A Comparative Study of Laparoscopic versus Open Management of Index Small Bowel Obstruction in Children.

INTRODUCTION: There is limited literature on the optimal approach to treat adhesive small bowel obstruction (ASBO) in children. We sought to compare rates and outcomes of laparoscopic (LAP) and open (OPEN) surgery for pediatric ASBO. METHODS: A California statewide database was used to identify children (<18 years old) with an index ASBO from 2007 to 2020. The primary outcome was the type of operative management: LAP or OPEN. Secondary outcomes were hospital characteristics, patient demographics, and postoperative complications. We excluded patients treated non-operatively. RESULTS: Our study group had 545 patients. 381 (70%) underwent OPEN and 164 (30%) LAP during the index admission. Over the study period, there was increasing use of laparoscopic surgery, with higher use in older children (p < 0.001). LAP was associated with fewer overall complications (65.2% vs. 81.6%, p < 0.001), with a decreasing trend in complications over time (p < 0.001). The LAP group had significantly lower rates of bowel resection (4.9% vs. 17.1%, p < 0.001), length of stay (LOS) (17 vs. 23 days, p < 0.001), and TPN use (12.2% vs. 29.1%, p < 0.001). Mortality rates were equivalent. Although the LAP group had lower readmission rates (22.6% vs. 37.3%, p < 0.001), the length of time between discharge and readmission was similar (171 vs. 165 days, p = 0.190). DISCUSSION: The use of laparoscopic surgery for index ASBO increased over the study period. However, it was less commonly utilized in younger children. LAP had fewer overall complications as well as shorter LOS, decreased TPN use, and fewer readmissions. The benefits and risks of each approach must be weighed. LEVEL OF EVIDENCE: III.

Trends in Management of Index Adhesive Small Bowel Obstruction in Children.

INTRODUCTION: To examine practice patterns and surgical outcomes of nonoperative versus operative management (OPM) of children presenting with an index adhesive small bowel obstruction (ASBO). METHODS: A California statewide health discharge database was used to identify children (<18 y old) with an index ASBO from 2007 to 2020. The primary study outcome was evaluating initial management patterns (nonoperative versus OPM and early [≤3 d] versus late surgery [>3 d]) of ASBO. Secondary outcomes were hospital characteristics, patient demographics, and postoperative complications. RESULTS: Of the 2297 patients identified, 1948 (85%) underwent OPM for ASBO during the index admission. Of these, 14.7% underwent early surgery within 3 d. Teaching hospitals had higher operative intervention than nonteaching centers (87.1% versus 83.7%, P = 0.034). OPM was the highest in 0-5-year-olds compared to other ages (89% versus 82%, P < 0.001). In comparison to early surgery, late surgery was associated with longer length of stay (early 7[interquartile range 5-10], late 9[interquartile range 6-17], P < 0.001), increased infectious complications (16.4% versus 9.8%, P = 0.004), and greater use of total parenteral nutrition (28.0% versus 14.3%, P = 0.001); there was no difference in bowel resection (21% versus 18%, P = 0.102) or mortality (P = 0.423). CONCLUSIONS: Our pediatric study demonstrated a high rate of OPM for index ASBO, especially in newborns and toddlers. Although operative intervention, especially late surgery, was associated with increased length of stay, increased infectious complications, and increased total parenteral nutrition use, the rates of bowel resection and mortality did not differ by management strategy. These trends need to be further evaluated to optimize outcomes.

The First Two Years of the Association of Pediatric Surgery Training Program Directors (APSTPD) Transition to Fellowship Course: Lessons Learned and Future Directions.

OBJECTIVE: The first transition to fellowship course for incoming pediatric surgery fellows was held in the US in 2018 and the second in 2019. The course aimed to facilitate a successful transition in to fellowship by introduction of the professional, patient care, and technical aspects unique to pediatric surgery training. The purpose of this study was to evaluate the feasibility and effectiveness of the first two years of this course in the US and discuss subsequent evolution of this endeavor. DESIGN: This is a descriptive and qualitative analysis of two years' experience with the Association of Pediatric Surgery Training Program Directors' (APSTPD) Transition to Fellowship course. Course development and curriculum, including clinical knowledge, soft skills, and hands-on skills labs, are presented. Participating incoming fellows completed multiple choice, boards-style pre- and post-tests. Scores were compared to determine if knowledge was effectively transferred. Participants also completed post-course evaluations and subsequent 3- or 12-month surveys inquiring on the lasting impact of the course on their transition into fellowship. Standard univariate statistics were used to present results. SETTING: The first APSTPD Transition to Fellowship course was held at the Johns Hopkins Hospital in Baltimore, Maryland in 2018, and the second course was held at the Oregon Health and Science University in Portland, Oregon in 2019. PARTICIPANTS: All fellows entering ACGME-certified Pediatric Surgery fellowships in the United States were invited to participate. Twenty fellows accepted and attended in 2018, and fourteen fellows participated in 2019. RESULTS: There were 34 incoming pediatric surgery fellow participants over 2 years. Faculty represented more than 10 institutions each year. Pre- and post-test scores were similar between years, with a significant improvement of scores after completion of the course (67±10% vs 79±8%, p < 0.001). Feedback from participants was overwhelmingly positive, with skills labs being attendees' favorite component. When asked about usefulness of individual course sessions, more attendees found clinical sessions more useful than soft skills (93% vs 73%, p = 0.011). Almost all (90%) of participants reported the course met its stated purpose and would recommend the course to future fellows. This was further reflected on 3 and 12 month follow up surveys wherein 85% stated they found the course helpful during the first few months of fellowship and 90% would still recommend it. CONCLUSIONS: A transition to fellowship course in the US for incoming pediatric surgery fellows is logistically feasible, effective in transfer of knowledge, and highly regarded among attendees. Feedback from each course has been used to improve the subsequent courses, ensuring that it remains a valuable addition to pediatric surgical training in the US.

Ovarian volume ratio is a reliable predictor of ovarian torsion in girls without an adnexal mass.

PURPOSE: The aims of this study were to identify ultrasound-based predictors of ovarian torsion in girls without an adnexal mass and establish a set of normal values for ovarian volume ratio (OVR). METHODS: A retrospective review was performed of all premenarchal patients ≥3 years of age with a normal pelvic ultrasound between January 2016 and January 2019. A comparison group of premenarchal girls presenting between 2011 and 2019 with torsion in the absence of an adnexal mass was utilized. RESULTS: Five-hundred and four premenarchal girls underwent pelvic ultrasound evaluation with a normal examination. The mean OVR was 1.6 ±â€¯0.7 (range 1.0-6.5). OVR did not vary with age (r = -0.06) as compared to ovarian width which increased steadily with age (r = 0.53, p < 0.001). OVR was increased in girls with torsion (7.6 vs 1.4, p < 0.0001), and by receiver operating characteristic (ROC) analysis a cutoff value of >2.5 demonstrated the best diagnostic accuracy of any predictive variable (sensitivity 100%, specificity 94%, AUC 0.991, p < 0.001). CONCLUSIONS: OVR is an excellent predictor of ovarian torsion in premenarchal girls without an adnexal mass. Unlike ovarian width, OVR does not increase with age, and a cutoff OVR > 2.5 demonstrates high sensitivity and specificity for identifying ovarian torsion in this population. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: Level III.

Patient-oriented online resources in pediatric surgery: Are we failing the readability test?

BACKGROUND: The study aim was to evaluate the readability of patient-oriented resources in pediatric surgery from children's hospitals in the US. METHODS: The websites of 30 children's hospitals were evaluated for information on 10 common pediatric surgical procedures. Hospitals of varying characteristics including bed number, geographic location and ACS Children's Surgery Verification (CSV) were selected for the study. Readability scores were calculated using validated algorithms, and text was assigned an overall grade level. RESULTS: Of 195 patient-oriented resources identified, only three (2%) were written at or below the recommended sixth grade level. Larger hospitals provided patient information at a higher grade level than medium and smaller sized centers (10.7 vs 9.3 vs 9.0 respectively, p < 0.001). Hospital size also correlated with availability of information, with large and medium sized hospitals having information more often. Hospitals with ACS CSV had information available more often, and written at a lower grade level, compared to nonverified centers (78% vs 62%, p = 0.023; 9.0 vs 10.0, p = 0.013). CONCLUSION: Most hospital provided patient-oriented resources in pediatric surgery are written at a grade level well above the national guidelines. Centers with ACS CSV status have improved availability and readability of this material, while larger hospitals have improved availability, but decreased readability. TYPE OF STUDY: Modeling study. LEVEL OF EVIDENCE: III.

Trephination versus wide excision for the treatment of pediatric pilonidal disease.

BACKGROUND/PURPOSE: To evaluate outcomes of trephination compared to wide excision in children undergoing initial surgical treatment of pilonidal disease. METHODS: A retrospective review was conducted of patients undergoing initial pilonidal excision between September 2017 and September 2018. Operations were categorized as either trephination or wide excision via an open or closed-wound technique. Outcomes were evaluated and data analyzed by chi-squared and one-way ANOVA tests. RESULTS: One-hundred and five patients were identified, with a mean follow-up of 4.6 months. Trephination was performed in 57% of patients, and of the remaining patients undergoing wide excision, 62% of wounds were left open. There were no demographic differences among the three groups. Compared to both the open and closed techniques, trephination was associated with fewer wound complications (17% vs 29% vs 3%, respectively, p = 0.006), and postoperative visits (4.4 vs 2.4 vs 1.4, respectively, p < 0.001). There was no difference in recurrence rates among groups. CONCLUSION: Trephination for pilonidal disease in pediatric patients is associated with a lower wound complication rate and fewer postoperative visits compared to wide excision. Recurrence rates are similar among the various surgical methods. Further prospective study would be useful to describe long-term outcomes of patients undergoing trephination for pilonidal disease. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: III (retrospective comparative).

Premenarchal patients present differently: A twist on the typical patient presenting with ovarian torsion.

BACKGROUND/PURPOSE: The aim of this study was to characterize differences between pre- and postmenarchal females with ovarian torsion. METHODS: A single-center review was conducted of all nonneonatal pediatric patients with ovarian torsion from 2011 to 2018. Clinical data were compared between pre- and postmenarchal patients. RESULTS: One hundred and twenty-seven patients were identified, and 25% were premenarchal. Premenarchal patients were more likely to have a delay in diagnosis (38% vs 20%, p = 0.042), develop ovarian necrosis (34% vs 17%, p = 0.036), and present without an associated adnexal mass (44% vs 0%, p < 0.001). All patients without a mass (n = 14) were premenarchal and presented with ovarian asymmetry. Patients without an associated mass underwent oophoropexy in the majority of cases. There were no differences in postoperative complication or recurrence rates between groups. CONCLUSION: Premenarchal females with ovarian torsion can present differently than females postmenarche and often have a delay in diagnosis. Premenarchal females are more likely to undergo torsion without an associated adnexal mass and are at higher risk for ovarian necrosis. Oophoropexy is an appropriate treatment in the absence of an adnexal mass. A high-index of suspicion for ovarian torsion should be maintained for premenarchal females presenting with abdominal pain and an otherwise negative workup. TYPE OF STUDY: Retrospective comparative study. LEVEL OF EVIDENCE: Level III.

Plasma D-dimer safely reduces unnecessary CT scans obtained in the evaluation of pediatric head trauma.

PURPOSE: Serum D-dimer has been proposed as a biomarker to aid in the diagnosis of pediatric traumatic brain injury (TBI). We investigated the accuracy of D-dimer in predicting the absence of TBI and evaluated the degree by which D-dimer could limit unnecessary computed tomography scans of the head (CTH). METHODS: Retrospective review of patients with suspected TBI from 2011 to 2013 who underwent evaluation with CTH and quantitative D-dimer. D-dimer levels were compared among patients with clinically-important TBI (ciTBI), TBI, isolated skull fracture and no injury. RESULTS: Of the 663 patients evaluated for suspected TBI, ciTBI was identified in 116 (17.5%), TBI in 77 (11.6%), skull fracture in 61 (9.2%) and no head injury in 409 (61.7%). Patients with no head injury had significantly lower D-dimer values (1531±1791pg/µL) compared to those with skull fracture, TBI and ciTBI (2504±1769, 2870±1633 and 4059±1287pg/µL, respectively, p<0.005). Using a D-dimer value <750pg/µL as a negative screen, no ciTBIs would be missed and 209 CTHs avoided (39.7% of total). CONCLUSION: Low plasma D-dimer predicts the absence of ciTBI for pediatric patient with suspected TBI. Incorporating D-dimer into current diagnostic algorithms may significantly limit the number of unnecessary CTHs performed in this population. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: I.

Preemptive Ladd Procedure in congenital diaphragmatic hernia and Abdominal Wall defects does not reduce the risk of future volvulus.

INTRODUCTION: Patients with congenital diaphragmatic hernia (CDH), gastroschisis, and omphalocele are prone to abnormalities of intestinal rotation and thus future midgut volvulus. Controversy exists whether routine preemptive Ladd procedure in this subgroup of asymptomatic patients is of value to reduce future volvulus. METHODS: The Pediatric Health Information System (PHIS) database was queried for neonates with CDH, gastroschisis, and omphalocele between January 2009 and September 2015. Standard univariate and multivariate analysis was completed to compare risk of volvulus between patients treated with a preemptive Ladd versus no Ladd (p<0.05). RESULTS: Seven thousand seven hundred forty-nine neonates with CDH (40%), gastroschisis (38%), omphalocele (14%), and abdominal wall malformation (NOS) (9%) were identified. Overall, 3.6% (n=279) had a preemptive. Thirty-two patients had subsequent volvulus. Ladd procedure did not reduce volvulus readmissions for any diagnosis and was associated with a significantly increased risk of volvulus for omphalocele patients (9.1% vs 0.1%, p<0.001). Overall, a Ladd procedure during the index admission was associated with a higher risk for volvulus (1.4% vs. 0.4%, p=0.021) and was associated with a 3.28 increased odds ratio of future volvulus (95% CI 1.12-9.68). CONCLUSION: Ladd procedure performed prophylactically in patients with CDH, gastroschisis, and omphalocele did not reduce subsequent volvulus. LEVEL OF EVIDENCE: III, Retrospective Comparative Study.

Low Infection-Related Re-Admission Rates in a Retrospective of 4725 Children with Appendicitis Using a Clinical Pathway in a Tertiary Care Pediatric Center.

BACKGROUND: Standardization of antibiotic management of appendicitis in tertiary care pediatric centers has been associated with improved outcomes. Rady Children's Hospital-San Diego implemented an appendicitis clinical pathway in 2005. We evaluated infection-related re-admission risk factors since 2010, when an electronic medical record was established, with the aim to optimize the clinical pathway. METHODS: Between January 2010 and August 2015, 4725 children with a diagnosis of appendicitis were evaluated for demographic data, pathology diagnoses, culture results, and inpatient and oral step-down antibiotic therapy regimens. From children originally admitted for appendicitis, those who were re-admitted with infection were compared with those who were not re-admitted for infection. The populations were controlled by severity of infection using a pathology-defined appendicitis severity scale: Grade 0, no appendicitis; grade 1, simple acute appendicitis with gross and microscopic evidence of inflammation, but no perforation; grade 2, gangrenous/necrotizing/micro-perforated appendicitis with subserosal or serosal exudate, but no frank or visually appreciated perforation; and grade 3, frank perforation. RESULTS: Of 4725 children (total population, TP) admitted with a diagnosis of appendicitis, only 199 (4.2%) were re-admitted, with 125 of these admissions for infection (2.65% of the TP). Age, race/ethnicity, language preference, and body mass index were not found to correlate with re-admission for infection. Length of stay significantly differed between the no infection-related re-admission population and infection-related re-admission population (3.02 vs. 4.03 d, p < 0.001). There was a trend toward higher infection-re-admission rates as the pathology grade increased (odds ratio grade 1 vs. grade 3 = 2.28, 95% confidence interval 1.03, 5.03). CONCLUSIONS: Infection-related re-admission rates for children on the clinical pathway in our institution were infrequent. The greater association of all-cause and infection-related re-admission rates with pathology grade suggest that defining appendicitis by pathology and clinical severity may provide an evidence-based scoring system to support clinical observation in the use and duration of antibiotic therapy.

Combined liver transplant and pancreaticoduodenectomy for inflammatory hilar myofibroblastic tumor: Case report and review of the literature.

IMT, previously known as IPT, is a relatively rare tumor that was originally described in the lungs, but case reports have reported the tumor in almost every organ system. Surgical resection is typically the mainstay of therapy; however, tumors have also been shown to respond to chemotherapy or anti-inflammatory therapy and some have spontaneously regressed. We present a literature review and case report representing the first documentation to date of liver transplant combined with PD for surgical resection of a myofibroblastic tumor non-responsive to medical therapy.

Ketorolac does not reduce effectiveness of pleurodesis in pediatric patients with spontaneous pneumothorax.

PURPOSE: Antiinflammatory medications are thought to reduce the effectiveness of pleurodesis performed for the treatment of spontaneous pneumothorax. We reviewed our experience with children undergoing video-assisted thorascopic surgery (VATS) with pleurodesis for pneumothorax to determine if ketorolac administration influences patient outcomes. METHODS: A retrospective review of patients who underwent VATS pleurodesis for spontaneous pneumothorax from 2009 to 2013 at a pediatric hospital was performed. Length of stay, radiographic pneumothorax resolution prior to discharge, and ipsilateral recurrence rates were compared in patients who did and did not receive perioperative ketorolac. RESULTS: Over a 50-month period, 51 patients underwent VATS with mechanical pleurodesis for spontaneous pneumothorax. The average age was 15.5years, and 76% were male. Ketorolac was administered to 26/51 patients. There were no differences in average length of stay (11.3 vs 10.9days, p=0.36), incidence of residual pneumothorax at discharge (22/41 vs 19/41, p=0.48), or ipsilateral recurrence (5/10 vs 5/10, p=1). CONCLUSIONS: Despite the intrinsic antiinflammatory properties of ketorolac, our data suggests that its use for patients undergoing pleurodesis for spontaneous pneumothorax does not detrimentally influence the outcomes of surgery. Therefore, we conclude that ketorolac can be used for pain control in this population. Large-scale studies are warranted to validate these findings.

Congenital-infantile fibrosarcoma presenting as a hemangioma: a case report.

INTRODUCTION: Congenital-infantile fibrosarcoma (CIFS) is a rare pediatric soft tissue tumor that on initial clinical presentation can mimic an infantile hemangioma. Management of these conditions differs drastically, and delay in diagnosis and treatment may affect prognosis. METHODS: A 6-month-old male infant originally presented to dermatology for evaluation of a suspected right buttock hemangioma, present at birth as a small red mass just lateral to the gluteal cleft. The lesion rapidly grew over 2 weeks to a size of 4×4.5×4.5 cm, with violaceous color change and central ulceration. The patient received a course of cephalexin; however, the mass became painful with several episodes of bleeding and did not show clinical improvement. Magnetic resonance imaging and ultrasound supported the diagnosis of atypical hemangioma. RESULTS: Plastic Surgery was consulted for excision due to recurrent bleeding. An excisional biopsy was undertaken and pathologic finding showed CIFS. The case was discussed with Hematology/Oncology and Pediatric Surgery, and the patient underwent re-excision and obtained clear margins. Initial metastatic workup was negative; however, patient had evidence of recurrent disease as well as pulmonary metastasis on his most recent surveillance imaging. He underwent re-excision of the gluteal mass and is currently receiving adjuvant chemotherapy. CONCLUSIONS: This case demonstrates a rare malignancy initially misdiagnosed as a hemangioma. Consideration of CIFS is crucial in cases of complicated hemangiomas not responding to standard treatment. Treatment calls for excision with wide margins and/or adjuvant chemotherapy if excision is not possible. Classic pathologic findings, immunohistochemistry, and/or reverse transcription-polymerase chain reaction can confirm the diagnosis. A correct and rapid diagnosis of CIFS is critical to optimizing treatment and subsequent prognosis.

Initial use of LESS for the ACE Malone procedure in children.

The Malone antegrade continence enema is effective in improving fecal continence in neurogenic bowel. We were able to perform this procedure laparoscopically via a single incision in a patient with spina bifida and fecal incontinence who did not require bladder reconstruction.

Early experience with single-incision thoracoscopic surgery in the pediatric population.

INTRODUCTION: Single-incision pediatric endosurgery is gaining popularity in children. We have recently applied the single-incision approach for thoracoscopic procedures. We report our initial experience with single-incision thoracoscopic surgery in the pediatric population. METHODS: A retrospective chart review of the first 10 single-incision thoracoscopic operations done at our institution was conducted. The patients' mean age and weight and the median operative time, postoperative length of stay, and time until discontinuation of chest tubes were determined. RESULTS: The 10 procedures were performed in eight patients (two patients each had bilateral procedures). The procedures performed included wedge resection and mechanical pleurodesis for spontaneous pneumothorax (n = 7), wedge biopsies for lymphoma (n = 1) and chronic granulomatous disease (n = 1), and resection of an apical extrapulmonary neuroblastoma (n = 1). All of the procedures were completed without intraoperative complication or significant blood loss. In each case, multiple trocars and/or unsheathed instruments were passed through a single small incision, which was subsequently used for the chest tube(s). The mean patient age was 13.5 years (range 3-18 years). The mean weight was 47 kilograms (range 16-63 kg). The median operative time was 64 minutes (range 50-201 minutes). The median postoperative length of stay was 7 days (range 3-19 days). The median time until chest tube removal was 3 days (range 2-15 days). The mean follow-up was 7 months (range 3-12 months). One patient developed a recurrent pneumothorax and persistent air leak after having undergone a wedge resection and pleurodesis for a spontaneous pneumothorax and required a reoperation. CONCLUSION: Single-incision thoracoscopic surgery is a feasible alternative to the traditional multiple-incision approach in the pediatric population. The in-line positioning of the camera and instruments often proves to be an advantage rather than a hindrance.

Early experience with laparoendoscopic single-site surgery in the pediatric urology patient population.

BACKGROUND AND PURPOSE: To present our initial experience with single-incision laparoscopy in the pediatric urologic population. PATIENTS AND METHODS: Four patients underwent eight single-incision laparoscopic procedures. One patient underwent bilateral nephrectomies, a hernia repair, and an orchiectomy. The remaining three patients underwent unilateral nephrectomies. All procedures were performed in children using the Covidien SILS port system via an umbilical incision. RESULTS: Procedures in three of four patients, representing seven of eight cases, were successfully performed via a single incision. Operative times ranged from 114 minutes to 360 minutes. There were no conversions to traditional laparoscopy. One nephrectomy was converted to an open procedure secondary to bleeding. There were no complications postoperatively, and at follow-up, all wounds were well healed. CONCLUSIONS: Single-incision laparoscopic urologic surgery is safe and feasible in the pediatric population. The umbilical (trans or peri) approach confers improved cosmesis over traditional laparoscopy, and the scar is essentially concealed.

Sternal cleft, Morgagni hernia, and ectopic liver: a unique chest wall anomaly.

Sternal clefts are rare chest wall anomalies which may occur in isolation or in association with cardiac, pericardial, and diaphragmatic defects. We present the first report of a unique chest wall anomaly consisting of a triad of sternal cleft, anterior diaphragmatic hernia, and ectopic, exophytic liver tissue. A partial diagnosis was made on prenatal ultrasound imaging and computed tomography imaging of the baby's chest, but the full extent of the anomaly was only identified at operation. Full repair was completed on the third day of life with excellent outcome.

Fibroblast growth factor 10 is required for survival and proliferation but not differentiation of intestinal epithelial progenitor cells during murine colon development.

Epithelial-mesenchymal interactions that govern the development of the colon from the primitive gastrointestinal tract are still unclear. In this study, we determine the temporal-spatial expression pattern of Fibroblast growth factor 10 (Fgf10), a key developmental gene, in the colon at different developmental stages. We found that Fgf10 is expressed in the mesenchyme of the distal colon, while its main receptor Fgfr2-IIIb is expressed throughout the entire intestinal epithelium. We demonstrate that Fgf10 inactivation leads to decreased proliferation and increased cell apoptosis in the colonic epithelium at E10.5, therefore resulting in distal colonic atresia. Using newly described Fgf10 hypomorphic mice, we show that high levels of FGF10 are dispensable for the differentiation of the colonic epithelium. Our work unravels for the first time the pivotal role of FGF10 in the survival and proliferation of the colonic epithelium, biological activities which are essential for colonic crypt formation.

The fibroblast growth factor pathway serves a regulatory role in proliferation and apoptosis in the pathogenesis of intestinal atresia.

BACKGROUND/PURPOSE: Intestinal atresia occurs in 1:5000 live births and is a neonatal challenge. Fibroblast growth factor receptor 2b (Fgfr2b) is a critical developmental regulator of proliferation and apoptosis in multiple organ systems including the gastrointestinal tract (GIT). Fgfr2b invalidation results in an autosomal recessive intestinal atresia phenotype. This study evaluates the role of Fgfr2b signaling in regulating proliferation and apoptosis in the pathogenesis of intestinal atresia. METHODS: Wild-type and Fgfr2b-/- embryos were harvested from timed pregnant mice. The GIT was harvested using standard techniques. Terminal deoxynucleotidyl transferase biotin-dUTP nick end labeling) was used to evaluate apoptosis and bromodeoxyuridine to assess proliferation by standard protocols. Photomicrographs were compared (Institutional Animal Care and Use Committee-approved protocol 32-02). RESULTS: Wild-type and mutant GIT demonstrate that deletion of the Fgfr2b gene results in inhibition of epithelial proliferation and increased apoptosis. Inhibited proliferation and increased apoptosis are specific to those tissues of normal Fgfr2b expression, corresponding to the site of intestinal atresia. CONCLUSIONS: The absence of embryonic GIT Fgfr2b expression results in decreased proliferation and increased apoptosis resulting in GIT atresia. The regulation of proliferation and apoptosis in intestinal cells as a genetically based cause of intestinal atresia represents a novel consideration in the pathogenesis of intestinal atresia.