Richard E. Redlinger

Twenty-One Years of Experience With Minimally Invasive Repair of Pectus Excavatum by the Nuss Procedure in 1215 Patients

Objective: To review the technical improvements and changes in management that have occurred over 21 years, which have made the minimally invasive repair of pectus excavatum safer and more successful. Summary Background Data: In 1997, we reported our 10-year experience with a new minimally invasive technique for surgical correction of pectus excavatum in 42 children. Since then, we have treated an additional 1173 patients, and in this report, we summarize the technical modifications which have made the repair safer and more successful. Methods: From January 1987 to December 2008, we evaluated 2378 pectus excavatum patients. We established criteria for surgical intervention, and patients with a clinically and objectively severe deformity were offered surgical correction. The objective criteria used for surgical correction included computed tomography (CT) scans of the chest, resting pulmonary function studies (spirometry and/or plethysmography), and a cardiology evaluation which included echocardiogram and electrocardiogram. Surgery was indicated if the patients were symptomatic, had a severe pectus excavatum on a clinical basis and fulfilled two or more of the following: CT index greater than 3.25, evidence of cardiac or pulmonary compression on CT or echocardiogram, mitral valve prolapse, arrhythmia, or restrictive lung disease. Data regarding evaluation, treatment, and follow up have been prospectively recorded since 1994. Surgical repair was performed in 1215 (51%) of 2378 patients evaluated. Of these, 1123 were primary repairs, and 92 were redo operations. Bars have been removed from 854 patients; 790 after primary repair operations, and 64 after redo operations. Results: The mean Haller CT index was 5.15 ± 2.32 (mean ±SD). Pulmonary function studies performed in 739 patients showed that FVC, FEV1, and FEF25–75 values were decreased by a mean of 15% below predicted value. Mitral valve prolapse was present in 18% (216) of 1215 patients and arrhythmias in 16% (194). Of patients who underwent surgery, 2.8% (35 patients) had genetically confirmed Marfan syndrome and an additional 17.8% (232 patients) had physical features suggestive of Marfan syndrome. Scoliosis was noted in 28% (340). At primary operation, 1 bar was placed in 69% (775 patients), 2 bars in 30% (338), and 3 bars in 0.4% (4). Complications decreased markedly over 21 years. In primary operation patients, the bar displacement rate requiring surgical repositioning decreased from 12% in the first decade to 1% in the second decade. Allergy to nickel was identified in 2.8% (35 patients) of whom 22 identified preoperatively received a titanium bar, 10 patients were treated successfully with prednisone and 3 required bar removal: 2 were switched to a titanium bar, and 1 required no further treatment. Wound infection occurred in 1.4% (17 patients), of whom 4 required surgical drainage (0.4% of the total). Hemothorax occurred in 0.6% (8 patients); 4 during the postoperative period and four occurred late. Postoperative pulmonary function testing has shown significant improvement. A good or excellent anatomic surgical outcome was achieved in 95.8% of patients at the time of bar removal. A fair result occurred in 1.4%, poor in 0.8%, and recurrence of sufficient severity to require reoperation occurred in 11 primary surgical patients (1.4%). Five patients (0.6%) had their bars removed elsewhere. In the 752 patients, more than 1 year post bar removal, the mean time from initial operation to last follow up was 1341 ± 28 days (SEM), and time from bar removal to last follow-up is 854 ± 51 days. Age at operation has shifted from a median age of 6 years (range 1–15) in the original report to 14 years (range 1–31). The minimally invasive procedure has been successfully performed in 253 adult patients aged 18 to 31 years of age. Conclusions: The minimally invasive repair of pectus excavatum has been performed safely and effectively in 1215 patients with a 95.8% good to excellent anatomic result in the primary repairs at our institution.

One hundred patients with recurrent pectus excavatum repaired via the minimally invasive Nuss technique—effective in most regardless of initial operative approach

PURPOSE Controversy exists as to the best operative approach to use in patients with failed pectus excavatum (PE) repair. We examined our institutional experience with redo minimally invasive PE repair along with the unique issues related to each technique. METHODS We conducted an institutional review board-approved review of a prospectively gathered database of all patients who underwent minimally invasive repair of PE. RESULTS From June 1987 to January 2010, 100 patients underwent minimally invasive repair for recurrent PE. Previous repairs included 42 Ravitch (RAV) procedures, 51 Nuss (NUS) procedures, 3 Leonard procedures, and 4 with previous NUS and RAV repairs. The median Haller index at reoperation was 4.99 (range, 2.4-20). Fifty-five percent of RAV patients and 25% of NUS patients required 2 or more bars (P = .01). Two RAV patients had intraoperative nonfatal cardiac arrest owing to thoracic chondrodystrophy--1 at insertion and 1 upon removal. Bar displacements occurred in 12% RAV and 7.8% NUS patients (P = .05). Overall reoperation for bar displacement is 9%. CONCLUSIONS The minimally invasive NUS technique is safe and effective for the correction of recurrent PE. Patients with prior NUS repair can have extensive pleural adhesions necessitating decortication during secondary repair. Patients with a previous RAV repair may have acquired thoracic chondrodystrophy that may require a greater number of pectus bars to be placed at secondary repair and greater risk for complications. We have a greater than 95% success rate regardless of initial repair technique.

Minimally invasive repair of pectus excavatum in patients with Marfan syndrome and marfanoid features

PURPOSE The presence of a pectus excavatum (PE) requiring surgical repair is a major skeletal feature of Marfan syndrome. Marfanoid patients have phenotypic findings but do not meet all diagnostic criteria. We sought to examine the clinical and management differences between Marfan syndrome patients and those who are marfanoid compared with all other patients undergoing minimally invasive PE repair. METHODS A retrospective institutional review board-approved review was conducted of a prospectively gathered database of all patients who underwent minimally invasive repair of PE. Patients were grouped according to diagnosis of Marfan syndrome (MAR), Marfanoid appearance (OID), and all others (ALL). Patient demographics, preoperative imaging and testing, operative strategy, complications, and postoperative surveys were evaluated. Fishers Exact test and chi(2) were applied for statistical analysis. RESULTS From June 1987 to September 2008, 1192 patients underwent minimally invasive PE repair (MAR = 33, OID = 212, ALL = 947). There was a significantly higher proportion of females with either MAR or OID who underwent repair (21.5%vs 15.5%, P = .04). The MAR patients had significantly more severe PE determined by computed tomography index (MAR = 8.75, OID = 5.82, ALL = 4.94, P < .0001) and required multiple pectus bars (> or =2) to be placed during operation (MAR = 58%, OID = 36%, ALL = 29%, P = .001). There was a trend toward higher wound infection rates in MAR patients (MAR = 6%, OID = 1.4%, ALL = 1.3%, P = .07). The recurrence rate was similar among all groups (MAR = 0%, OID = 2%, ALL = 0.7%, P = .12). Successful outcome from surgeon perspective in either MAR or OID patients was similar to ALL (98%vs 98%, P = .88) and correlated well with patient satisfaction after repair (96%vs 95%, P = .43). CONCLUSIONS Minimally invasive PE repair is safe in patients with Marfan syndrome or marfanoid features with equally good results. Patients with Marfan syndrome have clinically more severe PE requiring multiple bars for chest repair and may have slightly higher wound infection rates. Patients are satisfied with minimally invasive repair despite a phenotypically more severe chest wall defect.

Regional chest wall motion dysfunction in patients with pectus excavatum demonstrated via optoelectronic plethysmography

BACKGROUND Paradoxical chest wall motion is recognized clinically in pectus excavatum (PE). We report chest wall volume and motion differences between PE patients and unaffected individuals. METHODS A prospective, institutional review board-approved study compared nonoperated PE patients with normal controls (C). Subjects had deep breathing maneuvers captured by infrared cameras. Chest wall volume and excursion were calculated using optoelectronic plethysmography marker reconstruction combined with proprietary software (BTS Bioengineering, Milan, Italy). RESULTS One hundred nineteen patients underwent optoelectronic plethysmography analysis (PE: 64, C: 5). Total chest wall volume at rest was similar in both groups (PE: 13.6 L, C: 14.1 L, P = .55). During maximal inspiration, PE patients had a significant increase in the volume within the abdominal rib cage compartment (PE: 0.77 L, C: 0.6 L, P < .01). Patients with PE had 51% less midline marker excursion at the angle of Louis (P < .01), a 46% decrease at the level of the nipples (P < .01), and 28% less excursion at the xiphoid process (P = .02). At the level of the umbilicus, PE patients had 147% increase in midline marker excursion compared with controls (P < .01). CONCLUSIONS Optoelectronic plethysmography kinematic analysis allows for quantification of focal chest wall motion dysfunction. Patients with PE demonstrate significantly decreased chest wall motion at the area of the pectus defect and increased abdominal contributions to respiration compared with controls. This finding may help to explain exertional symptoms of easy fatigability or shortness of breath in PE.

Optoelectronic plethysmography demonstrates abrogation of regional chest wall motion dysfunction in patients with pectus excavatum after Nuss repair

PURPOSE We previously demonstrated that patients with pectus excavatum (PE) have significantly decreased chest wall motion at the pectus defect compared with the rest of the chest vs unaffected individuals and use abdominal respiratory contributions to compensate for decreased upper chest wall motion. We hypothesize that PE repair will reverse chest wall motion dysfunction. METHODS A prospective, institutional review board-approved study compared patients with PE before and after Nuss repair. Informed consent was obtained before motion analysis. Sixty-four patients with uncorrected PE ages 10 to 21 years underwent optoelectronic plethysmography analysis. Repeat analysis was performed in 42 patients 6 months postoperative (PO). RESULTS Volume of the chest wall and its subdivisions were calculated. Total chest wall volume at rest was significantly increased after repair and in each thoracic compartment. PO patients developed increased midline marker excursion at the pectus defect and significantly decreased excursion at the level of the umbilicus. CONCLUSIONS Optoelectronic plethysmography kinematic analysis demonstrates that chest wall remodeling during Nuss repair results in increased thoracic volume. Chest wall motion dysfunction at the pectus defect is reversed after Nuss repair. Abdominal respiratory contributions are also markedly decreased. These findings may help to explain why patients with PE report an improvement in endurance after the Nuss procedure.

Aortic remodeling after thoracic endovascular aortic repair for intramural hematoma.

BACKGROUND Intramural hematoma (IMH), penetrating atherosclerotic ulcer (PAU), and aortic dissection comprise a spectrum of acute aortic pathologies. Although thoracic endovascular aortic repair (TEVAR) has increasingly been applied to aortic dissection, there is a paucity of data on the anatomic effect of TEVAR for IMH. Our goal was to investigate the extent of aortic remodeling after TEVAR. METHODS A retrospective chart review from 2006 to 2012 was conducted on patients who underwent TEVAR for IMH. Data were collected from the electronic medical record. Radiology images were reviewed and primary data points included diameter (TLD) and volume measurements for aortic true lumen and total aortic diameter (TAD) and volume at the site of maximal pathology. Aortic remodeling was evidenced by a TAD/TLD ratio closest to 1.0. Patients with no imaging beyond 30 days postoperatively were excluded. RESULTS During the 6-year period, 44 patients underwent TEVAR for IMH. Twenty-five patients had an IMH with concomitant PAU. There were 25 (57%) female patients. Mean age was 71 ± 11 years, and 40 (91%) patients had hypertension. Operative indications included intractable pain in 31 (70%), rapidly progressing IMH or conversion to dissection in 13 (30%), rupture in 10 (23%), and uncontrolled hypertension in 6 (14%). Technically successful TEVAR was performed in all patients with 42 (95%) reporting complete relief of symptoms. The 30-day mortality rate was 5% with a 5% rate of permanent paraplegia or paraparesis. At a mean follow-up of 26 months, there were no additional aortic-related deaths and overall survival was 80% with a reintervention rate of 11%. For our imaging analysis, 10 patients were excluded because of lack of follow-up imaging beyond 30 days. At a mean follow-up of 13 months, all measured data points were statistically improved from before to after TEVAR: thickness of IMH (12 mm vs. 4 mm; P = .01), mean TLD (35 mm vs. 37 mm; P = .04), mean TAD (47 mm vs 42 mm; P = .02), TAD/TLD ratio (1.35 vs. 1.14; P < .01), and IMH volume (103 cm3 vs. 14 cm3; P < .01). The mean Δ in TAD/TLD ratio from before to after TEVAR for the reintervention group was Δ0.14, and the mean Δ in TAD/TLD ratio for the nonreintervention group was Δ0.29 (P = .05). Analysis of patients with isolated IMH and those with concomitant PAU revealed no statistical differences. CONCLUSIONS TEVAR is safe and effective in treating IMH and based on longitudinal computed tomography scan analysis, aortic remodeling is evidenced by normalization of all measured indices.

Dismorfología de las deformidades de la pared torácica: distribución de frecuencias de los subtipos de pectus excavatum típico y subtipos poco comunes

BACKGROUND/PURPOSE More than forty percent of patients with pectus excavatum have a family history of chest deformity. However, no studies of the frequency of the different phenotypes of pectus excavatum have been published. METHODS A random sample of 300 non-syndromic pectus excavatum patients, from the chest wall deformities clinic at Childrens Hospital of The Kings Daughters in Norfolk, Va., was studied and classified according to a previously described classification system. Photographs and computed tomography (CT) scans were utilized. RESULTS Typical pectus excavatum. Photo data: localized deep depression (cup-shaped) deformity occurred in 67%; diffuse (saucer-shaped) 21%, trench-like (furrow-shaped) 10%, and Currarino-Silverman (mixed pectus excavatum/chondromanubrial carinatum) 1%. The deepest point was to the right of midline in 80%, left in 10% and central in 10%. By photo, the deepest point was in the lower sternum in 75%. When asymmetric, the deepest point of the deformity was to the right of midline in 90%. CT data: the average Haller index was 4.9. Severe sternal torsion (>30 degrees) was associated with greater Haller index (6.3) than mild torsion (4.5). The deepest point of the depression was at the mid- or lower sternum in more than 99%. It proved impossible to estimate width or length of the depression because of poorly defined borders. CONCLUSIONS Typical PE is cup-shaped in 67% of cases, to the right of the midline in 80%, and involving the mid-to-lower sternum in 99%. However, other phenotypes, like the saucer and long trench, comprised one-third. Definition of the deformity is more reliable by CT scan.