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Severe pectus excavatum associated with cor pulmonale and chronic respiratory acidosis in a young woman.

Pectus excavatum has never been reported to cause hypercapnic respiratory failure. In this report, we describe the first such case in a young woman with severe pectus excavatum who presented with chronic respiratory acidosis, pulmonary hypertension, and chronic cor pulmonale. An extensive diagnostic workup failed to uncover any other cause of respiratory acidosis, which led us to conclude that the severe chest wall deformity and the resulting severe restrictive defect were responsible for the development of chronic respiratory acidosis and cor pulmonale.

Acidosis, Respiratory↗

[Evaluation of the degree of infundibuliform abnormality of the thorax and technics of the fixation of the sternum].

The author proposes an original method of determination of the degree of deformation in angular values on the basis of his experience in the treatment of 27 patients with infundibulum-like deformation of the chest. In case of surgical treatment the method of thoracoplasty after N. I. Kondrashin is preferable. The design formula of the size of the resected rib area is proposed. To stabilize the framework of the chest fixation by crossing wires on the ring support after G. A. Ilizarov is suggested. Submerged crossing wires with bent supporting ends were used for more continuous fixation.

Adolescent↗

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.

Adolescent↗

Repair of pectus excavatum deformities in children: a new perspective of treatment using minimal access surgical technique.

HYPOTHESIS: Minimally invasive correction of pectus excavatum (PE) deformities of the anterior chest wall in children is safe and effective. DESIGN: Prospective cohort study. SETTING: Tertiary pediatric referral center. PATIENTS: Between February 1996 and July 2000, 36 patients underwent minimally invasive repair (MIR) of PE deformities, and 6 patients had traditional Ravitch repairs (RR). MAIN OUTCOME MEASURES: Morbidity, operating time, estimated blood loss, days to tolerating a regular diet, and length of hospital stay. RESULTS: Thirty-six children underwent MIR for moderate to severe chest wall deformities, with a mean operative time of 1.6 hours, a mean blood loss of 22 mL, a mean time to tolerating a regular diet of 2.9 days, no intensive care unit admissions, and a mean length of hospital stay of 5.5 days. Six children had RR procedures performed for moderate to severe deformity, with a mean operative time of 5.2 hours, a mean blood loss of 222 mL, a mean time to tolerating a regular diet of 3.3 days, 2 patients admitted to the intensive care unit, and a mean length of hospital stay of 4.5 days. Complications for both procedures consisted mainly of postoperative pneumothorax. CONCLUSIONS: The MIR technique for PE is less invasive, less morbid, and better tolerated than traditional open RR of this common pediatric chest wall deformity. Elective surgical reconstruction can be safely performed in children rather than limiting repair to only symptomatic patients with severe deformities.

Adolescent↗

Chest wall constriction after too extensive and too early operations for pectus excavatum.

BACKGROUND AND METHODS: Since 1990 we have evaluated 12 children and teenagers in whom severe cardiorespiratory symptoms have developed due to failure of chest wall growth after very extensive pectus excavatum operations (removal of five or more ribs) at very early ages (< 4 years). RESULTS: Apparently these extensive procedures have removed or prevented growth center activity, which resulted in restriction of chest wall growth with marked limitation of ventilatory function. The forced vital capacity ranged from 30% to 50% of predicted and the forced expiratory volume in 1 second from 30% to 60%. All patients are symptomatic with mild exercise and cannot compete in running games. Our protocol for critical evaluation includes exercise pulmonary function studies and axial computed tomographic reconstruction. CONCLUSIONS: This report is an alert to recognize such patients and also to recommend delay in operative repair in small children until at least 6 to 8 years of age. The younger the patient the more limited the chest wall resection for pectus excavatum should be. Five of these patients have had a chest cavity expansion operation with encouraging early results.

Adolescent↗

Repair of pectus excavatum by sternal eversion.

Pectus excavatum was repaired by the sternal eversion (turnover) technique in 26 patients over a 7-year period. Vascular supply to the sternal graft was maintained by preservation of one internal mammary vascular pedicle. Good results were obtained in 21 (81%) patients followed for periods ranging from 2 to 76 months (mean, 32 months) postoperatively. Four patients (15%) had fair results; 2 patients with Marfan's syndrome had partial recurrence, as did 1 patient with skin necrosis and 1 with hypertrophic scar. One patient (4%) had a poor early result due to wound infection and distal sternal necrosis requiring reoperation. Other complications were minor: superficial wound seroma in 2 patients and pneumothorax in 1. The sternal eversion technique for repair of pectus excavatum utilizes the concave shape of the sternum when turned over to create a cosmetically acceptable convex anterior chest wall contour. Judicious tailoring of the costal cartilages and shaping of the anterior sternum corrects asymmetrical deformities. The chest wall is very stable after repair. Since no prosthetic struts or pins are used, a second operation for removal is avoided. Preservation of the vascular supply to the sternum should allow normal growth of the anterior chest wall. The results have been sufficiently encouraging for us to recommend sternal eversion as the primary method for repair of pectus excavatum.

Adolescent↗

Management of a floating sternum after repair of pectus excavatum.

PURPOSE: The aim of this study was to examine the authors' experience with patients who have floating sternum after correction of pectus excavatum via the classical Ravitch procedure. A floating sternum is defined as a sternum in which the only attachment to the chest wall is its superior (cranial) border, and in which the body is secured only by the manubrium and whatever lateral and inferior fibrous bands are present. Typically, a floating sternum is caused by either extensive resection of the costal cartilages and perichondrium during correction of pectus excavatum or failure of proper regrowth of these cartilages. METHODS: The authors retrospectively assessed the charts of all patients diagnosed with a floating sternum noting age at original correction of pectus excavatum, time from original correction of pectus excavatum to diagnosis of floating sternum, age at correction of floating sternum, complaints before stabilization of the sternum, methods of repair, and postoperative complications. RESULTS: Between July 1993 and June 1999, floating sternum was diagnosed in 7 patients. The mean age of patients who underwent operative correction of a floating sternum was 28.9 years (range, 16 to 42 years). The mean time interval between original correction of pectus excavatum, or "redo," and diagnosis of a floating sternum was 9.9 years (range, 2 to 20 years). Complaints before correction of the floating sternum included sternal pain and instability, exercise intolerance, and difficulty breathing. Operative repair consisted of mobilizing the lateral and inferior edges of the sternum, detaching the fibrous perichondrium, performing anterior sternal osteotomies, and finally supporting the sternum with substernal Adkins struts. All 7 patients had successful stabilization of the sternum. Two of 7 patients underwent 2 procedures to successfully stabilize the sternum. One patient has Adkins struts still in place because of hematopoetic malignancy. Six of 7 patients are now without symptoms. CONCLUSIONS: A floating sternum is a morbid phenomenon that may manifest many years after the original procedure. It can cause significant sternal pain, chest wall instability, and respiratory dysfunction, which are the hallmark indications for correction. Repair of a floating sternum can be accomplished successfully.

Adolescent↗

Efficacy of left thoracoscopy and blunt mediastinal dissection during the Nuss procedure for pectus excavatum.

BACKGROUND/PURPOSE: The minimally invasive Nuss procedure is emerging as the preferred technique for repair of pectus excavatum. Original methods of pectus bar placement have been modified to improve safety and efficacy and avoid cardiothoracic complications. The currently reported modifications to facilitate retrosternal pectus bar placement include routine use of right thoracoscopy or a subxiphoid incision. The purpose of this article is to describe additional modifications of the Nuss procedure to improve safety and efficacy. METHODS: A retrospective analysis was performed on 51 patients who have had a thoracoscopic-assisted Nuss procedure at The Children's Hospital, Denver, Colo, between 1999 and 2002. Technical modifications included patient positioning, routine use of left thoracoscopy, and an Endo-kittner. RESULTS: Fifty-one patients have successfully undergone the Nuss procedure using the new modifications. Surgical time ranged from 45 to 120 minutes. There have been no intraoperative or postoperative bleeding complications. There have been 2 large pneumothoraces requiring needle thoracenteses in the operating room before extubation. No chest tubes were required postoperatively. Subjectively, all patients have been satisfied with their surgical correction. Average length of hospital stay was 4 to 6 days. CONCLUSIONS: By using left chest thoracoscopy and Endo-kittner dissectors, the risk of cardiothoracic injury can be eliminated. Moreover, other methods to ensure safe substernal dissection are unnecessary.

Adolescent↗

Rasterstereography in the measurement and postoperative follow-up of anterior chest wall deformities.

Rasterstereography is a contact-free photogrammetric method for the measurement of body surfaces. As such it is particularly suited for the objective documentation of anterior chest wall deformities and the results of surgery. An example of its application and first results are presented. The possibilities of a thorough data analysis with regard to diagnosis and therapy control are outlined.

Adolescent↗

Simultaneous repair of cardiovascular disorders and pectus deformity in a patient with Sprintzen-Goldberg syndrome: A case report.

We report a 12-year-old girl with Sprintzen-Goldberg syndrome (SGS) who was complicated with annuloaortic ectasia with aortic regurgitation, mitral valve prolapse with mitral regurgitation, and a severe pectus excavatum. In this patient, aortic root replacement, mitral valve replacement, and sternal elevation were simultaneously performed, and a version of Ravitch's procedure that was technically modified to support the sternum was used for sternal elevation. This modified sternal elevation technique gave excellent operative exposure, and maintained chest wall stability after the operation.

Aortic Aneurysm↗

[Effect of therapy with beta-adrenoblockers and vitamin complexes on indices of oxyproline excretion in various hereditary connective tissue diseases].

Excretion of hydroxyproline with urine was studied in 16 children (5-14 years old) with Marphan-Like syndrome and Marphan, Ehlers-Dunlos and Larson syndromes after therapy involving propranolol and a complex of vitamins (ascorbic acid, riboflavin and pyridoxine) and recommended on the basis of echocardiographic analyses. The therapeutic course appears to cause quantitative and qualitative correction of collagen and apparently of elastin fibrilles development. Depending on initial patterns of hydroxyproline excretion and the syndrome form the correction could be complete or partial, while positive effect of the treatment was stable or provisional. The data obtained suggest that the complex treatment developed might be applied as a preoperative therapy of the patients with Marphan-like syndrome as well as with syndromes of Marphan and Ehlers-Dunlos before thoracoplastics caused by hereditary chest deformation and by impairments of cardiovascular system.

Adolescent↗

[Atypical thoracic deformities: evaluation and operative sequelae].

Until 1982 we have performed 1410 chest wall corrections in our clinic. 0.5 percent were related to cleft sternum or osteocartilagineous defects, 2.3 percent to premature sternal synostosis, 0.5 percent followed the reconstruction of congenital diaphragmatic hernias. Another 17 percent were characterized by extreme flat chest, atypical anterolateral asymmetric or extreme position of the punctum maximum. Morphological parameters are taken by pelvimeter and profile measurements, operative consequences derived from the morphological typing and grading and the clinical finding.

Airway Obstruction↗

[One stage operation for sternal turnover with preserved rectus muscle pedicles and aortic root replacement associated with Marfan's syndrome].

A 40-year-old man with Marfan's syndrome had annulo aortic ectasia with Sellers grade 4 aortic valve regurgitation and Wada grade 3 pectus excavatum. Simultaneous operation was successfully performed by aortic valve composite graft insertion and sternal turnover with the rectus muscle pedicles. Following a midline skin incision, the cost-sterno complex (plastron) was dissected together with the bilateral rectus muscle pedicles, and the sternum was divided transversely through the second intercostal space. The plastron with muscle pedicles was retracted away from the anterior chest toward the abdomen and was covered by the moistened sternal bag made of polyethylene to prevent dryness and contamination during the composite graft insertion. The aortic root was replaced with a composite graft consisting of a 25 mm SJM valve and a 26 mm Hemashield graft. A short interposed 10 mm Hemashield graft was inserted between the ostia of the left coronary artery and the composite graft. The right coronary artery was reimplanted in the aortic conduit using the button technique with a doughnut pledget. This one stage method offered excellent operative exposure and enabled us to prevent possible necrosis of the sternum, infection of the mediastinal sinus, and postoperative cardiac failure resulting from chest wall compression. In this procedure, active usage of the rapid autologous transfusion system effectively reduced the total amount of blood transfusion.

Adult↗

[The effect of the age of the child at the time of surgery for pectus excavatum on respiratory function and anthropometric parameters of the thorax].

Respiratory function and anthropometric parameters of the chest were studied in two groups of children aged 15, undergoing pectus excavatum repair before and after the age of 12, respectively. Mean values of static and dynamic functional respiratory parameters were significantly lower in children operated before the age of 12. Sagital diameters of the chest determined at the level of sternal angle and costal arch intersection were significantly shorter in children operated before the age of 12. Restricted respiratory motility of the chest and shorter sagital diameters are very likely caused by the damage of active ossification centers in the sternum of children. The results support the indications for pectus excavatum repair beyond the age of 12.

Adolescent↗

[Corrective surgery of congenital chest malformations of the type Pectus excavatum and Pectus carinatum (author's transl)].

The authors presents an original process for surgical treatment of deformations of chest anterolateral wall, insisting on the procedures and techniques of the recommended process. The compulsory operative steps are: wide chondrectomy, retrosternal loosening, transversal osteotomy of the sternum and contention with retrosternal metallic plates. The author considers surgery to be necessary for all these anterolateral chest deformations and the best age for their correction to be after 10 to 12 years. The original technique for loosening and preserving the perichondrium in its wholeness is described together with the complete resection of deformed cartilages.

Adolescent↗

Evaluation of pectus excavatum with repeated CT scans.

The use of a single CT scan to evaluate the severity of pectus excavatum has been popular since its inception. However, there is no objective data to address the evolution of the deformity. Using repeated CT scans taken an average of 1 year and 3 months apart, eight children with pectus excavatum were prospectively followed. The initial pectus indices ranged from 3.6 to 6.8 (mean 5.4 +/- 1.3) and the follow-up indices, from 3.5 to 6.7 (mean 5.3 +/- 1.0). No progress of pectus excavatum was found during the study period. Our routine five axial chest CT sections also helped to specify a pectus index based on the central section taken through the deepest part of the deformity, and provided useful information for further conservative or surgical management.

Adolescent↗

Ten year experience of bioabsorbable mesh support in pectus excavatum repair.

INTRODUCTION: We reviewed 10 years experience in the treatment of this deformity using a retro-sternal bioabsorbable mesh in place of a metallic device to lift and stabilise the sternum. Moreover, the mesh supports the thoracic and upper abdominal wall reconstruction. MATERIALS AND METHODS: From January 1990 to December 2000, in our Thoracic Surgery Unit, 65 patients with PE were assessed for surgical repair, mean age 16+/- 3.5 years, fronto sagittal thoracic index (FSTI) 0.21, ranging from 0.15-0.33. Twenty-three of them underwent surgical correction after initial assessment, 22 were deferred and sent to physiotherapy. At a subsequent assessment, five of the patients sent to physiotherapy were deemed to require surgery. RESULTS: Of the 28 patients who underwent surgery, 2 (10%) presented a mild recurrence of PE after 1 year (0.30 0.34), meanwhile all other patients maintained a FSTI>0.34. For all patients the improvement in FSTI was statistically significant, p = 0.001. Patients satisfaction after 24 months was thus shared: excellent 18 patients (65%), good seven patients (25%), fair one patient (3.5%) and poor two patients (7%). No major complications were observed in preoperative period. Patients mobilisation was soon achieved thanks to the postoperative pain control and the absence of retro-sternal metallic support. CONCLUSIONS: The introduction of bioabsorbable mesh in the Robicsek technique is a safe procedure related to a high percentage of success. The high tolerance of the material reduces the inflammatory reaction. Moreover, the procedure prevents patients from having complications caused by retro-sternal device dislodgment, avoiding a second intervention for device reposition and reducing the postoperative chest pain achieving an early patient mobilisation. In the end a complete reconstruction of the upper abdomen wall has been produced.

Absorbable Implants↗

[Relation between infections during the pregnancy and the rate of hydroxyproline excretion in children with congenital chest deformities].

In a group of women, which had children with hereditary isolated and syndromal chest deformations, acute respiratory diseases, tonsillitis or influenza occurred during pregnancy in 42.9 +/- 6.6% and 53.8 +/- 13.8% of cases, respectively, whereas in the control group only 17.8 +/- +/- 7.2% of the women were impaired with identical infectious diseases during pregnancy (P less than 0.02 and P less than 0.05, respectively). Excretion of hydroxyproline was distinctly dissimilar in 22 children with isolated and in 13 children with syndromal chest deformations depending on presence or absence of the above-mentioned infections during pregnancy. Under conditions of these infections the higher level of total hydroxyproline excretion was noted as well as relatively lower content of bound hydroxyproline was detected in those peptides, which appear to be responsible for the content of newly synthesized collagen. The data obtained suggest that acute respiratory diseases, influenza, chronic and acute tonsillitis may impair collagen metabolism in children with hereditary chest deformations as well as that these infectious diseases occurred during pregnancy may increase the risk of the pathology development in the children.

Adolescent↗