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Primary versus secondary mitral valve prolapse: clinical features and implications.

This study considers the implications that two types of mitral valve prolapse exist. One is primary, and consists of a basic abnormality of the mitral apparatus. While the etiology of this lesion may not always be the same, the major gross abnormalities are usually constant. Secondary mitral valve prolapse generally exists merely as a consequence of reduced or abnormal ventricular dimensions, and usually appears to be a benign phenomenon.

Anorexia Nervosa↗

Lung growth and function in children and adolescents with idiopathic pectus excavatum.

Patterns of lung growth and function were studied retrospectively in 103 patients (73 male and 30 female) with idiopathic pectus excavatum in order to determine: 1) the prevalence of restrictive lung defect and/or other lung function abnormalities; 2) the possible association between type of lung function abnormalities and age of the patient; and 3) whether the type of lung function is associated with other clinical conditions. Forty-three patients (42%) were between 5-9 years of age; 36 (35%) were between 10-14 years; and 24 (23%) were between 15-19 years. Restrictive lung disease was detected only in 5 patients (5%), whereas 42 patients (41%) had evidence of obstructive pattern, and the remaining 56 patients (54%) had a normal pattern. The mean values for lung volume (total lung capacity (TLC) and/or forced vital capacity (FVC)) were lowest in the 10-14-year group, whereas the mean values of the indices of lower airway function (forced expiratory volume at 1 sec (FEV(1)), FEV(1)/FVC, forced expiratory flow at 25-75% of forced vital capacity (FEF(25-75)), and FEF(25-75)/FVC) were higher in the younger group. Residual volume (RV) and RV/TLC were elevated in all age groups, but they declined toward normalization with increasing age. There were no significant differences between groups (stratified either by pattern of lung function or by age) with regard to their demographics or clinical characteristics (scoliosis, direction of sternal rotation, history of asthma/reactive airways disease, or exercise intolerance). We conclude that idiopathic pectus excavatum may be associated with a variety of lung function abnormalities (in particular, lower airway obstruction) even in the absence of overt clinical symptoms. There was no evidence of significant worsening of lung function with increasing age.

Adolescent↗

Pectus excavatum defect on liver-spleen scintigraphy.

A 58-year-old man with a pectus excavatum demonstrated a photon-deficient defect within the left lobe of the liver on liver-spleen scintigraphy using Tc-99m SCOL. Computed tomography of the liver clearly revealed the abnormal defect seen on the liver-spleen scan to be due to the pectus excavatum. This case confirms the impression that these skeletal aberrations may cause false-positive hepatic defects.

Funnel Chest↗

Echocardiographic evaluation of the aortic root and mitral valve in children and adolescents with isolated pectus excavatum: comparison with Marfan patients.

Pectus excavatum, mitral valve prolapse (MVP), and dilated aortic root occur frequently in patients with Marfan's syndrome (MS). Patients with isolated pectus excavatum (IPE) have a high prevalence of MVP, but it is not known whether aortic root dilatation is a risk in those patients. To test the hypothesis that IPE and MS represent a spectrum of connective tissue dystrophy with MV and aortic root involvement, two-dimensional (2D) echocardiography was used to measure the aortic root diameter and assess for MVP in IPE (n = 31), MS (n = 14), and normal (n = 16) gender- and age-matched patients. Aortic root was measured in parasternal long- and short-axis views, just above the aortic sinuses, at end systole, in six cardiac cycles, and averaged. Parasternal long-axis view was used to assess for MVP. Aortic root diameter in IPE patients was not different from that in normal subjects, 24 +/- 4 mm vs 22 +/- 4 mm (p = NS), respectively, both were significantly smaller than that in MS patients (30 +/- 5 mm; p less than 0.05). MVP was present in 17 of 31 (55%) IPE patients vs 12 of 14 (86%) MS patients (p = NS) and in only 1 of 16 (6%) normal subjects (p less than 0.05) vs both IPE and MS. We conclude that young patients with IPE represent an isolated form of connective tissue abnormality because of the presence of pectus excavatum and MVP, but this is different from the systemic involvement of MS because of the lack of other systemic findings, including aortic root dilatation and changes in body habitus.

Adolescent↗

Echo planar imaging of an infant with pectus excavatum.

Echo planar imaging has enabled us to image safely and without sedation the thorax of an infant with pectus excavatum deformity. The heart was displaced into the left side of the thorax, and the right lung was calculated to be 1.6 times larger than the left lung.

Funnel Chest↗

An autosomal recessive disorder with retardation of growth, mental deficiency, ptosis, pectus excavatum and camptodactyly.

Two strikingly similar brothers issued from consanguineous parents in the second degree present the following patterns of anomalies: retardation of growth, mental deficiency, ocular abnormalities, pectus excavatum and camptodactyly. The ocular abnormalities include ptosis, microphthalmia and hypertelorism. No endocrine or metabolic aberrations were found. The authors conclude that the disorder has probably an autosomal recessive mode of transmission.

Abnormalities, Multiple↗

Pectus excavatum in children: diagnostic significance for mitral valve prolapse.

Eighty-seven patients with pectus excavatum underwent cardiac examination and echocardiography (M-mode) to determine the diagnostic significance of pectus in children for mitral valve prolapse (MVP). Patients' ages ranged from 1 month to 18 years with a mean age of 5.4 years. Sixty-seven were males. Twenty of the 87 pectus patients (23%) had echocardiographic evidence of MVP, whereas 11 of these had auscultatory findings of a non-ejection click or late systolic murmur, and 4 had significant mitral insufficiency. Fourteen of the 77 patients (18%) with mild pectus, and 6 of the 10 patients (60%) with severe pectus had MVP. Two of the patients (3.4%) with mild pectus and 2 of the patients (20%) with severe pectus also had significant mitral insufficiency. Ten of the 23 patients (44%) older than 8 years of age and 10 of the 64 younger patients (16%) had MVP. Although MVP was present more frequently in females (30%) than in males (21%), the difference was not statistically significant. This study indicates the high incidence of MVP in children of 8 years of age and older, especially when pectus deformity is severe. This information is particularly helpful to heighten suspicion of MVP in children with pectus excavatum.

Adolescent↗

Nuss procedure: pediatric surgical solution for adults with pectus excavatum.

INTRODUCTION: The aim of the study was to compare results of the Nuss procedure in adults and children. METHODS: The data were collected prospectively. Antibiotic prophylaxis was used in all patients. Differences were tested statistically. RESULTS: A total of 35 adults (28 men; male/female ratio 4:1) with a median age of 23 years (range 18 years to 47 years 1 month) were included in the study. A previous Welch operation had been performed in one (3%). The median operating time was 65 minutes (range 45-105 minutes). Two bars were inserted in one patient, and stabilizers were used in all. Pneumothorax occurred in 9 (26%), wound abscess in 3 (9%), and bar slips in 5 (14%). The median hospital stay was 7 days (range 4-10 days). Bars were removed in 14 (40%) patients without complications. Follow-up occurred 6 and 12 months after bar removed, at which times no recurrence of the pectus and no sternal protrusions were seen. In the other part of the study, 141 children and adolescents (105 boys; male/female ratio 3:1) with a median age of 13 years (range 4 years 11 months to 17 years 8 months) were included. A previous Welch operation had been performed in five (4%). Two bars were inserted in four, and stabilizers were used in 100 (71%). The median operating time was 65 minutes (40-185 minutes). Pneumothorax occurred in 33 (24%), wound abscess in 1 (0.7%), and bar slips in 18 (13%). The median hospital stay was 7 days (range 5-18 days). Bars were removed in 77 (55%) without complications. Follow-up occurred 6 and 12 months after bar removed, and the last follow-up was eventually planned at the end of growth (age 18). The pectus recurred in one patient with Marfan syndrome, and sternal protrusion occurred in one. Differences between groups were as follows: a significantly higher proportion of adult patients were treated with stabilizers (P < 0.001), and there were significantly more wound abscesses in adults (P < 0.001). CONCLUSIONS: The Nuss procedure is as effective for correcting pectus excavatum in adults as it is in children. However, in adults the bars tended to be less stable, and wound abscesses occurred more frequently.

Adolescent↗

Pleural macrophages are the dominant cell population in the thoracic cavity with an inflammatory cytokine profile similar to peritoneal macrophages.

Numerous human macrophage (mphi) subpopulations with different behavior have been identified in adults. It is well known that peritoneal mphi are activated by abdominal surgery and subsequently contribute to a systemic inflammatory response that leads to immune suppression, increased morbidity and mortality. Information on the role of pleural mphi in adults is scarce and information on their role in children is lacking. We investigated the behavior of pleural versus peritoneal mphi in children and adolescents. As a first step, we compared the cellular composition of the pleural and peritoneal surface in children and adolescents. Pleural and peritoneal lavages were performed in 21 patients undergoing non-contaminated laparoscopic and thoracoscopic surgical procedures. We observed a significantly higher percentage of mphi in the pleural compared to the peritoneal cavity with less lymphocytes, a small amount of polymorphonuclear cells (PMNs) and other cells. To further study the mphi inflammatory response, we measured the spontaneous and LPS triggered cytokine release of isolated pleural versus peritoneal mphi (IL-1beta, IL-6, and IL-10). The pattern of cytokine release was similar in both, pleural and peritoneal mphi. Directly after lavage, they showed a strong activation, with no difference between stimulated and non-stimulated cells. After 24 h resting, mphi of both compartments reacted to LPS with a similar significant increase in the cytokine release. In conclusion, our results demonstrate that pleural mphi represent the dominant cell population in the pleural cavity of the young. They show a similar inflammatory response as peritoneal mphi and should be considered to play a major role in the local inflammatory response to thoracic surgery.

Adolescent↗

Reduced hospitalization cost for patients with pectus excavatum treated using minimally invasive surgery.

BACKGROUND: Currently, few data exist regarding the relative costs associated with open and minimally invasive pectus excavatum repair. The aim of this study was to compare the surgical and hospitalization costs for these two surgical techniques and to identify factors responsible for cost differences. METHODS: A retrospective review of hospital charts, patient and parent questionnaires, and hospital accounting records was performed for 68 patients who underwent surgical correction of pectus excavatum between June 1996 and December 1999. RESULTS: In this series, 25 patients underwent open repair, whereas 43 patients underwent minimally invasive repair of pectus excavatum (MIRPE). The patient ages ranged from 4 to 19 years. The average ages for open repair (12 years) and MIRPE (11 years) did not differ significantly. As compared with open repair, MIRPE was associated with a 27% lower overall cost of hospitalization ( p < 0.05). The operating room costs were 12% higher for the patients who underwent MIRPE ( p < 0.05). The mean operative time for open repair was 3 h 15 min, whereas MIRPE required 1 h 10 min ( p < 0.001). The hospital stay for open repair averaged 4.4 days, as compared with 2.4 days for MIRPE ( p < 0.001). In contrast to other published series, the postoperative analgesia after MIRPE in this series consisted of narcotics, ketorolac, and methocarbamol. No patient received epidural analgesia, regardless of the repair technique selected. The postoperative complication rate was 4% in the open group and 14% in the MIRPE group. Most of the patients treated with either open or MIRPE reported postoperative oral narcotic usage for 2 weeks or less and returned to routine activities within 3 weeks. The patients and parents alike reported good to excellent overall outcomes in 85% or more of the open repair cases and 90% or more of the MIRPE cases. CONCLUSIONS: These data demonstrate for the first time that the use of an alternate pain management strategy including, narcotics, NSAIDs, and methocarbamol, but without epidural catheters, results in reduced hospital length of stay and decreased overall hospitalization costs for MIRPE, as compared with open pectus repair. This cost benefit was achieved without compromising pain management or patient satisfaction with surgical care.

Adolescent↗

Anesthetic management for the minimally invasive Nuss procedure in 21 patients with pectus excavatum.

The aim of this study was to assess the anesthetic management and postoperative analgesic effect of continuous epidural infusion for the minimally invasive Nuss procedure. A total of 21 operated cases were analyzed retrospectively. Thoracoscopy was used in all cases. General anesthesia with endotracheal intubation was induced and maintained with oxygen, air, sevoflurane, and fentanyl in all cases. Thoracic epidural anesthesia was performed after induction at the level between Th4 and 12. When the bar was placed via insertion under the sternum, six patients exhibited sinus tachycardia and one showed premature atrial contraction for 2-4 beats before recovering spontaneously within 1 min. Operations were uneventful. The mean operating time was 115 min and anesthetic time was 193 min. In X-ray findings, residual pneumothorax and pleural effusion were found in seven (33.3%) and eight (38.0%) patients, respectively. In all cases, these symptoms were resolved spontaneously within 5 days. Epidural fentanyl (0.3 microg.kg(-1).h(-1)) in 0.125% bupivacaine (0.15 ml.kg(-1).h(-1)) or 0.2% ropivacaine (0.15 ml.kg(-1).h(-1)) were used for 3 days to relieve postoperative pain. Postoperatively, 12 (57.1%) patients required no additional analgesics, and 4 (19.0%) patients required a single dose of dicrofenac sodium or pentazocine. Although the Nuss procedure is minimally invasive, we should pay attention to the possibility of many intra- and postoperative complications. Continuous epidural infusion of fentanyl with local anesthetics provides effective postoperative pain relief and prevents complications such as bar displacement after the Nuss procedure.

Adolescent↗

Recent experiences with minimally invasive pectus excavatum repair "Nuss procedure".

OBJECTIVE: To review the new technical modifications and results of 668 patients who have had pectus excavatum repair utilizing the minimally invasive technique. METHODS: A retrospective chart review was conducted of 668 patients undergoing minimally invasive pectus repair from 1987 through July 2004. Since 1997, a standardized treatment pathway was implemented. Preoperative evaluation included computed tomography (CT) scan, pulmonary function tests, and cardiac evaluations with electrocardiogram and echocardiogram. Indications for operation included at least 2 of the following: progression of the deformity, Haller CT index greater than 3.25, mitral valve prolapse, cardiac compression or displacement, pulmonary function studies that indicate restrictive or obstructive airway disease, previous failed open or minimally invasive pectus repair. Technical and design modifications since 1998 have included routine thoracoscopy, the use of an introducer/dissector for creating the substernal tunnel, elevating the sternum, and routine use of a wired lateral stabilizer and polydioxanone suture (PDS) sutures around the bar and underlying rib to prevent bar displacement. The bar is removed as an outpatient procedure in 2 to 4 years. RESULTS: In 668 patients undergoing minimally invasive pectus repairs, single bars were used in 78.1% and double in 21.7%. Lateral stabilizers were applied in 99.8% and were wired for further stability in 71%. Bar shifts before the use of stabilizers were 14.3%, which decreased to 4.6% after stabilizers were placed and 0.8% with a wired stabilizer and PDS sutures. Results were excellent in 78.5%, good in 13.1%, fair in 4.7% and failed in 3.7% after more than 1 year post bar removal. CONCLUSION: The minimally invasive technique has evolved into an effective method of pectus excavatum repair. Modifications of the technique have reduced complications. Long-term results continue to be excellent.

Adolescent↗