Recent Modifications of the Nuss Procedure: The Pursuit of Safety During the Minimally Invasive Repair of Pectus Excavatum.

OBJECTIVE: To review standardized Nuss correction of pectus excavatum and vacuum bell treatment over the last 10 years. SUMMARY OF BACKGROUND DATA: In 2010, we reported 21 years of the Nuss procedure in 1215 patients. METHODS: Over the last 10 years, 2008-2018, we evaluated 1885 pectus excavatum patients. Surgery was indicated for well-defined objective criteria. A consistent operation was performed by 8 surgeons in 1034 patients, median 15 years, (range 6-46); 996 were primary, and 38 redo operations. Surgical patients' mean computed tomography index was 5.46. Mitral valve prolapse was present in 5.4%, Marfan syndrome in 1.1% and scoliosis in 29%. Vacuum bell treatment was introduced for 218 patients who did not meet surgical criteria or were averse to surgery. RESULTS: At primary operation, 1 bar was placed in 49.8%; 2 bars, 49.4%; and 3 bars, 0.7%. There were no deaths. Cardiac perforation occurred in 1 patient who had undergone previous cardiac surgery. Paraplegia after epidural catheter occurred once. Reoperation for bar displacement occurred in 1.8%, hemothorax in 0.3%, and wound infection in 2.9%; 1.4% required surgical drainage. Allergy to stainless steel was identified in 13.7%. A good anatomic outcome was always achieved at bar removal. Recurrence requiring reoperation occurred in 3 primary surgical patients. Two patients developed carinate overcorrection requiring reoperation. Vacuum bell treatment produced better results in younger and less severe cases. CONCLUSIONS: A standardized Nuss procedure was performed by multiple surgeons in 1034 patients with good overall safety and results in primary repairs. Vacuum bell treatment is useful.

Combined en bloc liver-double kidney transplantation in an infant with IVC thrombosis.

We report a case of a pediatric en bloc liver-double kidney transplant in a patient with IVC thrombosis below the renal veins. The patient is an 11-month-old girl diagnosed with congenital nephrotic syndrome at two months of age. Multifocal liver masses were identified on routine ultrasound at eight months of age. Alpha fetoprotein level was 55 319. Biopsy confirmed hepatoblastoma. CT scan confirmed multiple lesions in both lobes, which would require liver transplantation for resection. She was also found to have thrombosis of her infrarenal IVC secondary to multiple central lines. She was listed for combined liver-kidney transplant and began chemotherapy. After four cycles of chemotherapy, she underwent bilateral nephrectomies followed by a combined en bloc liver-double kidney transplant from a size matched donor. In order to provide adequate venous outflow from the kidneys in the absence of a recipient infrarenal IVC, the donor liver and kidneys were procured en bloc with a common arterial inflow via the infrarenal aorta and common outflow via the suprahepatic IVC. Kidney transplantation in the absence of adequate recipient venous drainage may require unusual vascular reconstruction techniques. This case demonstrates a novel approach in patients who may require combined liver-kidney transplantation.

Selective use of sternal elevation before substernal dissection in more than 2000 Nuss repairs at a single institution.

BACKGROUND/PURPOSE: Evaluate the safety of sternal elevation (SE) used selectively before creating the substernal tunnel during the Nuss procedure. METHODS: An IRB-approved (01-05-EX-0175-HOSP), single institution, retrospective review was performed (1/1/1997-11/20/2017). Primary and secondary Nuss repairs (i.e., previous Nuss, Ravitch, thoracotomy, or sternotomy) are included. SE use, cardiac injuries, and pectus bar infections are reported. Chi square and Fisher's exact test (FE) were used (critical p < .05). RESULTS: 2037 patients [(80% male; mean age 15.2 years (SD = 4.4, range 3-46); mean Haller index (HI) 5.3 (SD = 5.7, range 1.73-201)] underwent Nuss repair. SE was used before creating the substernal tunnel in 171 (8.4%): 160 (8.2%) of 1949 primary and 11 (12.5%) of 88 secondary repairs. SE use increased significantly [χ2(2) = 118.93; p < .001] over time and with increasing HI [χ2(3) = 59.9; p < .001]. No cardiac injuries occurred in primary repairs but two occurred in patients with previous sternotomy. Infection rates were not different with (2.9%) or without SE (1.8%) [χ2(1) =1.14; p = .285] and not higher with off-label VB (1.5%) versus other SE techniques (3.8%) [FE, p = .65)]. CONCLUSION: Selective use of sternal elevation before substernal dissection during the Nuss procedure is safe but may not prevent cardiac injuries in patients with previous sternotomy. Infection rates were not increased with SE. TYPE OF STUDY: Retrospective review. LEVEL OF EVIDENCE: IV.

Development and validation of the Pectus Carinatum Body Image Quality of Life (PeCBI-QOL) questionnaire.

INTRODUCTION: While body image disturbances and quality of life in persons with pectus excavatum (PE) have been well documented, very little has been done to systematically measure and document the same in patients with pectus carinatum (PC). Because of this, the current study aimed to develop and validate an instrument to assess body image related quality of life in patients with PC and their parents. METHOD: Participants: Two waves of data collection took place. The development phase enrolled 78 PC patients and 76 matched parents. The validation phase enrolled 50 PC patients and 50 parents. Mean age at the initiation of treatment was 15.14 (SD = 2.54). Participants were mostly boys (85.9%) and White or Caucasian (89.7%). Instrument development, refinement, and validation: A group of 5 experts in chest wall deformities used existing measures of body image disturbances in PE, combined with the broader body image literature, to develop larger item pools for patients and their parents. Item analysis from this phase was used to remove poorly performing or statistically redundant items. In the validation phase, refined patient and parent instruments were examined using exploratory principal components factor analysis (EFA) with parallel analysis for factor retention, followed by Varimax rotation to identify a final factor solution. RESULTS/DISCUSSION: This development and refinement process yielded a final questionnaire for patients (18 items) and parents (15 items). The patient questionnaire includes four subscales, each with good internal consistency: Body Image Disturbance; Treatment Motivation/Engagement; Physical Limitations; and Social Disadvantage. The parent questionnaire includes 3 subscales: Body Image Disturbance; Treatment Motivation/Engagement; Physical Limitations. Patient and parent scales showed moderate correlations. Among patients with measures pre- and posttreatment, there was a significant improvement in overall PeCBI-QOL score. We demonstrate, in this study, that body image and related quality of life can be reliably and validly assessed with the PeCBI-QOL, which has implications for more comprehensively documenting the negative psychological and functional consequences of pectus carinatum. TYPE OF STUDY/LEVEL OF EVIDENCE: Study of diagnostic test/III.

Complications associated with the minimally invasive repair of pectus excavatum.

The minimally invasive pectus excavatum repair (Nuss repair) is performed by pediatric general surgeons and pediatric and adult thoracic surgeons around the world. Complications related to pediatric surgical procedures are always a major concern for surgeons and their patients, and as with all surgery, especially pectus surgery, complications can be life-threatening. The purpose of this article is to discuss early and late complications of pectus excavatum surgery and potential preventive strategies to minimize them.

Selective versus routine patch metal allergy testing to select bar material for the Nuss procedure in 932 patients over 10years.

AIM OF THE STUDY: The aim of the study was to determine the role of patch metal allergy testing to select bar material for the Nuss procedure. METHODS: An IRB-approved (11-04-WC-0098) single institution retrospective, cohort study comparing selective versus routine patch metal allergy testing to select stainless steel or titanium bars for Nuss repair was performed. In Cohort A (9/2004-1/2011), selective patch testing was performed based on clinical risk factors. In Cohort B (2/2011-9/2014), all patients were patch tested. The cohorts were compared for incidence of bar allergy and resultant premature bar loss. Risk factors for stainless steel allergy or positive patch test were evaluated. MAIN RESULTS: Cohort A had 628 patients with 63 (10.0%) selected for patch testing, while all 304 patients in Cohort B were tested. Over 10years, 15 (1.8%) of the 842 stainless steel Nuss repairs resulted in a bar allergy, and 5 had a negative preoperative patch test. The incidence of stainless steel bar allergy (1.8% vs 1.7%, p=0.57) and resultant bar loss (0.5% vs 1.3%, p=0.23) was not statistically different between cohorts. An allergic reaction to a stainless steel bar or a positive patch test was more common in females (OR=2.3, p<0.001) and patients with a personal (OR=24.8, p<0.001) or family history (OR=3.1, p<0.001) of metal sensitivity. CONCLUSION: Stainless steel bar allergies occur at a low incidence with either routine or selective patch metal allergy testing. If selective testing is performed, it is advisable in females and patients with a personal or family history of metal sensitivity. A negative preoperative patch metal allergy test does not preclude the possibility of a postoperative stainless steel bar allergy. LEVEL OF EVIDENCE: Level III Treatment Study and Study of Diagnostic Test.

Nonoperative management of pectus excavatum with vacuum bell therapy: A single center study.

PURPOSE: The purpose of this study was to determine variables predictive of an excellent correction using vacuum bell therapy for nonoperative treatment of pectus excavatum. METHODS: A single institution, retrospective evaluation (IRB 15-01-WC-0024) of variables associated with an excellent outcome in pectus excavatum patients treated with vacuum bell therapy was performed. An excellent correction was defined as a chest wall depth equal to the mean depth of a reference group of 30 male children without pectus excavatum. RESULTS: Over 4years (11/2012-11/2016) there were 180 patients enrolled with 115 available for analysis in the treatment group. The reference group had a mean chest wall depth of 0.51cm. An excellent correction (depth≤0.51cm) was achieved in 23 (20%) patients. Patient characteristics predictive of an excellent outcome included initial age≤11years (OR=3.3,p=.013), initial chest wall depth≤1.5cm (OR=4.6,p=.003), and chest wall flexibility (OR=14.8,p<.001). Patients that used the vacuum bell over 12 consecutive months were more likely to achieve an excellent correction (OR=3.1,p=.030). Follow-up was 4months to 4years (median 12months). CONCLUSION: Nonoperative management of pectus excavatum with vacuum bell therapy results in an excellent correction in a small percentage of patients. Variables predictive of an excellent outcome include age≤11years, chest wall depth≤1.5cm, chest wall flexibility, and vacuum bell use over 12 consecutive months. TYPE OF STUDY: Retrospective chart review. LEVEL OF EVIDENCE: Level III treatment study.

Multivariate analysis of risk factors for Nuss bar infections: A single center study.

BACKGROUND/PURPOSE: Our previously published data suggested several risk factors for infection after the Nuss procedure. We aimed to further elucidate these findings. METHODS: An IRB-approved (14-03-WC-0034), single institution, retrospective review was performed to evaluate the incidence of postoperative Nuss bar infections associated with seven variables. These were subjected to bivariate and multivariable analyses. A broad definition of infection was used including cellulitis, superficial infection with drainage, or deep infection occurring at any time postoperatively. RESULTS: Over 7years (4/1/2009-7/31/2016), 25 (3.2%) of 781 patients developed a postoperative infection after primary Nuss repair. Multivariable analyses demonstrated an increased risk of infection with perioperative clindamycin versus cefazolin for all infections (AOR 3.72, p=.017), and specifically deep infections (AOR 5.72, p=.004). The risk of a superficial infection was increased when antibiotic infusion completed >60min prior to incision (AOR 10.4, p=.044) and with the use of peri-incisional subcutaneous catheters (OR 8.98, p=.008). CONCLUSION: Following primary Nuss repair, the rate of deep bar infection increased with the use of perioperative clindamycin rather than cefazolin. The rate of superficial infection increased when perioperative antibiotic infusion was completed more than 60min prior to incision and with the use of peri-incisional subcutaneous catheters. Further studies are needed to better understand these findings. TYPE OF STUDY: Retrospective chart review. LEVEL OF EVIDENCE: Level III treatment study.

Laparoscopic vertical sleeve gastrectomy for adolescents with morbid obesity.

Obesity is a worldwide epidemic, and surgical weight loss operations have been performed for over 20 years. Laparoscopic sleeve gastrectomy was first performed as part of the biliopancreatic diversion over 30 years ago. Recently, laparoscopic sleeve gastrectomy has led to excellent excess weight loss with limited morbidity, especially compared to Roux-en-Y gastric bypass. In adolescents, laparoscopic sleeve gastrectomy is an excellent option to provide excess weight loss and remission of comorbid conditions without long-term malabsorptive risks.

Hypertrophic osteoarthropathy in intestinal transplant recipients.

We report 3 patients with periosteal new bone formation consistent with hypertrophic osteoarthropathy (HOA), in the context of intestinal allograft rejection. Patient 1 developed thick periosteal new bone formation of the right arm during a prolonged episode of intestinal acute cellular rejection (ACR) 2 months posttransplant. Patient 2 developed ankle pain and swelling during an episode of severe ACR. Plain films showed periosteal new bone formation of the left ankle. In patient 3, the right wrist became swollen during an episode of moderate ACR, whereas plain films demonstrated mild periosteal reaction. Patients 2 and 3 had resolution of their symptoms once the ACR resolved with treatment. This is the first case series of HOA occurring in association with intestinal ACR. We speculate that an immune-mediated process is responsible for the bone disease. Further inquiry will help establish if HOA is related to transplant status, intestinal inflammation, or allograft rejection in general.

Autologous intestinal reconstruction surgery.

Multidisciplinary management of intestinal failure has progressed over the last 30 years, facilitating the transition to enteral autonomy in many pediatric patients. However, there remains a select group of patients who reach a plateau in advancement of their enteral nutrition. Numerous surgical options have been pursued to attempt to slow intestinal transit, taper dilated bowel, and promote intestinal adaptation. The purpose of this chapter is to review the current literature on autologous intestinal reconstruction surgery, including a brief historical perspective, descriptions of procedures, and reported surgical outcomes.