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.

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.

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.

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.

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).

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.

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.

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.

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.

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.

Dickkopf-1 (DKK1) reveals that fibronectin is a major target of Wnt signaling in branching morphogenesis of the mouse embryonic lung.

Members of the Dickkopf (Dkk) family of secreted proteins are potent inhibitors of Wnt/beta-catenin signaling. In this study we show that Dkk1, -2, and -3 are expressed distally in the epithelium, while Kremen1, the needed co-receptor, is expressed throughout the epithelium of the developing lung. Using TOPGAL mice [DasGupta, R., Fuchs, E., 1999. Multiple roles for activated LEF/TCF transcription complexes during hair follicle development and differentiation. Development 126, 4557-4568] to monitor the Wnt pathway, we show that canonical Wnt signaling is dynamic in the developing lung and is active throughout the epithelium and in the proximal smooth muscle cells (SMC) until E12.5. However, from E13.5 onwards, TOPGAL activity is absent in the SMC and is markedly reduced in the distal epithelium coinciding with the onset of Dkk-1 expression in the distal epithelium. To determine the role of Wnt signaling in early lung development, E11.5 organ cultures were treated with recombinant DKK1. Treated lungs display impaired branching, characterized by failed cleft formation and enlarged terminal buds, and show decreased alpha-smooth muscle actin (alpha-SMA) expression as well as defects in the formation of the pulmonary vasculature. These defects coincide with a pattern of decreased fibronectin (FN) deposition. DKK1-induced morphogenetic defects can be mimicked by inhibition of FN and overcome by addition of exogenous FN, suggesting an involvement of FN in Wnt-regulated morphogenetic processes.

Fibroblast growth factor-10 serves a regulatory role in duodenal development.

PURPOSE: Duodenal obstruction occurs in 1 of 6000 live births and requires urgent surgical intervention. Duodenal atresia previously has been ascribed to a developmental failure of luminal recanalization; however, the cause of duodenal atresia remains incompletely understood. Although familial intestinal atresias have been described and syndromic associations are known, no specific genetic link has been established. Fibroblast growth factor-10 (Fgf10) is a known regulatory molecule relevant to mesenchymal-epithelial interactions, and mice deficient in Fgf10 demonstrate congenital anomalies in several organ systems including the gastrointestinal tract. The authors hypothesized that Fgf10 could serve a regulatory role in establishing normal duodenal development. METHODS: Wild-type mice with beta-galactosidase under the control of the Fgf10 promoter were harvested from timed-pregnancy mothers. The expression of Fgf10 in the duodenum during development was evaluated by developing the embryos in X-Gal solution. Wild-type and mutant Fgf10(-/-) embryos were harvested from timed-pregnancy mothers at 18.5 days postconception (near term) and were analyzed for duodenal morphology (Institutional Animal Care and Use Committee-approved protocol 32-02). Photomicrographs were reviewed. RESULTS: Fibroblast growth factor-10 is active in the duodenum at a late stage of development. The Fgf10(-/-) mutants demonstrate duodenal atresia with a variable phenotype similar to clinical findings. The duodenum fails to develop luminal continuity and has proximal dilation. The phenotype occurs in an autosomal recessive pattern with incomplete penetrance (38%). CONCLUSIONS: Fibroblast growth factor-10 serves as a regulator in normal duodenal growth and development. Its deletion leads to duodenal atresia and challenges traditionally accepted theories of pathogenesis. This novel, genetically mediated duodenal malformation reflects an animal model that will allow further evaluation of the pathogenesis of this surgically correctable disease. By studying the mechanism of Fgf10 function in foregut development, the authors hope to better understand these anomalies and to explore possible therapeutic alternatives.

Colonic atresia without mesenteric vascular occlusion. The role of the fibroblast growth factor 10 signaling pathway.

BACKGROUND/PURPOSE: Colonic atresia occurs in 1:20,000 live births, offering a neonatal surgical challenge. Prenatal expression of fibroblast growth factor 10 (Fgf10), acting through fibroblast growth factor receptor 2b (Fgfr2b), is critical to the normal development of the colon. Invalidation of the Fgf10 pathway results in colonic atresia, inherited in an autosomal recessive pattern. Classically, disturbance of the mesenteric vasculature has been thought to cause many forms of intestinal atresia. The purpose of this study was to evaluate the role of vascular occlusion in the pathogenesis of colonic atresia. METHODS: Wild type (Wt), Fgf10(-/-), and Fgfr2b(-/-) mutant mouse embryos were harvested from timed pregnant mothers. Immediately following harvest, filtered India ink was infused via intracardiac microinjection. The gastrointestinal tract was dissected, and photomicrographs of the mesenteric arterial anatomy were taken at key developmental time points. RESULTS: Photomicrographs after India ink microinjections demonstrate normal, patent mesenteric cascades to the atretic colon at the time points corresponding to the failure of colonic development in the Fgf10(-/-) and Fgfr2b(-/-) mutants. The mesenteric arterial anatomy of the colon demonstrates no difference between the Wt and mutant colonic atresia. CONCLUSIONS: The absence of embryonic expression of Fgf10 or its receptor Fgfr2b results in colonic atresia in mice. India ink microinjection is a direct measure of mesenteric arterial patency. Colonic atresia in the Fgf10(-/-) and Fgfr2b(-/-) mutants occurs despite normal mesenteric vascular development. Thus the atresia is not the result of a mesenteric vascular occlusion. The patent colonic mesentery of the Fgf10(-/-) and Fgfr2b(-/-) mutants challenges an accepted pathogenesis of intestinal atresia. Although colonic atresia can occur as a result of vascular occlusion, new evidence exists to suggest that a genetic mechanism may play a role in the pathogenesis of this disease.

Fibroblast growth factor 10 (Fgf10) invalidation results in anorectal malformation in mice.

BACKGROUND/PURPOSE: Anorectal malformations occur in 1 per 4,000 live births and represent a surgical challenge. Although critically important, the basic mechanisms of normal anorectal union are incompletely understood. Fgf10 signaling is known to serve a key role in mesenchymal/epithelial interactions in many organ systems including the gastrointestinal tract (GIT). The authors therefore hypothesized that Fgf10 signaling has a central role in normal anorectal development. METHODS: Fgf10 expression in wild-type (Wt) embryos was evaluated using whole-mount in situ hybridization. Wt and Fgf10-/- embryos were harvested from timed pregnant mothers at E12.5 through E17.5 and were analyzed for anorectal phenotype. RESULTS: Wt development of union between anorectal structures is completed between E12.5 and E13.5 with luminal communication between distal rectal epithelium and anus. Fgf10 is discretely expressed at E12.5 in the distal rectum. Fgf10-/- mutants show failure of union of the rectum and anus at an early stage (E13.5) and near term (E17.5). CONCLUSIONS: Fgf10 is expressed in the rectum at the time when anorectal continuity is established, indicating a role in normal anorectal development. Fgf10 invalidation (Fgf10-/- mutant) results in a genetically reproducible anorectal malformation phenotype. Fgf10 function is critical for normal anorectal development.

Fibroblast growth factor receptor 2 IIIb invalidation--a potential cause of familial duodenal atresia.

BACKGROUND/PURPOSE: Duodenal atresia (DA) occurs in 1 in every 6,000 live births and represents a significant surgically correctable cause of intestinal obstruction in the neonate. Familial or congenital DA has been reported, implying that at least some cases of DA are the result of genetic, heritable abnormalities. The genes controlling duodenal development are incompletely understood. Fibroblast growth factor receptor 2IIIb (Fgfr2b) is known to play a critical role in the development of multiple organ systems including other gastrointestinal tract (GIT) structures. This study shows the key role of Fgfr2b in normal duodenal development and the pathogenesis of DA. METHODS: Wild type (Wt) and Fgfr2b-/- embryos were harvested from timed pregnant mothers at stage E18.5 and were analyzed for duodenal phenotype. RESULTS: Inactivation of Fgfr2b results in DA. DA is present in the Fgf2b-/- mutants with a 35% penetrance. The duodenal phenotype of the Fgf2b-/- mutants ranges from normal to a mucosal web, type I, and type III atresia. CONCLUSIONS: Fgfr2b is a critical regulatory gene in the development of the duodenum. Fgfr2b invalidation (Fgfr2b-/- mutant) results in a reproducible, autosomal recessive duodenal atresia phenotype with incomplete penetrance and a variable phenotype.