scale and to determine what type of bowel management is used. RESULTS: Twenty-six patients were included. Median age at operation was 7 months, and median age at follow-up was 4 years old, with a range of 1-9. Bowel management regimen results revealed that 19 % (n = 5) use no bowel management regimen, 58 % (n = 15) use laxatives only, and 23 % (n = 6) use enemas. Enema use was not associated with different spine or sacral anomalies (p = 0.77). Fifteen patients (58 %) answered the Baylor Continence Scale questions and had a median score of 14. No difference was found in scores when accounting for lesion level (p = 0.43), quality of needle placement (p = 0.46), or quality of sphincter muscles (p = 0.75). CONCLUSIONS: Using MRI guidance in the repair of anorectal malformations shows promise in both the qualitative and quantitative functional outcomes of this complex patient population. LEVEL OF EVIDENCE: Level III.

Ultrasound-Guided Epigastric Hernia Repair in the Pediatric Population: One Institution's Experience and Outcomes.

Introduction: Epigastric hernia repair is a common procedure performed on pediatric patients. These hernias have been demonstrated to be readily identified by ultrasound. This manuscript describes and assesses a novel technique in which ultrasound is used to identify and repair the hernias in a minimally invasive manner. Methods: A retrospective chart review evaluates all the ultrasound-guided epigastric hernia repairs done with prolene suture at one institution over 20 months. Operative details and postoperative complications were compared to patients who underwent traditional open epigastric hernia repair at the same institution during the same time frame. Results: Thirty-two pediatric patients underwent epigastric hernia repair between May 2021 and December 2022. Thirteen (41%) underwent the ultrasound-guided technique with prolene suture and using only a meniscus needle and no incision. Nineteen (59%) underwent standard open repair. There were no recurrences or postoperative complications in either group. The average operative time for the ultrasound hernia repair was 24.4 minutes, which was shorter than the average operative time of 33.6 minutes for the open repair (P = .08). In 3 ultrasound cases (23%), an additional epigastric hernia that had not been clinically apparent was identified and simultaneously repaired. Conclusions: Ultrasound-guided epigastria hernia repair is a feasible alternative to traditional open repair. It has a comparable safety and efficacy profile, has a short operative time, and has the advantages of being minimally invasive and having the ability to identify and repair concurrent epigastric hernias.

Ultrasound-Guided Enteropexy for Repair of Ostomy Prolapse.

Background: Enterostomies provide fecal diversion for numerous conditions, but anatomical complications-prolapse, stricture, and retraction-occur in up to 25%. Given up to 76% of these complications require surgical intervention, effective minimally invasive repair techniques for their management are needed. This article describes a new technique for prolapse repair utilizing image-guided surgery for incisionless repair of ostomy prolapse. Methods: To perform the procedure, the prolapsed bowel is reduced and evaluated for feasibility for ultrasound repair. Under direct ultrasound guidance sutures are used to pexy the bowel loop to the overlying fascia. Sutures are tied with knots and sutures buried below the skin to securely tack the bowel to the abdominal wall. Results: Four patients aged 2-10 years underwent ultrasound-guided enteropexy for repair of major prolapse of an end ileostomy (2 patients), loop colostomy, and end colostomy. All patients remained free of major prolapse for 3-10 months after the procedure, 2 of whom have progressed to ostomy takedown without complications. Conclusions: Ultrasound-guided enteropexy is a noninvasive effective way to manage ostomy prolapse.

Ultrasound assessment of abdominal adhesions in neonates: data over dogma for re-operative timing.

INTRODUCTION: Neonatal abdominal reoperation is difficult and can be complicated by abdominal adhesions. Identifying patients who could safely undergo early reoperation would save TPN and central line days, decrease associated infection and liver injury, and NICU and hospital length of stay. We sought to determine if ultrasound (US) could accurately assess the location and severity of adhesions in neonates as an objective dynamic marker capable of informing reoperation timing. METHODS: After IRB approval, we conducted a prospective observational study including neonates undergoing abdominal operations. Patients received surgeon-performed US approximately every 2 weeks until reoperation or discharge. Adhesions were assessed in five zones: right upper quadrant (RUQ), right lower quadrant (RLQ), left upper quadrant (LUQ), left lower quadrant (LLQ) and peri-incision (INC). RESULTS: Over a 6-month study period, 16 neonates were enrolled. Median gestational age was 34 weeks at birth and median weight 2.2 kg. 6 underwent reoperation within initial NICU admission. At time of operation US correctly identified the absence or presence and severity of adhesions in: RUQ (3/3); RLQ (6/6); LUQ (4/5); LLQ (6/6); and INC (5/5). CONCLUSION: US can identify location and severity of post-operative adhesions in neonates, potentially identifying patients who can safely undergo reoperation earlier than predetermined wait periods. LEVEL OF EVIDENCE: IV.

Do preoperative factors predict success of antegrade continence enemas in children?

INTRODUCTION: Antegrade continent enemas (ACE) procedures are one treatment option in children with medically refractory constipation or encopresis and predicting success is difficult. We hypothesize that there are preoperative factors that can be identified to help with patient selection and family counseling. METHODS: We conducted a retrospective study of children who underwent a cecostomy or appendicostomy for an ACE program between 2015 and 2021. Underlying diagnosis, pre-operative bowel regimen and imaging were analyzed. Patients were reviewed for success at 3-, 6- and 12-months post-procedure. Data was analyzed with Fisher's Exact, Kruskal-Wallis and logistic regression where applicable with significance defined as p < 0.05. RESULTS: Forty-three children were identified; 28 were male, 15 were female, mean age at time of operation was 8 years old. 76% were considered successful at 3-months, 86% at 6-months, and 87% at 12-months post- procedure. Univariate analysis showed that a pre-ACE retrograde enema program predicted success at 3-months (94% vs. 64% p = 0.03) but no difference at 6- or 12-months. At one year after ACE procedure there was a significant reduction in number of enteral medications (2 to 0, p < 0.01) and 94% of patients were on one or fewer at one year follow-up. Age, gender, weight at time of operation, contrast enema, anorectal manometry and colonic transit time results were not predictive of outcomes. CONCLUSION: In this study, we characterized expected time to success in our population as well as identified use of a pre-operative retrograde enema program as a potential predictor of success at 3-months in children undergoing an ACE procedure. LEVEL OF EVIDENCE: IV. TYPE OF STUDY: Prognosis study.

Ultrasound-Guided Gastrostomy Tube Placement: An Evaluation of Postoperative Complications in the Pediatric Population.

Background: Using ultrasound guidance has been demonstrated as a feasible alternative method for gastrostomy tube placement in the pediatric population. The aim of this study is to evaluate short- and long-term postoperative complications after ultrasound-guided gastrostomy tube placement (USGTP) and to compare them with complications after laparoscopic gastrostomy tube placement (LGTP). Methods: A retrospective chart review evaluated patients who underwent USGTP (n = 41) and LGTP (n = 120) at the same institution. Comparisons were made between the two groups in the context of demographics as well as 30-day and 6-month postoperative complications. A phone survey (n = 26) further identified USGTP complications potentially not captured in the electronic medical records. Results: There were no significant differences in age, gender, and indication for procedure between the two groups. Chart review revealed that USGTP and LGTP had statistically comparable rates of emergency department (ED) visits for postoperative complications. Among USGTP patients, 8% had a recorded ED visit within 30 days of the operation and 13% presented to the ED within 6 months, compared with 6% and 11%, respectively, in the LGTP group (P = .65, P = .69). The USGTP phone survey reported total complications over an average postoperative follow-up time of 34.6 months (range 8-87) and revealed a total ED visit rate of 35%, which is comparable with rates reported in the literature for minimally invasive feeding tube placement. Conclusion: USGTP is a safe and feasible alternative option for gastrostomy tube placement in the pediatric population and it has postoperative complication rates that are comparable with LGTP.

An Alternative Approach to Single-Stage Cloaca Repair: Image-Guided Anorectoplasty with Delayed Vaginoplasty.

BACKGROUND: Cloacal anomalies occur when a fetus's rectum, vagina, and urethra fail to separate and result in 1 common channel at birth. They are commonly managed by complex reconstruction in the first year of life. This manuscript presents an alternative approach to management in patients with absent or nondilated Mullerian structures. CASE: Image-guided, combined endoscopic and laparoscopic surgery (CELS) was used to perform an anorectal pull-through at 5 months of age on a patient with persistent cloaca and no definite vaginal or uterine structures seen on MRI and endoscopy. Urogenital reconstruction is delayed until adolescence. SUMMARY AND CONCLUSION: We hypothesize that performing a minimally invasive anorectoplasty on patients with complicated anatomy and low risk for hydrocolpos could potentially result in improved urologic function and better psychosocial outcomes. Delaying vaginoplasty will enable determination of the function of remnant Mullerian structures and allow the patient to direct the augmentation approach.

Ultrasound-guided pediatric inguinal hernia repair.

PURPOSE: Inguinal hernias are amongst the most common surgical conditions in children. Typically, these repairs are performed through an open or laparoscopic approach, using a high ligation of the hernia sac. The use of ultrasound has been described in identifying and evaluating hernia contents in children. Our goal was to determine if ultrasound guidance could be used to perform a high ligation of the hernia sac in pediatric patients. METHODS: Following IRB approval, a retrospective review of all female patients at a single center undergoing ultrasound guided inguinal hernia repair between 2017 and 2018 was performed. Pre-operative characteristics, intra-operative outcomes, and post-operative outcomes were all evaluated. Laparoscopy was used to evaluate the repair and evaluate for a contralateral hernia. Male patients did not undergo ultrasound inguinal hernia repair to avoid damage to the vas deferens and vessels. RESULTS: A total of 10 patients with 13 hernias total were found during the study period. A total of one patient was converted to a laparoscopic repair. No patients were found to have an inappropriate repair or a missed contralateral hernia, and there were no vascular injuries or injuries to surrounding structures. No patients had a hernia recurrence during the study period. CONCLUSION: This study demonstrates the safety and feasibility of ultrasound guided inguinal hernia repairs in female pediatric patients. Further study is needed to compare these repairs to existing techniques, evaluate for recurrences over time, and evaluate if these repairs can be performed without general anesthetic in some patients.

Fetal Surgery in the Primate 4.0: A New Technique 30 Years Later.

INTRODUCTION: Open fetal surgery requires a hemostatic hysterotomy that minimizes membrane separation. For over 30 years, the standard of care for hysterotomy in the gravid uterus has been the AutoSuture Premium Poly CS*-57 stapler. OBJECTIVE: In this study, we sought to test the feasibility of hysterotomy in a rhesus monkey model with the Harmonic ACE®+7 Shears. METHODS: A gravid rhesus monkey underwent midgestation hysterotomy at approximately 90 days of gestation (2nd trimester; term = 165 ± 10 days) using the Harmonic ACE®+7 Shears. A two-layer uterine closure was completed and the dam was monitored by ultrasound intermittently throughout the pregnancy. At 58 days after hysterotomy (near term), a final surgery was performed to evaluate the uterus and hysterotomy site. RESULTS: A 3.5-cm hysterotomy was completed in 2 min 7 s. The opening was hemostatic and the membranes were sealed. Immediately after closure and throughout the pregnancy, ultrasound revealed intact membranes without separation and normal amniotic fluid levels. At term, the scar was well healed without signs of thinning or dehiscence. CONCLUSIONS: The Harmonic ACE®+7 Shears produced a hemostatic midgestation hysterotomy with membrane sealing in the rhesus monkey model. Importantly, healing was acceptable.

CT validation of SVC-RA junction location for pediatric central line placement: is vertebral bodies below the carina accurate?

PURPOSE: The key landmark for tip position of a central venous catheter (CVC) is the SVC-RA junction. In adults, localization of the SVC-RA junction may be assessed as a function of vertebral body units (VBU) below the carina during CVC placement. We investigated the relationship between the SVC-RA junction and the carina in children. MATERIALS AND METHODS: 584 CT scans of 0-18 years were analyzed. The carina was marked automatically by software while the SVC-RA junction and vertebrae were marked manually. The SVC-RA junction to carina (JC) distance was the primary study measurement reported in both VBU and mm. RESULTS: The data show an average JC distance of 1.25 VBU for 0-1 year, 1.27 VBU for 1-4 years, 1.34 VBU for 4-9 years, 1.53 VBU for 9-15 years, and 1.64 VBU for 15-18 years. A positive relationship between weight and JC distance was also demonstrated. CONCLUSION: JC distance is a useful predictor of SVC-RA junction location in children. Significant relationships were shown between JC distance and both age and weight. Due to small differences between age groups, however, average JC distance for all comers (1.48 VBU, 95% CI 0.7 - 2.3) can be used for SVC-RA junction identification in CVC placement.

Current Image-Guided Interventions in Pediatric Surgery.

Traditionally, surgical technique has not included imaging modalities. Image guidance had largely been left to radiology specialties. However, in recent years, tremendous advances in imaging have taken place with improvements in image quality, portability, and accessibility. With these advances, surgeons have begun to realize the benefits of fusing image guidance with traditional surgical approaches. Subsequently, many novel surgical approaches utilizing image guidance have been developed that allow for precise, safe, and minimally invasive management of conditions that previously required open surgical intervention.

Safety of delayed decannulation of venoarterial cannulas in patients with congenital diaphragmatic hernia.

BACKGROUND: The practice of "cutting-away" from venoarterial extracorporeal life support (ECLS) and leaving indwelling heparinized cannulas prior to decannulation is controversial. This study aims to determine the safety and efficacy of this strategy in patients with congenital diaphragmatic hernia (CDH) who require ECLS. METHODS: A single-center retrospective review of electronic health records was performed on all patients with CDH who underwent elective ECLS decannulation between January 2014 and September 2018. Descriptive statistics are presented as medians with interquartile range. RESULTS: Seventy-three percent (19/26) of patients who underwent venoarterial ECLS for CDH were electively decannulated. After a median ECLS run of 10.7 days [6.1-19.5], patients were "cut-away" for a median of 26 h [19.8-43] prior to decannulation. One patient required re-initiation at 36 h for a pulmonary hypertensive crisis (5%). There were no major bleeding or embolic events while "cut-away", and four (21%) patients had clots removed from the cannulas without clinical sequelae. One patient was recannulated 16 days following initial decannulation. CONCLUSIONS: Our data suggests that "cutting-away" from ECLS in patients with congenital diaphragmatic hernia is safe and allows a period of observation without significant complications. This strategy may be particularly helpful in patients at risk for recannulation, but better prognostic criteria are needed. LEVEL OF EVIDENCE: Level IV. TYPE OF STUDY: Treatment Study.

Salvage Laparoscopic-Assisted Anorectoplasty after Failed Vestibular Fistula Repair Using Magnetic Resonance Image Guidance.

Patients with vestibular fistula have a good functional outcome after posterior sagittal anorectoplasty (PSARP). While continence is often preserved, close follow-up and management of constipation are often required. Redo anorectal surgery has been associated with worse functional outcomes compared with primary procedures, possibly due to injury and scarring of the pelvic floor musculature and sphincter complex. Our group has a growing experience in the use of intraoperative real-time magnetic resonance imaging (MRI) for anorectal malformation repairs. We present a case of salvage operation of a failed PSARP for vestibular fistula.

Efficacy and risk profile of self-expandable stents in the management of pediatric esophageal pathology.

PURPOSE: The purpose of this study was to evaluate the efficacy and risk profile of esophageal stents in the management of complicated pediatric esophageal disease. METHODS: An IRB-approved, single-center, retrospective review was performed on all pediatric patients (n = 13) who underwent esophageal stent placement (2005-2017). Demographic, perioperative, and outcome data were analyzed (p < 0.05). RESULTS: Forty-one stents were placed due to recalcitrant strictures (n = 36), perforations (n = 2), and/or fistulae (n = 3). Median age at initial stent placement was 23.8 months (range, 50 days to 16 years), and median stent duration was 36 days (range, 3-335). The recurrence rate for strictures after initial stent removal was 100%. Four (31%) children subsequently underwent definitive operative repair. There were 5 deaths, including 2 related to stent placement. Seventy-one percent of stents were associated with an adverse event, most commonly intraluminal migration (56%). Younger children experienced an increased risk for airway compression and retching (p = 0.010). CONCLUSION: These data suggest that stents are associated with high complication rates and are not effective as definitive therapy for recalcitrant strictures in children. Although there may be a temporizing role for stents in selected patients, further refinements in stent technologies are needed to help manage this difficult patient population. LEVEL OF EVIDENCE: Level IV.

Evaluating a Solely Mechanical Articulating Laparoscopic Device: A Prospective Randomized Crossover Study.

BACKGROUND: The FlexDex® (FD) is a solely mechanical articulating device that combines the functionality of robotic surgery with the relative low cost and simplicity of laparoscopy. We sought to evaluate the performance of first-time FD users while performing a simple suture task at locations of varying degrees of difficulty. STUDY DESIGN: A prospective, randomized crossover study was performed comparing the FD to standard laparoscopy (SL). Two specific groups were evaluated; Group 1 consisted of complete novices, and Group 2 consisted of surgical trainees. Participants performed a simple suture with both FD and SL locations of varying degrees of difficulty (Easy, Moderate, and Hard). The following outcomes were evaluated: Instrument Function and Ergonomics (Comfort/Ergonomics survey), Task Difficulty (National Aeronautics and Space Administration Task Load Index [NASA-TLX]), Task Performance Quality (Objective Structured Assessment of Technical Skills [OSATS]), and Time (seconds). RESULTS: Twenty-two participants were enrolled with 12 participants in Group 1 and 10 participants in Group 2. Group 1-FD participants experienced overall less shoulder strain (1.2 ± 0.40 versus 1.9 ± 0.90, P = .01), and Group 2-FD participants experienced less shoulder (2.5 ± 0.66 versus 4.0 ± 0.50, P = .01), back (1.1 ± 0.32 versus 1.9 ± 0.74, P = .01), and forearm strain (1.9 ± 0.88 versus 2.5 ± 1.1, P = .04). Group 1 participants using the FD experienced higher mental demand (73 ± 17 versus 48 ± 27, P < .01) and perceived effort (70 ± 20 versus 54 ± 23, P < .001). Both Group 1 and Group 2 FD participants performed tasks at the Hard location more effectively. Both Group 1 (70 versus 87, P = .21) and Group 2 (53 versus 60, P = .55) performed tasks at the Hard location in similar times, while Group 1 (80 versus 177, P = .03) and Group 2 (33 versus 70, P = .001) performed tasks at the Easy location in shorter times using SL. CONCLUSIONS: This study demonstrates the first assessment of the FD, a mechanically articulating laparoscopic tool. First-time FD users demonstrated improved ergonomics and effectiveness suturing at difficult locations. Future studies will focus on comparison to robotic surgery and translation into clinical applications.

Ultrasound guidance improves safety and efficiency of central line placements.

BACKGROUND: Use of ultrasound-guidance for central venous access in adults is the standard of care. There is, however, less clarity in the role of routine ultrasound use in obtaining venous access in children. We sought to evaluate safety and efficiency of the placement of central lines utilizing an ultrasound-guided approach compared to the traditional, landmark approach in pediatric patients. STUDY DESIGN: A single-institution retrospective chart review, using CPT codes, was performed for all tunneled central venous catheters in children between 2005 and 2017 by the same pediatric surgery group. During the study period, a practice change occurred from exclusively landmark-based line placement to ultrasound-guided line placement. Groups were divided into three phases: a traditional/landmark era (Phase 1), transitional period (Phase 2), and the ultrasound era (Phase 3). The primary outcomes analyzed were postoperative chest tube insertions and operative time. RESULTS: A total of 2010 tunneled central lines were included for analysis: Phase 1 (N = 930), Phase 2 (N = 313) and Phase 3 (N = 767). Venous access for chemotherapy was the most common indication (29%). Phase 1 had a chest tube placement rate of 9.7/1000 procedures, while Phase 2 had a rate of 6.4/1000 procedures, and Phase 3 had no chest tube insertions (p = 0.009). Phase 1 had longer OR times compared to Phase 2 (57 vs. 49, p = 0.0026) and Phase 3 (57 vs. 46 min, p < 0.001). CONCLUSIONS: This study represents the largest analysis of ultrasound-guided access for children. A complete practice transition to the ultrasound-guided approach was feasible within a two-year period. The ultrasound-guided approach had a shorter operative time and less chest tube insertions than the traditional, landmark technique in children. Level III evidence.

An Early Investigation into Possible Alternatives to Stapled Hysterotomy in Open Fetal Surgery.

PURPOSE: We hypothesized that surgical energy could be used to create hysterotomies in open fetal surgery. STUDY DESIGN: Initial studies compared the LigaSure Impact and Harmonic ACE + 7 Shears in the efficiency of hysterotomy and thermal damage. Pregnant ewes at an estimated gestational age (EGA) of 116 to 120 days (term = 145; n = 7) underwent hysterotomy using either device. Hysterotomy edges were resected, and thermal injury extent was determined by histopathological assessment. Upon determining a superior device, subsequent studies compared this to the AutoSuture Premium Poly CS*-57 Stapler in uterine healing. Pregnant ewes (n = 6) at an EGA of 87 to 93 days underwent 6-cm hysterotomy in each gravid horn with either the stapler (n = 5) or Harmonic (n = 5) followed by closure and animal recovery. After 37 to 42 days, uterine healing was assessed by evaluating tensile strength and histopathology. RESULTS: Thermal damage was more extensive with the LigaSure (n = 11 hysterotomies) than with the Harmonic (n = 11; 5.6 ± 1 vs. 3.1 ± 0.6 mm; p < 0.0001);therefore, the Harmonic was selected for healing studies. Gross scar appearance and tensile strength were the same between the Harmonic and stapler. The stapler caused more fibrosis (4/7 samples with "moderate" fibrosis vs. 0/8 with the Harmonic; p = 0.02). CONCLUSION: The Harmonic ACE + 7 caused less thermal injury than the LigaSure Impact and performed similar to the CS*-57 Stapler in uterine healing with continued gestation.