Laparoscopic needle-assisted inguinal hernia repair in 495 children.

Minimally invasive surgery for inguinal hernia repair in children has been a controversial topic for pediatric surgeons. Our method for inguinal hernia repair using laparoscopic techniques has comparable outcomes to the standard open technique. We describe our technique and experience with the laparoscopic needle-assisted repair of inguinal hernia (LNAR). We report 502 cases (710 hernias) from 2009 to 2013 by 3 surgeons. We reviewed our prospectively collected outcomes database of all patients receiving LNAR from 1/2009 to 3/2013. 502 cases in 495 patients <13 years old with 710 inguinal hernias were identified for analysis and review. Hernia repair is accomplished with a single-port needle-assisted technique. After identification of a patent processus vaginalis, the internal ring is encircled in an extraperitoneal plane using a 22G-Touhy needle for placement of a purse-string suture, tied extracorporally, and buried beneath the skin. The technique was standardized for all cases. 710 inguinal hernias were laparoscopically repaired in 495 patients (408 boys and 87 girls) age range 11 days to 12.8 years (mean 29.2 months; median 15.5 months). 294 patients had unilateral repair (199R and 95L) and 208 had bilateral repair. Mean operating time for unilateral was 20.5 min, and bilateral was 26.4 min. 21 minor complications were identified (9 superficial wound infections, 8 suture granulomas, and 4 recurrent hydroceles) and 4 recurrences. Mean time since surgery is 30 months (3-54 months). Mean follow-up was 10.7 months (0.3-38.4 months). Post-operative data show our technique is safe with a 4 % rate of minor complication. Recurrence rate was 0.56 % for the total number of hernias (4/710). This recurrence rate is comparable and in many cases less than open technique. Furthermore, laparoscopy objectively identifies asymptomatic or occult contralateral defect, uses a smaller incision, and eliminates dissection of the cord structures potentially reducing the risk of cord injury.

Clinical practice guidelines for pediatric appendicitis evaluation can decrease computed tomography utilization while maintaining diagnostic accuracy.

OBJECTIVES: The objective of this study was to compare usage of computed tomography (CT) scan for evaluation of appendicitis in a children's hospital emergency department before and after implementation of a clinical practice guideline focused on early surgical consultation before obtaining advanced imaging. METHODS: A multidisciplinary team met to create a pathway to formalize the evaluation of pediatric patients with abdominal pain. Computed tomography scan utilization rates were studied before and after pathway implementation. RESULTS: Among patients who had appendectomy in the year before implementation (n = 70), 90% had CT scans, 6.9% had ultrasound, and 5.7% had no imaging. The negative appendectomy rate before implementation was 5.7%. In patients undergoing appendectomy in the postimplementation cohort (n = 96), 48% underwent CT, 39.6% underwent ultrasound, and 15.6% had no imaging. The negative appendectomy rate was 5.2%. We demonstrated a 41% decrease in CT use for patients undergoing appendectomy at our institution without an increase in the negative appendectomy rate or missed appendectomy. The results were even more striking when comparing the rate of CT scan use in the subset of patients undergoing appendectomy without imaging from an outside hospital. In these patients, CT scan utilization decreased from 82% to 20%, a 76% reduction in CT use in our facility after protocol implementation. CONCLUSIONS: Implementation of a clinical evaluation pathway emphasizing examination, early surgeon involvement, and utilization of ultrasound as the initial imaging modality for evaluation of abdominal pain concerning for appendicitis resulted in a marked decrease in the reliance on CT scanning without loss of diagnostic accuracy.

Celiac disease and diabetes mellitus diagnosed in a pediatric patient with Hirschsprung disease.

Hirschsprung disease is a disorder of neural crest migration characterized by intestinal aganglionosis along a variable segment of the gastrointestinal tract. It is a complex disorder associated with several syndromes. Celiac disease is an autoimmune enteropathy characterized by dietary intolerance to gluten proteins and can be associated with autoimmune conditions such as diabetes mellitus. Celiac disease can mimic Hirschsprung disease when presenting with constipation and abdominal distention. We present the case of celiac disease diagnosed in a patient with Hirschsprung disease who subsequently developed type one diabetes mellitus.

Increasing severity of pectus excavatum is associated with reduced pulmonary function.

OBJECTIVE: To determine whether pulmonary function decreases as a function of severity of pectus excavatum, and whether reduced function is restrictive or obstructive in nature in a large multicenter study. STUDY DESIGN: We evaluated preoperative spirometry data in 310 patients and lung volumes in 218 patients aged 6 to 21 years at 11 North American centers. We modeled the impact of the severity of deformity (based on the Haller index) on pulmonary function. RESULTS: The percentages of patients with abnormal forced vital capacity (FVC), forced expiratory volume in 1 second (FEV(1)), forced expiratory flow from 25% exhalation to 75% exhalation, and total lung capacity findings increased with increasing Haller index score. Less than 2% of patients demonstrated an obstructive pattern (FEV(1)/FVC <67%), and 14.5% demonstrated a restrictive pattern (FVC and FEV(1) <80% predicted; FEV(1)/FVC >80%). Patients with a Haller index of 7 are >4 times more likely to have an FVC of ≤80% than those with a Haller index of 4, and are also 4 times more likely to exhibit a restrictive pulmonary pattern. CONCLUSIONS: Among patients presenting for surgical repair of pectus excavatum, those with more severe deformities have a much higher likelihood of decreased pulmonary function with a restrictive pulmonary pattern.

Excision of esophageal duplication cysts with robotic-assisted thoracoscopic surgery.

Esophageal duplication cysts are infrequent anomalies of the gastrointestinal tract that are predominantly found in children. The conventional surgical approach for removal of these cysts is an open surgery one with a posterolateral thoracotomy incision. However, more recently, these cysts have been excised via video-assisted thoracoscopic surgery (VATS). In this article, we present 2 pediatric patients treated with successful excision of an esophageal duplication cyst via robotic-assisted thoracoscopic surgery (RATS) using the da Vinci surgical system. With robotic technology, precise dissection and complete resection of the thoracic mass was achieved without violating the esophageal mucosa. There were no complications, and the patients did not require placement of a postoperative chest tube. Pathological examination of the mass was consistent with an esophageal (foregut) duplication cyst in both cases.

Pectus bar removal - why, when, where and how.

OBJECTIVE: Despite its less invasive nature, the widespread use of the minimally invasive repair of pectus excavatum (MIRPE) procedure has been associated with a significant number of serious complications. On the other hand, Pectus bar removal (PBR) is often considered a simple procedure and often scheduled in an outpatient setting. However, several studies report near-fatal complications not only during bar placement, but also during bar removal. The aim of our study was to clarify why a pectus bar should be removed, timing for removal, where PBR should be performed, and overall setup for safe removal. METHODS: A comprehensive review was performed in accordance with PRISMA guidelines, searching for articles published since 1998 in English. "Pectus bar removal AND (near-fatal) complications" were the applied terms. Inclusion criteria were articles reporting on the focus of PBR after MIRPE. Eligible study designs included (retrospective) case study series, case report and reviews. Full-text articles in which the technique in general was described were omitted. RESULTS: Recently published results of an online survey raised awareness about type and number of possible complications during PBR. Furthermore, our comprehensive literature review identified only a few, but serious complications during PBR. CONCLUSIONS: PBR has a high safety profile but in rare cases may be associated with major complications such as life-threatening hemorrhage from various thoracic sources. This risk is higher in patients with a history of complex MIPRE. In an effort to decrease these complications we recommend bilateral opening of surgical incisions, unbending the bar and meticulous mobilization of the bar. To manage these complications if they occur, we recommend removal in a hospital setting with adequate resources and personal including cardiac surgeons. If the postoperative course is uneventful discharge on the same day is reasonable.

Long-term outcomes of pediatric laparoscopic needled-assisted inguinal hernia repair: A 10-year experience.

PURPOSE: Laparoscopic inguinal hernia repair (LIHR) has gained wide acceptance over the past decade, although studies with longer term follow-up are lacking. We present one of the largest cohorts of children undergoing laparoscopic needle-assisted repair (LNAR) with long-term follow-up. METHODS: A clinical quality database was maintained for children ≤14 years of age who underwent laparoscopic needle-assisted repair between 2009 and 2017 with review of follow-up through 2019. De-identified data was reviewed. RESULTS: 1023 patients with 1457 LNAR were included during the 10-year period. Mean age at surgery was 2.56 years (2 days to14 years). The overall hernia recurrence rate was 0.75% (11/1457). A total of four postoperative hydroceles required intervention. Preterm infant repair done <60w post conceptional age had a significantly lower recurrence rate (0.63%) than other patients (0.82%) (p < 0.01). 64.2% of patients had clinical follow-up over a period of 11 years with a mean follow-up of 5.97 years. CONCLUSION: We present a large cohort study of consecutive pediatric laparoscopic hernia repairs followed over an 11-year period. LNAR is safe and effective for term and preterm patients with similar complication rates to other techniques, including open repair. Additionally, our results suggest that preterm infants may have superior outcomes with this method. LEVEL OF EVIDENCE: Level III - Retrospective Comparative Study.

Successful treatment of late, recurrent wilms tumor with high-dose chemotherapy and autologous stem cell rescue in third complete response.

SUMMARY: Wilms tumor relapses are infrequent, occurring in approximately 15% of favorable histology patients. Very few cases of late recurrent relapse exist in the literature. Long-term survival after autologous stem cell rescue ranges from 40% to 73%, but there are very few reports of patients transplanted in their third complete response. We report a late recurrent relapse of Wilms tumor successfully treated with high-dose chemotherapy and autologous stem cell rescue in his third complete response who remains disease free 15 months posttransplant.

Minimally invasive repair of pectus excavatum.

The minimally invasive approach for repair of pectus excavatum has become widely accepted by pediatric and thoracic surgeons primarily because of increased patient awareness and good long-term outcomes. Although the technique is considered fairly new, it was quickly embraced by many, resulting in a significant increase in the number of such procedures being performed in North America over the last decade. Early reports demonstrated the potential risks and complications of this procedure. Important technical modifications were implemented, and the operation as well as selection criteria for surgery were modified to minimize risks and improve overall outcomes. This article reviews the indications for treatment of patients with pectus excavatum, with emphasis on the evolution and outcomes of the procedure known as the minimally invasive repair or Nuss technique.

To Nuss or not to Nuss? Two opposing views.

Although the issue of the appropriate approach for the repair of pectus excavatum remained unsettled for decades, just when we thought that the consensus was clear, an entirely new method was introduced: the Nuss operation. This technique now challenges not only the previously established standards, but also the basic conceptual views of pectus surgery. In the following text, 2 opposing views on the subject are presented: the angle from which Francis Robicsek, a pioneer in conventional pectus excavatum surgery, views the issue, and the opinion of Andre Hebra, who has extensive experience with the Nuss operation.

Volvular small bowel obstruction secondary to adherence of a Meckel's diverticulum at a previous umbilical laparoscopic port site.

This is a case of an otherwise asymptomatic Meckel's diverticulum, which became fibrously adherent to a previous umbilical laparoscopic port site, causing volvulus and small bowel obstruction in a pediatric patient. The diverticulum was diagnosed and resected laparoscopically, remaining bowel viability was maintained, and the child recovered without further sequelae. This complication, though rare, should be considered in the differential diagnosis when a child presents with abdominal pain after undergoing previous laparoscopic surgery. More important, this supports the consideration for the resection of asymptomatic Meckel's diverticulm when discovered incidentally, which is currently a controversial topic.

Minor and Major Complications Related to Minimally Invasive Repair of Pectus Excavatum.

The technique for minimally invasive repair of pectus excavatum (MIRPE) has been adopted by most surgeons as the preferred method for treatment of this condition. However, there is limited awareness about the complications related to the procedure. Most importantly, the prevalence and potential for life-threatening complications have frequently been underestimated. The purpose of this communication is to increase awareness of the risk of minor and major (life-threatening) complications, as well as the operative steps and modifications which have been developed to prevent them. One of the most common complications reported in the early series of MIRPE cases was bar displacement, frequently requiring reoperation. Fortunately, technical modifications to the technique have resulted in a decrease in the incidence of such events from 10 to less than 2%. It is important for surgeons to have a good understanding of the potential complications, particularly the major type of complications post-MIRPE and postpectus bar removal. Although rare, the risk of mortality is real and it is the surgeon's responsibility to ensure optimal patient safety when performing this procedure. Factors such as operative technique, patient age, pectus severity and asymmetry, previous chest surgery, and the surgeon's experience play a role in the overall incidence of adverse events. These preventable events can be avoided with proper training, mentoring, and careful patient selection.

Hurricane Irma Impact on the Inpatient Population at a Tertiary Children's Hospital in Florida.

Hurricane Irma resulted in the evacuation of 6.3 million people in Florida in September, 2017. Our tertiary Children's Hospital activated our incident command center (ICC) 24 hours before storm landfall, and preparations were made to accommodate vulnerable pediatric patients (VPP) or children with medical complexity. Our ICC was active for 92 hours and the hospital was staffed with 467 associates and 40 physicians. Urgent operative and interventional radiology procedures were performed during the storm. Thirteen patients were transferred to our facility and 13 VPP were sheltered. During the lockdown period, our facility operated at 90 per cent capacity inclusive of VPP. Personnel were used in critical areas in the hospital, independent of their base units. There were no adverse outcomes or complications. Timely activation of ICC and deployment of Team A 24 hours before storm hit allowed for safe hospital operations. Planning for the inflow of patients is imperative to allow for preemptive deployment of staff and resources for inpatients, transfers, emergency room admissions, and VPP. VPP should be monitored regionally as they will consume hospital resources during natural disasters and must be accounted for to allow for safe and effective care delivery for all patients.

Life-threatening complications and mortality of minimally invasive pectus surgery.

The prevalence and type of life-threatening complications related to the minimally invasive repair of pectus excavatum (MIRPE) and bar removal are unknown and underreported. The purpose of this communication is to make surgeons aware of the risk of these life threatening complications as well as the modifications which have been developed to prevent them. METHODS: Data related to life-threatening complications of Pectus Excavatum (PE) patients was obtained from four sources: 1. A survey of Chest Wall International Group (CWIG) surgeons who specialize in repairing congenital chest wall malformations, 2. Papers and case reports presented at CWIG meetings, 3. Review of medico-legal cases from the USA and 4. A systematic review of the literature related to major complications post MIRPE. RESULTS: From 1998 to 2016, we identified 27 published cases and 32 unreported life-threatening complications including: cardiac perforation, hemothorax, major vessel injury, lung injury, liver injury, gastrointestinal problems, and diaphragm injury. There were seven cases of major complications with bar removal (reported and non-reported) with two lethal outcomes. Mortality data with bar placement surgery: Four published death cases and seven unpublished death cases. The overall incidence of minor & major complications post MIRPE has been reported in the literature to be 2-20%. The true incidence of life-threatening complications and mortality is not known as we do not know the overall number of procedures performed worldwide. However, based on data extrapolated from survey information, the pectus bar manufacturer in the USA, literature reports, and data presented at CWIG meetings as to the number of cases performed we estimated that approximately fifty thousand cases have been performed and that the incidence of life-threatening complications is less than 0.1% with many occurring during the learning curve. Analysis of the cases identified in our survey revealed that previous chest surgery, pectus severity and inexperience were noted to be significant risk factors for mortality. CONCLUSIONS: Published reports support the safety and efficacy of MIRPE; however major adverse outcomes are underreported. Although major complications with MIRPE and pectus bar removal surgery are very rare, awareness of the risk and mortality of life-threatening complications is essential to ensure optimal safety. Factors such as operative technique, patient age, pectus severity and asymmetry, previous chest surgery, and the surgeon's experience play a role in the overall incidence of such events. These preventable events can be avoided with proper training, mentoring, and the use of sternal elevation techniques. TYPE OF STUDY: Treatment Study. LEVEL OF EVIDENCE: Level IV.

Prospective multicenter study of surgical correction of pectus excavatum: design, perioperative complications, pain, and baseline pulmonary function facilitated by internet-based data collection.

BACKGROUND: Given widespread adoption of the Nuss procedure, prospective multicenter study of management of pectus excavatum by both the open and Nuss procedures was thought desirable. Although surgical repair has been performed for more than 50 years, there are no prospective multicenter studies of its management. STUDY DESIGN: This observational study followed pectus excavatum patients treated surgically at 11 centers in North America, according to the method of choice of the patient and surgeon. Before operation, all underwent evaluation with CT scan, pulmonary function tests, and body image survey. Data were collected about associated conditions, hospital complications, and perioperative pain. One year after completion of treatment, patients will repeat the preoperative evaluations. This article addresses early results only. RESULTS: Of 416 patients screened, 327 were enrolled; 284 underwent the Nuss procedure and 43 had the open procedure. Median preoperative CT index was 4.4. Pulmonary function testing before operation showed mean forced vital capacity of 90% of predicted values; forced expiratory volume in 1 second (FEV(1)), 89% of predicted; and forced expiratory flow during the middle half of the forced vital capacity (FEF(25% to 75%)), 85% of predicted. Early postcorrection results showed that operations were performed without mortality and with minimal morbidity at 30 days postoperatively. Median hospital stay was 4 days. Postoperative pain was a median of 3 on a scale of 10 at time of discharge; the worst pain experienced was the same as was expected by the patients (median 8), and by 30 days after correction or operation, the median pain score was 1. Because of disproportionate enrollment and similar early complication rates, statistical comparison between operation types was limited. CONCLUSIONS: Anatomically severe pectus excavatum is associated with abnormal pulmonary function. Initial operative correction performed at a variety of centers can be completed safely. Perioperative pain is successfully managed by current techniques.

Pectus excavatum repair after sternotomy: the Chest Wall International Group experience with substernal Nuss bars.

OBJECTIVES: Patients with pectus excavatum (PE) after prior sternotomy for cardiac surgery present unique challenges for repair of PE. Open repairs have been recommended because of concerns about sternal adhesions and cardiac injury. We report a multi-institutional experience with repair utilizing substernal Nuss bars in this patient population. METHODS: Surgeons from the Chest Wall International Group were queried for experience and retrospective data on PE repair using sub-sternal Nuss bars in patients with a history of median sternotomy for cardiac surgery (November 2000 to August 2015). A descriptive analysis was performed. RESULTS: Data for 75 patients were available from 14 centres. The median age at PE repair was 9.5 years (interquartile range 10.9), and the median Haller index was 3.9 (interquartile range 1.43); 56% of the patients were men. The median time to PE repair was 6.4 years (interquartile range 7.886) after prior cardiac surgery. Twelve patients (16%) required resternotomy before support bar placement: 7 pre-emptively and 5 emergently. Sternal elevation before bar placement was used in 34 patients (45%) and thoracoscopy in 67 patients (89%). Standby with cardiopulmonary bypass was available at 9 centres (64%). Inadvertent cardiac injury occurred in 5 cases (7%) without mortality. CONCLUSIONS: Over a broad range of institutions, substernal Nuss bars were used in PE repair for patients with a history of sternotomy for cardiac surgery. Several technique modifications were reported and may have facilitated repair. Cardiac injury occurred in 7% of cases, and appropriate resources should be available in the event of complications. Prophylactic resternotomy was reported at a minority of centres.

Minimally invasive repair of pectus excavatum in adult patients.

Since 1996, the technique for minimally invasive repair of pectus excavatum (MIRPE) has gained increasing acceptance among pediatric patients. However, the feasibility of the operation and outcomes have not yet been evaluated in adult patients. This study was a retrospective analysis of the author's experience combined with a survey of members of the American Pediatric Surgical Association in treating adult patients with MIRPE. Thirty adults (age range, 18-32 years; mean, 23 years; 75% men) with severe pectus excavatum (chest index > 3.2) were treated with MIRPE. The main indication for surgery was cosmetic (80%). One 32-year-old female patient underwent simultaneous MIRPE and breast augmentation. In 60 per cent of cases, the operative time was 1 to 2 hours. Thoracic epidural was successfully used for postoperative pain management in 90 per cent of patients. Two pectus bars were necessary in 16 per cent of cases, and bilateral stabilizers were used in 53 per cent of patients. Complications included seroma (10%), bar displacement (6%), pneumothorax requiring tube thoracostomy (6%), superficial wound infection (3%), and stabilizer bar fracture (3%). Two patients required conversion to modified Ravitch repair. Patient satisfaction was rated as excellent (50%), good (36%), and fair (14%). Less than 50 per cent of patients achieved 100 per cent correction of their deformity. MIRPE can be used safely for repair of pectus excavatum in adult patients. The complication rate appears to be similar to previously reported series of pediatric patients. Although adult patients may have residual asymmetry of the chest postrepair, overall satisfaction with the repair was very good or excellent in 86 per cent of patients.

Minimally invasive repair of pectus excavatum.

Pectus excavatum, an acquired or congenital depression of the anterior chest wall, is the most commonly occurring chest wall deformity. Patients with pectus excavatum experience psychosocial and physiologic consequences such as impaired social development and pulmonary and/or cardiac dysfunction as a result of the deformity. Traditionally, repair of the defect was performed with a major open operation, the most common being based on modifications of the Ravitch procedure. In the late 1990's, the operative approach was challenged with a new minimally invasive technique described by Dr. Donald Nuss. This approach utilizes thoracoscopic visualization with small incisions and placement of a temporary metal bar positioned behind the sternum for support it while the costal cartilages remodel. Since introduction, the minimally invasive repair of pectus excavatum (MIRPE) has become accepted in many centers as the procedure of choice for repair of pectus excavatum. In experienced hands, the procedure has excellent outcomes, shorter procedural length, and outstanding cosmetic results. However, proper patient selection and attention to technical details are essential to achieve optimal outcomes and prevent significant complications. In the following, we describe our perspective on pectus excavatum deformities, operative planning, and technical details of the MIRPE procedure.