Charles M. Leys

Laparoscopic Adrenalectomy in Children: A Multicenter Experience

INTRODUCTION Laparoscopic adrenalectomy is now being recognized as the standard approach for adrenalectomy for benign lesions in adults. The published experience in children and adolescents has been limited to sporadic small case series. Therefore, we conducted a large multicenter review of children who have undergone laparoscopic adrenalectomy. METHODS After Institutional Review Boards approval, a retrospective review was conducted on all patients who have undergone laparoscopic adrenalectomy at 12 institutions over the past 10 years. Operative times included unilateral adrenalectomy without concomitant procedures. RESULTS About 140 patients were identified (70 males [50%]). Laterality included 76 (54.3%) left-sided lesions, 59 (42.1%) right, and 5 (3.6%) bilateral. Mean operative time was 130.2 ± 63.5 minutes (range 43-406 minutes). The most common pathology was neuroblastoma in 39 cases (27.9%), of which 23 (59.0%) had undergone preoperative chemotherapy. Other common pathology included 30 pheochromocytomas (21.4%), 22 ganglioneuromas (15.7%), and 20 adenomas (14.3%). There were 13 conversions to an open operation (9.9%). Most conversions were because of tumor adherence to surrounding organs, and tumor size was not different in converted cases (P=.97). A blood transfusion was required in 2 cases. The only postoperative complication was renal infarction after resection of a large neuroblastoma that required skeletonization of the renal vessels. At a median follow-up of 18 months, there was only one local recurrence, which was in a patient with a pheochromocytoma. CONCLUSIONS The laparoscopic approach can be applied for adrenalectomy in children for a wide variety of conditions regardless of age with a 90% chance of completing the operation without conversion. The risk for significant blood loss or complications is low, and it should be considered the preferred approach for the majority of adrenal lesions in children.

Laparoscopic Removal of a Gastric Trichobezoar in a Pediatric Patient

Trichobezoars from the ingestion of hair typically develop into a large, spherical foreign body within the stomach. Due to the frequently large size, endoscopic removal is usually not feasible beyond diagnosis. Likewise, the size often has forced surgeons to remove these trichobezoars via laparotomy. Laparoscopic removal of gastric trichobezoars has been reported, but the concern with this technique is that the complete removal of the large bezoar without spillage of hairs in the peritoneal cavity may be difficult. As such, most of the reports are laparoscopic assisted, but still utilize an extended incision to facilitate direct removal from the peritoneal cavity. In this case, we describe in this paper the complete laparoscopic removal of a giant trichobezoar in a 10-year-old child without spillage, using piecemeal removal through the umbilical incision without extending the incisions.

Perioperative management and outcomes of esophageal atresia and tracheoesophageal fistula

BACKGROUND/PURPOSE Esophageal atresia/tracheoesophageal fistula (EA/TEF) is a rare congenital anomaly lacking contemporary data detailing patient demographics, medical/surgical management and outcomes. Substantial variation in the care of infants with EA/TEF may affect both short- and long-term outcomes. The purpose of this study was to characterize the demographics, management strategies and outcomes in a contemporary multi-institutional cohort of infants diagnosed with EA/TEF to identify potential areas for standardization of care. METHODS A multi-institutional retrospective cohort study of infants with EA/TEF treated at 11 childrens hospitals between 2009 and 2014 was performed. Over the 5year period, 396 cases were identified in the 11 centers (7±5 per center per year). All infants with a diagnosis of EA/TEF made within 30days of life who had surgical repair of their defect defined as esophageal reconstruction with or without ligation of TEF within the first six months of life were included. Demographic, operative, and outcome data were collected and analyzed to detect associations between variables. RESULTS Prenatal suspicion or diagnosis of EA/TEF was present in 53 (13%). The most common anatomy was proximal EA with distal TEF (n=335; 85%) followed by pure EA (n=27; 7%). Clinically significant congenital heart disease (CHD) was present in 137 (35%). Mortality was 7.5% and significantly associated with CHD (p<0.0001). Postoperative morbidity occurred in 62% of the population, including 165 (42%) cases with anastomotic stricture requiring intervention, anastomotic leak in 89 (23%), vocal cord paresis/paralysis in 26 (7%), recurrent fistula in 19 (5%), and anastomotic dehiscence in 9 (2%). Substantial variation in practice across our institutions existed: bronchoscopy prior to repair was performed in 64% of cases (range: 0%-100%); proximal pouch contrast study in 21% (0%-69%); use of interposing material between the esophageal and tracheal suture lines in 38% (0%-69%); perioperative antibiotics ≥24h in 69% (36%-97%); and transanastomotic tubes in 73% (21%-100%). CONCLUSION Contemporary treatment of EA/TEF is characterized by substantial variation in perioperative management and considerable postoperative morbidity and mortality. Future studies are planned to establish best practices and clinical care guidelines for infants with EA/TEF. LEVEL OF EVIDENCE Type of study: Treatment study. Level IV.

A call for a standardized definition of perforated appendicitis.

BACKGROUND Abscess rates have been reported to be as low as 1% and as high as 50% following perforated appendicitis (PA). This range may be because of lack of universal definition for PA. An evidence-based definition (EBD) is crucial for accurate wound classification, risk-stratification, and subsequent process optimization. ACS NSQIP-Pediatric guidelines do not specify a definition of PA. We hypothesize that reported postoperative abscess rates underrepresent true incidence, as they may include low-risk cases in final calculations. METHODS Local institutional records of PA patients were reviewed to calculate the postoperative abscess rate. The ACS NSQIP-Pediatric participant use file (PUF) was used to determine cross-institutional postoperative abscess rates. A PubMed literature review was performed to identify trials reporting PA abscess rates, and definitions and rates were recorded. RESULTS 20.9% of our patients with PA developed a postoperative abscess. The ACS NSQIP-Pediatric abscess rate was significantly lower (7.61%, p<0.001). In the eighteen published studies analyzed, average abscess rate (14.49%) was significantly higher than ACS NSQIP-Pediatric (p<0.001). There was significantly more variation in trials that do not employ an EBD of perforation (Levenes test F-value =6.980, p=0.018). CONCLUSIONS A standard EBD of perforation leads to lower variability in reported postoperative abscess rates following PA. Nonstandard definitions may be significantly altering the aggregate rate of postoperative abscess formation. We advocate for adoption of a standard definition by all institutions participating in ACS NSQIP-Pediatric data submission. LEVEL OF EVIDENCE III.

Screening for occult nodal metastasis in squamous cell carcinoma of the vulva.

Metastases to inguinofemoral lymph nodes in patients with carcinoma of the vulva alter the prognosis and treatment of this disease. Our goal was to determine if immunohistochemical staining could reveal occult metastatic nodal disease not detected with routine hematoxylin and eosin staining. We retrospectively examined a total of 110 lymph nodes from 10 patients who had undergone lymph node dissection and found to have all negative nodes. Paraffin embedded lymph nodes were immunostained with a monoclonal antibody directed against multiple low-and high-molecular weight cytokeratins. Micrometastases were not detected in any lymph nodes examined with immunohistochemistry. All positive and negative controls yielded satisfactory results. It is concluded that immunohistochemistry with cytokeratin antibodies does not provide greater sensitivity than routine hematoxylin and eosin staining for the detection of nodal metastases in vulvar carcinoma.

Challenging surgical dogma in the management of proximal esophageal atresia with distal tracheoesophageal fistula: Outcomes from the Midwest Pediatric Surgery Consortium

PURPOSE Perioperative management of infants with esophageal atresia and tracheoesophageal fistula (EA/TEF) is frequently based on surgeon experience and dogma rather than evidence-based guidelines. This study examines whether commonly perceived important aspects of practice affect outcome in a contemporary multi-institutional cohort of patients undergoing primary repair for the most common type of esophageal atresia anomaly, proximal EA with distal TEF. METHODS The Midwest Pediatric Surgery Consortium conducted a multicenter, retrospective study examining selected outcomes on infants diagnosed with proximal EA with distal TEF who underwent primary repair over a 5-year period (2009-2014), with a minimum 1-year follow up, across 11 centers. RESULTS 292 patients with proximal EA and distal TEF who underwent primary repair were reviewed. The overall mortality was 6% and was significantly associated with the presence of congenital heart disease (OR 4.82, p=0.005). Postoperative complications occurred in 181 (62%) infants, including: anastomotic stricture requiring intervention (n=127; 43%); anastomotic leak (n=54; 18%); recurrent fistula (n=15; 5%); vocal cord paralysis/paresis (n=14; 5%); and esophageal dehiscence (n=5; 2%). Placement of a transanastomotic tube was associated with an increase in esophageal stricture formation (OR 2.2, p=0.01). Acid suppression was not associated with altered rates of stricture, leak or pneumonia (all p>0.1). Placement of interposing prosthetic material between the esophageal and tracheal suture lines was associated with an increased leak rate (OR 4.7, p<0.001), but no difference in the incidence of recurrent fistula (p=0.3). Empiric postoperative antibiotics for >24h were used in 193 patients (66%) with no difference in rates of infection, shock or death when compared to antibiotic use ≤24h (all p>0.3). Hospital volume was not associated with postoperative complication rates (p>0.08). Routine postoperative esophagram obtained on day 5 resulted in no delayed/missed anastomotic leaks or a difference in anastomotic leak rate as compared to esophagrams obtained on day 7. CONCLUSION Morbidity after primary repair of proximal EA and distal TEF patients is substantial, and many common practices do not appear to reduce complications. Specifically, this large retrospective series does not support the use of prophylactic antibiotics beyond 24h and empiric acid suppression may not prevent complications. Use of a transanastomotic tube was associated with higher rates of stricture, and interposition of prosthetic material was associated with higher leak rates. Routine postoperative esophagram can be safely obtained on day 5 resulting in earlier initiation of oral feeds. STUDY TYPE Treatment study. LEVEL OF EVIDENCE III.

Laparoscopic Resection of Massive Ovarian Mucinous Cystadenoma

Benign mucinous cystadenoma of the ovary is extremely rare in children and adolescents. Because of the benign, slow-growing nature, they can be extremely large at the point of presentation. These large abdominal tumors present a challenging opportunity for the application of minimally invasive techniques. The authors present a case of an extraordinarily large childhood mucinous cystadenoma removed laparoscopically.

Prospective validation of the shock index pediatric-adjusted (SIPA) in blunt liver and spleen trauma: An ATOMAC + study☆

BACKGROUND Age-adjusted pediatric shock index (SIPA) does not require knowledge of age-adjusted blood pressure norms, yet correlates with mortality, serious injury, and need for transfusion in trauma. No prospective studies support its validity. METHODS A multicenter prospective observational study of patients 4-16years presenting April 2013-January 2016 with blunt liver and/or spleen injury (BLSI). SIPA (maximum heart rate/minimum systolic blood pressure) thresholds of >1.22, >1.0, and >0.9 in the emergency department were used for 4-6, 7-12 and 13-16year-olds, respectively. Patients with ISS ≤15 were excluded to conform to the original paper. Discrimination outcomes were compared between SIPA and shock index (SI). RESULTS Of 1008 patients, 386 met inclusion. SI was elevated in 321, and SIPA elevated in 282. The percentage of patients with elevated index (SI or SIPA) and blood transfusion within 24 hours (30% vs 34%), BLSI grade ≥3 requiring transfusion (28% vs 32%), operative intervention (14% vs 16%) and ICU admission (64% vs 67%) was higher in the SIPA group. CONCLUSION SIPA was validated in this multi-institutional prospective study and identified a higher percentage of children requiring additional resources than SI in BLSI patients. SIPA may be useful for determining necessary resources for injured patients with BLSI. LEVEL OF EVIDENCE Level II prognosis.

Computed tomography-related radiation exposure in children transferred to a Level i pediatric trauma center

PURPOSE Pediatric trauma patients presenting to referring facilities (RF) often undergo computed tomography (CT) scans to identify injuries before transfer to a Level I pediatric trauma center (PTC). The purpose of our study was to evaluate RF compliance with the American College of Radiology (ACR) guidelines to minimize ionizing radiation exposure in pediatric trauma patients and to determine the frequency of additional or repeat CT imaging after transfer to a PTC. METHODS After institutional review board approval, a retrospective review of all pediatric trauma admissions from January 2010 to December 2011 at our American College of Surgeons Level I PTC was performed. Patient demographics, means of arrival, Injury Severity Score, and disposition were analyzed. Patients who underwent CT were grouped by means of arrival: those who were transferred from an RF versus those who presented primarily to the PTC. Compliance with ACR guidelines and need for additional or repeat CT scans were assessed for both groups. RESULTS Six hundred ninety-seven children (aged <18 years) were identified, with a mean age of 10.6 years. Three hundred twenty-one (46%) patients presented primarily to the PTC. Three hundred seventy-six (54%) were transferred from an RF, of which 90 (24%) patients underwent CT imaging before transfer. CT radiation dosing information was available for 79 (88%) of 90 patients. After transfer, 8 (9%) of 90 of children imaged at an RF required additional CT scans. In comparison, 314 (98%) of 321 patients who presented primarily to the PTC and underwent CT received appropriate pediatric radiation dosing. Mean radiation dose at PTC was approximately half of that at RF for CT scans of the head, chest, and abdomen/pelvis (p < 0.01). CONCLUSION Pediatric trauma patients transferred from RF often undergo CT scanning with higher than recommended radiation doses, potentially placing them at an increased carcinogenic risk. Fortunately, few RF patients required additional CT scans after PTC transfer. Finally, compliance with ACR radiation dose limit guidelines is better achieved at a PTC. LEVEL OF EVIDENCE Care management study, level IV.

Congenital H-type tracheoesophageal fistula: A multicenter review of outcomes in a rare disease

OBJECTIVE To perform a multicenter review of outcomes in patients with H-type tracheoesophageal fistula (TEF) in order to better understand the incidence and causes of post-operative complications. BACKGROUND H-type TEF without esophageal atresia (EA) is a rare anomaly with a fundamentally different management algorithm than the more common types of EA/TEF. Outcomes after surgical treatment of H-type TEF are largely unknown, but many authoritative textbooks describe a high incidence of respiratory complications. METHODS A multicenter retrospective review of all H-type TEF patients treated at 14 tertiary childrens hospital from 2002-2012 was performed. Data were systematically collected concerning associated anomalies, operative techniques, hospital course, and short and long-term outcomes. Descriptive analyses were performed. RESULTS We identified 102 patients (median 9.5 per center, range 1-16) with H-type TEF. The overall survival was 97%. Most patients were repaired via the cervical approach (96%). The in-hospital complication rate, excluding vocal cord issues, was 16%; this included an 8% post-operative leak rate. Twenty-two percent failed initial extubation after repair. A total of 22% of the entire group had vocal cord abnormalities (paralysis or paresis) on laryngoscopy that were likely because of recurrent laryngeal nerve injury. Nine percent required a tracheostomy. Only 3% had a recurrent fistula, all of which were treated with reoperation. CONCLUSIONS There is a high rate of recurrent laryngeal nerve injury after H-type TEF repair. This underscores the need for meticulous surgical technique at the initial repair and suggests that early vocal cord evaluation should be performed for any post-operative respiratory difficulty. Routine evaluation of vocal cord function after H-type TEF repair should be considered. THE LEVEL OF EVIDENCE RATING Level IV.