Reduced Long-Term Bowel Obstruction Risk With Minimally Invasive Diaphragmatic Hernia Repair.

INTRODUCTION: The introduction of minimally invasive surgery (MIS) for repair of congenital diaphragmatic hernias (CDH) has reduced postoperative length of stay, postoperative opioid consumption, and provided a more esthetic repair. In adult abdominal surgery, minimally invasive techniques have been associated with decreased long-term rates of small bowel obstruction (SBO), although it is unclear if this benefit carries over into the pediatric population. Our objective was to evaluate the rates of SBO following open versus MIS CDH repair. MATERIAL AND METHODS: Infants who underwent CDH repair between 2010 and 2021 were identified using the PearlDiver Mariner database. Kaplan-Meier curves and Cox proportional hazards models were used to evaluate time to SBO by surgical approach (MIS versus open) while adjusting for mesh use, patient sex, and length of stay. RESULTS: Of 1033 patients that underwent CDH repair, 258 (25.0%) underwent a minimally invasive approach. The overall rate of SBO was 7.5% (n = 77). Rate of SBO following MIS repair was lower than open repair at 1 y (0.8% versus 5.1%), 3 y, (2.3% versus 9.0%), and 5 y (4.4% versus 10.1%, P = 0.004). Following adjustment, the rate of SBO following MIS repair remained significantly lower than open repair (adjusted hazard ratio: 0.37, 95% confidence interval: 0.18, 0.79). CONCLUSIONS: Following CDH repair, long-term rates of SBO are lower among patients treated with MIS approaches. Long-term risk of SBO should be considered when selecting surgical approach for CDH patients.

Prophylactic antireflux procedures are not necessary in neurologically impaired children undergoing gastrostomy placement.

PURPOSE: Fundoplication is frequently used in children with neurologic impairment even in the absence of reflux due to concerns for future gastric feeding intolerance, but supporting data are lacking. We aimed to determine the incidence of secondary antireflux procedures (fundoplication or gastrojejunostomy (GJ)) post gastrostomy tube (GT) placement in children with and without neurologic impairment. METHODS: Children under 18 undergoing a GT placement without fundoplication between 2010 and 2020 were identified utilizing the PearlDiver Mariner national patient claims database. Children with a diagnosis of cerebral palsy or a degenerative neurologic disease were identified and compared to children without these diagnoses. The incidence of delayed fundoplication or conversion to GJ were compared utilizing Kaplan-Meier and Cox proportional hazards regression analyses. RESULTS: A total of 14,965 children underwent GT placement, of which 3712 (24.8%) had a diagnosis of neurologic impairment. The rate of concomitant fundoplication was significantly higher among children with a diagnosis of neurologic impairment as compared to those without (9.3% vs 6.4%, p < 0.001). While children with neurologic impairment had a significantly higher rate of fundoplication or GJ conversion at 5 years compared to children without (12.6% [95% confidence interval (CI): 11.4%-13.8%] vs 8.6% [95% CI 8.0%-9.2%], p < 0.001), the overall incidence remained low. CONCLUSION: Although children with neurologic impairment have a higher rate of requiring an antireflux procedure or GJ conversion than other children, the overall rate remains less than 15%. Fundoplication should not be utilized in children without clinical reflux on the basis of neurologic impairment alone.

A shortened course of Amoxicillin/Clavulanate is the preferred antibiotic treatment after surgery for perforated appendicitis in children.

BACKGROUND/PURPOSE: Despite evidence supporting short course outpatient antibiotic treatment following appendectomy for perforated appendicitis, evidence of real-world implementation and consensus for antibiotic choice is lacking. We therefore aimed to compare outpatient antibiotic treatment regimens in a national cohort. METHODS: We identified children who underwent surgery for perforated appendicitis between 2010 and 2018 using the PearlDiver database and compared 45-day disease-specific readmission between children who received shortened (5-8 days) versus prolonged (10-14 day) total antibiotic courses (inpatient intravenous and/or oral) completed with outpatient Amoxicillin/Clavulanate versus Ciprofloxacin/Metronidazole, and compared antibiotic type (5-14 days) to each other. RESULTS: 4916 children were identified, 2001 (90.0%) treated with Amoxicillin/Clavulanate (5-14 days), 381 (19.0%) with shortened (5-8 days), 1464 (73.2%) with prolonged (10-14 days) courses. 222 (10.0%) were treated with Ciprofloxacin/Metronidazole, 44 (19.8%) with shortened, 174 (78.4%) with prolonged courses. Freedom from readmission was not different between prolonged and shortened course whether they received Amoxicillin/Clavulanate (adjusted hazard ratio [AHR] 1.54, 95%CI 0.95-2.5) or Ciprofloxacin/Metronidazole (AHR 3.49, 95%CI 0.45-27.3). Antibiotic type did not affect readmission rate (Amoxicillin/Clavulanate versus Ciprofloxacin/Metronidazole, AHR 1.21, 95%CI 0.71-2.05). CONCLUSION: Prolonged antibiotic regimens are routinely prescribed despite evidence suggesting shorter courses and antibiotic choice are not associated with greater treatment failure. As it is better tolerated, we recommend a shortened course of Amoxicillin/Clavulanate for oral management of perforated appendicitis. STUDY DESIGN: Retrospective. LEVEL OF EVIDENCE: Level III.

Minimally Invasive Approaches and Use of a Patch Are Not Associated with Increased Recurrence Rates After Congenital Diaphragmatic Hernia Repair.

Background: Minimally invasive surgery (MIS) is increasingly used for repair of congenital diaphragmatic hernia (CDH). Reported recurrence after MIS repair varies and is limited by short follow-up and low volume. Our objective was to compare recurrence after MIS versus open repair of CDH. Materials and Methods: Infants who underwent CDH repair between 2010 and 2020 were identified using the PearlDiver Mariner database, a national patient claims data set allowing longitudinal follow-up of patients across systems. Kaplan-Meier analysis and Cox proportional hazards regression models were used to evaluate the association of surgical approach (MIS versus open) and use of a patch with time to recurrence while adjusting for comorbidities (congenital heart disease and pulmonary hypertension) and length of stay (LOS). Results: In a cohort of 629 infants, 25.6% (n = 161) underwent MIS repair with a median follow-up of 4.8 years and recurrence rate of 38.6% (n = 243). Rates of recurrence after MIS repair were lower than open (5 years: 38.6% versus 44.3%; P = .03) and higher with use of patch (5 years: 60.1% versus 40.1%; P = .02). After adjustment for comorbidities and LOS as a proxy for patient complexity, there was no significant difference in recurrence based on approach (adjusted hazard ratio [aHR]: 0.79; confidence interval [95% CI]: 0.57-1.10; P = .16) or use of patch (aHR: 1.22; 95% CI: 0.83-1.79; P = .32). Conclusions: Recurrence rates after repair of CDH were not different based on surgical approach or use of patch after adjustment. Previous data were likely biased by patient complexity, and surgeons should consider these factors in determining approach.

Long-Term Recurrence Risk Following Pleurectomy or Pleurodesis for Primary Spontaneous Pneumothorax.

INTRODUCTION: Recurrent primary spontaneous pneumothorax (PSP) is often managed with a wedge resection (or blebectomy) and either pleurectomy or pleurodesis. There is a conflicting data regarding which approach is superior to reduce recurrence. Our objective is to evaluate the long-term recurrence rates following pleurectomy versus mechanical pleurodesis for recurrent PSP. METHODS: The PearlDiver Mariner Patient Claims Database was queried for patients aged 10-25 who were presented with PSP and underwent either pleurectomy or mechanical pleurodesis between 2010 and 2020. The primary outcome was recurrence and secondary outcomes included 30-day opioid prescriptions, pain diagnoses, and reimbursement. Kaplan-Meier analysis and Cox proportional hazards regression models were used with adjustment for age and sex. RESULTS: Of 18,955 patients presenting with PSP, 5.1% (n = 968) were managed operatively with either pleurectomy (18.3%, n = 177) or mechanical pleurodesis (81.7%, n = 791). There was no difference in the rate of recurrence between pleurectomy and mechanical pleurodesis (5-year risk of recurrence: 25.8% versus 26.5%, adjusted hazard ratio (HR) = 1.12 [95% confidence interval (CI): 0.79, 1.58]). Furthermore, there was no difference in rate of outpatient opioid prescription (49.2% versus 52.8%, P = 0.58) or pain diagnoses (22.0% versus 22.8%, P = 0.46) between pleurectomy and mechanical pleurodesis, respectively. The median reimbursement was higher following pleurectomy as compared to mechanical pleurodesis ($14,040 versus $5,811, P = 0.02). CONCLUSIONS: There is no significant difference in recurrence based on type of procedure performed for recurrent primary spontaneous pneumothorax. However, reimbursement is higher following pleurectomy. Given the similar outcomes but higher cost, we recommend mechanical pleurodesis over pleurectomy for recurrent PSP.

Recurrence following laparoscopic repair of bilateral inguinal hernia in children under five.

BACKGROUND: Reported recurrence rates after laparoscopic versus open inguinal hernia repair have been limited to high volume centers with short follow-up. We sought to compare national rates of recurrence after laparoscopic versus open bilateral inguinal hernia repair. METHODS: Children under five who underwent bilateral inguinal hernia repair between 2010 and 2020 were identified using the PearlDiver Mariner database. Time to recurrence was compared using Kaplan Meier analysis and Cox proportional hazards regression models. RESULTS: Hernia recurrence requiring reoperation occurred in 182 (2.2%) of 8,367 children. Rate of recurrence was higher following laparoscopic repair compared to open (1-year: 2.8% vs. 1.5%; 3-year: 3.7% vs. 2.0%; p < 0.01). This difference remained after adjustment for demographic and operative characteristics (adjusted hazard ratio [aHR]: 2.00 [95% confidence interval [CI]: 1.31, 3.05]). CONCLUSIONS: Risk of recurrence was higher after laparoscopic compared to open repair of bilateral inguinal hernia repair in a national cohort of children under age five.

The effect of inpatient versus outpatient location on postoperative healthcare utilization after neonatal circumcision.

BACKGROUND/PURPOSE: Neonatal circumcision is a common pediatric procedure performed in both the inpatient and outpatient setting. We aimed to determine if procedure location affected 30-day post-procedure healthcare utilization rates, inpatient length of stay (LOS), and amount charged. METHODS: We performed a retrospective cohort study comparing 30-day postoperative healthcare utilization (emergency department (ED) visits, office visits, readmissions) of full-term infants who underwent an outpatient versus inpatient (same admission as birth) circumcision from 2015 to 2020. Statistical analyses included Chi-square tests, multivariable adjusted logistic regression models when appropriate. RESULTS: 3137 infants were included, 1426 (45.5%) had an outpatient circumcision, 1711 (54.5%) an inpatient. Outpatient had similar overall healthcare utilization rates as inpatients (5.7% vs. 5.6%, p = 0.933). The number of ED visits (1.5% vs 0.8%, p = 0.055), office visits (4.5% vs. 5.1%, p = 0.437), and readmissions (0.2% vs. 0.0%, p = 0.058) were not significantly different. Infants with inpatient circumcisions had longer LOS after adjusting for age, ethnicity and delivery type (Cesarean versus vaginal) with an incident rate ratio of 1.97 (95% confidence interval 1.84-2.11, p<0.001). Outpatient circumcision resulted in average charges of $372 more than inpatient. CONCLUSIONS: Outpatient circumcision has a minimal effect on healthcare utilization rates but lead to a shorter hospital stay following birth and increased charge. STUDY DESIGN: Retrospective LEVEL OF EVIDENCE: III.

Percutaneous peritoneal drain placement: A pilot study of pediatric surgery simulation-based training for general surgery residents.

INTRODUCTION: General surgery residents often feel unprepared to perform pediatric surgery procedures since case volume and experience may be low. Previously, we successfully implemented a simulation-based training (SBT) module for placement of a silastic silo for gastroschisis. Therefore, we designed a single institution pilot study to assess whether SBT for placement of a percutaneous peritoneal drain for perforated necrotizing enterocolitis (NEC) was feasible and lead to skill acquisition and increased confidence. METHODS: Our newly created NEC module within our pediatric surgery SBT curriculum for general surgery residents was used. Residents completed two simulation sessions three months apart with confidence testing before and after each session. Skill acquisition and performance were assessed using a standardized case scenario and procedure checklist. Changes in residents' confidence and performance were determined using Wilcoxon Signed-Rank Tests. RESULTS: Nine post-graduate-year three general surgery residents completed this curriculum. Following completion, residents reported improved confidence completing each step of the procedure initially (p = 0.005) and at 3 months (p = 0.008) with improved technical scores (p = 0.011). The number of residents deemed proficient significantly improved (p = 0.031). CONCLUSION: Implementation of SBT module for perforated NEC was feasible and improved residents' confidence and proficiency completing the procedure.

The effect of standardized discharge instructions after gastrostomy tube placement on postoperative hospital utilization.

BACKGROUND/PURPOSE: Gastrostomy tube (GT) placement is a common pediatric procedure with high postoperative resource utilization. We aimed to determine if standardized discharge instructions (SDI) reduced healthcare utilization rates. METHODS: We performed a retrospective cohort study comparing postoperative hospital utilization of patients who underwent initial GT placement pre- and post-SDI protocol implementation from 2014-2019. Statistical analyses included Chi-square tests, multivariable adjusted logistic regression, adjusted Cox proportion hazard regression, and adjusted Poisson regression models when appropriate. RESULTS: 197 patients were included, 102 (51.8%) before and 95 (48.2%) after protocol implementation. On primary analysis, SDI patients did not have significantly different total postoperative hospital utilization events at 30-days (48.0% vs. 38.9%, p = 0.25). On secondary analysis, SDI patients had lower rates of ED (8.4% vs. 19.6%, p = 0.026) and office visits (11.6% vs. 25.5%, p = 0.017) at 30-days. Non-SDIs patients had greater odds of ED visits (OR2.7, 95%CI 1.3-5.9, p = 0.01), office visits (OR3.7, 95%CI 1.7-8.1, p = 0.001) and phone calls (OR2.6, 95%CI 1.2-5.7, p = 0.016) at 1-year. The adjusted hazard ratio was 2.0 (95%CI 1.4-3.0, p < 0.001). Incident rate ratio were 1.8 (95%CI 1.2-2.5, p = 0.002) at 30-days and 1.9 (95%CI 1.5-2.4, p < 0.001) at 1-year post-discharge. CONCLUSIONS: SDIs post-GT placement may reduce multiple aspects of postoperative hospital utilization.

Outcomes of Laparoscopic Versus Open Ladd Procedures and Risk Factors for Conversion.

Background: Malrotation is a common congenital anomaly that can lead to bowel obstruction and ischemia if not corrected with a Ladd procedure. Controversy exists between open and laparoscopic approaches. We sought to compare postoperative outcomes and determine risk factors for conversion to an open procedure. Methods: The National Surgical Quality Improvement Program (NSQIP)-Pediatric was used to identify patients undergoing Ladd procedures from 2013 to 2018. Propensity score matching was used to account for differences in patient characteristics between open and laparoscopically treated cohorts. Chi-square tests and adjusted logistic regression analysis were used to determine patient outcomes differences between treatment groups and factors associated with conversion. Results: A total of 2437 patients were identified, 1889 (77.5%) open, 548 (22.5%) laparoscopic, and 193 (35.2%) laparoscopic converted to open. Patients undergoing laparoscopic compared with open procedures had shorter length of stay (5 versus 7 days, P < .001) and lower overall complication rates (13.1% versus 18.1%, P = .025), despite longer operative times (108.9 versus 93.7 minutes, P < .001). Patients requiring conversion were more likely to be younger, have an urgent/emergent case, sepsis/septic shock, and nutritional support requirement. Conclusions: After risk adjustment, laparoscopic Ladd procedure is associated with decreased complications and minimal operative time increases compared with an open approach. Risk factors associated with conversion should be considered during operative planning.

Pediatric Surgery Simulation-Based Training for the General Surgery Resident.

BACKGROUND: Surgical simulation-based training (SBT) can increase resident confidence and improve performance. SBT in pediatric surgery is in its infancy and often geared toward training pediatric surgery fellows. Since case volume for various pediatric surgery-specific procedures can be low based on the rarity of the pathology involved and the level of care provided by the institution, our aim was to create a pediatric surgery simulation-based curriculum for general surgery residents to address this need. MATERIALS AND METHODS: We performed an institutional needs assessment consisting of 4 pediatric surgeons' and 28 general surgery residents' confidence in resident ability to independently perform pediatric surgery-specific tasks and procedures using a Likert-scaled survey. These included the placement of a silastic silo for gastroschisis, a percutaneous drain for perforated necrotizing enterocolitis, and completion of a laparoscopic pyloromyotomy for pyloric stenosis. Models simulating these pathologies and curriculum for performing each procedure were generated. RESULTS: We successfully created a model and SBT curriculum to teach general surgery residents how to place a silastic silo for patients with gastroschisis, a percutaneous drain for patients with perforated necrotizing enterocolitis, and how to complete a laparoscopic pyloromyotomy for patients with pyloric stenosis. These were deemed high fidelity models based on a survey of our pediatric surgeons. CONCLUSIONS: We created a pediatric surgery SBT curriculum for general surgery residents, which can be used to supplement learning of various high-acuity, low-occurrence procedures. Assessment of residents and validation of scores is underway.

Silo placement in gastroschisis: A pilot study of simulation-based training for general surgery residents.

INTRODUCTION: General surgery residents often feel unprepared for rotations on pediatric surgical services as case volume and experience performing pediatric procedures may be inadequate for high acuity, low volume procedures. We designed a single institution pilot study to assess whether simulation-based training (SBT) for placement of a silastic silo for gastroschisis was feasible and lead to skill acquisition, retention and increased resident confidence. METHODS: We used our newly created gastroschisis module within our pediatric surgery SBT curriculum for general surgery residents. Residents completed two simulation sessions three months apart, completed confidence testing before and after each session, and were assessed using a standardized case scenario and procedure checklist. Wilcoxon Signed-Rank Tests evaluated changes in residents' confidence and performance. RESULTS: Ten post-graduate-year three general surgery residents completed this curriculum. Residents reported improved confidence completing each step of the procedure initially (p=0.008) and at 3 months (p=0.005). They had improved technical scores across all steps of the procedure (p=0.005). The number of residents deemed proficient significantly improved (p=0.008). CONCLUSION: We demonstrated the feasibility of assessing the technical skills of general surgery residents performing a simulated placement of a silastic silo for gastroschisis. Residents' confidence and proficiency improved over the three-month period. STUDY TYPE: Prospective LEVEL OF EVIDENCE: Level II.

Mother's own milk dose is associated with decreased time from initiation of feedings to discharge and length of stay in infants with gastroschisis.

OBJECTIVE: To determine if mother's own milk (MOM) dose after gastroschisis repair is associated with time from feeding initiation to discharge. Secondary outcomes included parenteral nutrition (PN) duration and length of stay (LOS). STUDY DESIGN: Retrospective study of 44 infants with gastroschisis examined demographics, gastroschisis type, PN days, timing of nutrition milestones, feeding composition, and LOS. RESULTS: MOM dose was significantly associated with shorter time to discharge from feeding initiation (adjusted hazard ratio [HR] for discharge per 10% increase in MOM dose, 1.111; 95% CI, 1.011-1.220, p = 0.029). MOM dose was also significantly associated with shorter LOS (adjusted HR for discharge per 10% increase in MOM dose, 1.130; 95% CI, 1.028-1.242, p = 0.011). CONCLUSIONS: MOM dose was significantly associated with a decrease in time to discharge from feeding initiation and LOS in a dose-dependent manner. Mothers of gastroschisis patients should receive education and proactive lactation support to optimize MOM volume for feedings.

A national analysis of operative treatment of adult patients with Hirschsprung's disease.

PURPOSE: Hirschsprung's disease is primarily a disease of infancy, but in rare cases, adults with this condition require surgery. The aim of this study is to identify the types of operations and postoperative outcomes in adults with Hirschsprung's disease on a national level. METHODS: The National Surgical Quality Improvement Program database was used to perform a retrospective review of all adult patients diagnosed with Hirschsprung's disease. Patients were divided into two groups depending on the type of operation: restoration of bowel continuity or diversion of fecal stream; clinicopathologic data and 30-day outcomes were compared between the two groups. RESULTS: A total of 32 patients were analyzed. Fourteen patients (43.8%) underwent diversion and 18 (56.2%) underwent restorative procedures. The median age was 49.5 years old for the diversion group and 23.5 years old for the reconstructive group (p = 0.001). The restorative surgery group was more likely to have an ASA 1-2 while the diversion group had a higher frequency of ASA 3-5 (p = 0.011). The median length of stay for the diversion surgery was 9.5 days and 5 days for the restoration group (p = 0.045). Complications occurred in 57% of patients in the diversion group and in 22% of patients in the restoration group (p = 0.049). There were otherwise no statistically significant differences in intraoperative data and postoperative complications. CONCLUSION: This is the first study using a national database to evaluate the surgical treatment of Hirschsprung's disease in adult patients. Complications are common and were more frequent in the older, sicker diversion group, with restoration of continuity being better tolerated in the younger, healthier patient population.

Irreversible Electroporation as an Effective Technique for Ablating Human Metastatic Osteosarcoma.

Irreversible electroporation (IRE) induces apoptosis in tumor cells with electric energy, allowing treatment of unresectable tumors. One potential application is metastatic osteosarcoma (OS) in the pediatric population. A 12-year-old underwent thoracotomy with resection of metastatic OS. IRE was applied to 1 resected tumor section. Using 2 probes, 100 pulses with width of 90 ms were delivered. Efficacy was measured by increase in current draw during treatment. The treated sample was analyzed with hematoxylin and eosin and transmission electron microscopy. Default voltage of 1800 kV was ineffective. Voltage of 2700 kV caused excessive current draw and was aborted to prevent thermal injury. At 2200 kV, current draw rise was 9 amps, signifying successful treatment. Untreated specimen showed viable OS, normal surrounding lung tissue. Treated tumor had edema within the tumor and in surrounding lung tissue, with intra-alveolar hemorrhage and cellular architecture destruction. There was also evidence for cellular destruction such as disruption of lipid bilayer and release of intracellular fluid. Optimal voltage for treatment was 2200 kV, likely higher due to electrical conduction variation in the aerated lung. IRE may be an option for pediatric patients with unresectable metastatic OS.

Early Closure of Gastroschisis After Silo Placement Correlates with Earlier Enteral Feeding.

OBJECTIVES: Gastroschisis is a congenital anomaly affecting 2.3-4.4/10,000 births. Previous studies show initiation of early enteral feeds predicts improved outcomes. We hypothesize that earlier definitive closure after silo placement; can lead to earlier enteral feed initiation. Design/ Setting/ Duration: Retrospective review of patients with gastroschisis from 2005 and 2014 at a single institution. MATERIAL AND METHODS: The data, including ethnicity, gestational age, birth weight, time to definitive closure, and time of first and full feeds, were analyzed using both Spearman's rho and the Kruskal-Wallis rank sum test where appropriate; a p value less than 0.05 was considered significant. RESULTS: Forty-three patients (24 males, 19 females) born with gastroschisis were identified. Overall survival rate was 88% (38/43). Forty of the 43 patients had a silo placed prior to definitive closure. Median days to closure were 6 (0 to 85) days. First feeds on average began on day of life (DOL) 17, and full feeds on DOL 25. Earlier closure of gastroschisis correlated with early initiation of feeds (p=0.0001) and shorter time to full feeds (p=0.018), closure by DOL4 showed a trend toward earlier feeding (p=0.13). CONCLUSION: Earlier closure of gastroschisis after silo placement was associated with earlier feed initiation and shorter time to full feeds.

Case Volume and Outcomes of Congenital Diaphragmatic Hernia Surgery in Academic Medical Centers.

OBJECTIVE: The outcome of patients with congenital diaphragmatic hernia (CDH) has not improved in the last decade and surgical repair remains the mainstay of treatment. The purpose of the present study was to assess whether a volume-outcome relationship exists in the U.S. academic medical centers performing surgical repair of neonatal CDH. STUDY DESIGN: A retrospective cross-sectional analysis of discharge data for neonates undergoing CDH repair in academic medical center members of the University Health-System Consortium was employed. Unadjusted mortality was compared between lower and higher surgical volume centers. A binary logistic regression model was fit to test the relationship of surgical volume with mortality. RESULTS: A total of 3,738 patients underwent surgical repair in 122 unique academic medical centers in the United States. The overall rate of survival was 75.2%. There was no difference in unadjusted mortality between lower and higher volume centers. After controlling for patient and hospital variables, there was no difference in the odds of mortality between lower and higher volume centers (odds ratio 1.03 [95% confidence interval, 0.86-1.23, p = 0.730]). CONCLUSIONS: Neonates born with congenital diaphragmatic hernia can undergo surgical repair in the U.S. academic medical centers independent of center procedure volume and expect good surgical outcomes.

Serous borderline tumor of the fallopian tube in a patient with Klippel-Trenaunay syndrome.

We describe a case of a 3-year-old girl with Klippel-Trenaunay syndrome who presented with an enlarging abdominal mass caused by a serous borderline tumor of the fallopian tube. This case is notable for the rarity of this neoplasm in a premenarchal patient as well as the association with this syndrome. We briefly review these entities and the significance of malignancy in Klippel-Trenaunay syndrome.

Congenital absence of intestinal smooth muscle: a case report and review of the literature.

Herein is reported case of an otherwise healthy full-term infant girl who presented with numerous spontaneous intestinal perforations with congenital absence of intestinal muscularis mucosae and muscularis propria. Few other cases are reported in the English literature with varying presentations. We review those cases, theories of pathogenesis, embryology, and possible connections to various clinical presentations.