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PubMed1999

A 26-year review of pectus deformity repairs, including simultaneous intracardiac repair.

BACKGROUND: We reviewed our operative experience and long-term results with repair of pectus excavatum and carinatum deformities through a vertical midline approach, including those cases with simultaneous intracardiac repair. METHODS: From 1972 through 1998, 120 children underwent pectus deformity repair. Operative technique used a vertical midline incision with subperichondrial resection of deformed cartilages and an anterior sternal osteotomy. Thirty-five patients had a temporary metal bar for retrosternal support for 6 months; 85 underwent repair without a bar. Patients and parents were asked to assess the outcome after pectus repair as poor, fair, good, or excellent. RESULTS: There were 94 male and 26 female patients (mean age, 8.4 years; range, 3 to 21 years). There were 111 cases of pectus excavatum and 9 of pectus carinatum. Fourteen children (11.5%) had an associated congenital heart defect; 9 patients had simultaneous pectus and intracardiac repair. One patient was referred for emergent open heart repair and pectus repair after attempted "Nuss" repair resulted in a perforated right atrium, perforated right ventricle, and partially disrupted tricuspid valve apparatus. There were no deaths and only one significant complication, which required a return to the operating room for bleeding. Morbidity was not higher in patients with simultaneous intracardiac repair. Long-term follow-up was established in 83% of patients. Results were classified as excellent in 64 patients (64%), good in 25 (25%), fair in 8 (8%), and poor in 3 (3%). Thirty (86%) of 35 patients with a sternal bar had excellent results versus 34 (52%) of 65 without a bar (p = 0.004); 97% of patients who underwent repair with a sternal bar classified the result as excellent or good. CONCLUSIONS: Long-term results of pectus excavatum and carinatum repair through a vertical midline approach are excellent. Outcome with a temporary sternal bar is superior to outcome without a bar. Concomitant repair of congenital heart defects and pectus deformity may be performed successfully without additional morbidity.

Adolescent↗
PubMed2000

Congenital Heart Surgery Nomenclature and Database Project: vascular rings, tracheal stenosis, pectus excavatum.

The extant nomenclature for vascular rings, tracheal stenosis, and pectus deformities is reviewed for the purpose of establishing a unified reporting system. The subject was debated and reviewed by members of the STS-Congenital Heart Surgery Database Committee and representatives from the European Association for Cardiothoracic Surgery. All efforts were made to include all relevant nomenclature categories using synonyms where appropriate. Vascular rings are subclassified as double aortic arch, right arch/left ligamentum, pulmonary artery sling, and innominate compression. Tracheal stenosis is subclassified as congenital complete tracheal rings (localized or long-segment) or acquired postintubation types. Pectus deformities are subclassified as pectus excavatum and carinatum (mild, moderate, severe). A comprehensive database set is presented which is based on a hierarchical scheme. Data are entered at various levels of complexity and detail which can be determined by the clinician. These data can lay the foundation for comprehensive risk stratification analyses. A minimum database set is also presented which will allow for data sharing and would lend itself to basic interpretation of trends. Outcome tables relating diagnoses, procedures, and various risk factors are presented.

Aorta, Thoracic↗
PubMed1984

A summary of preoperative and postoperative cardiorespiratory performance in patients undergoing pectus excavatum and carinatum repair.

Preoperative and postoperative pulmonary function tests and progressive work exercise performances were compared for fourteen pectus excavatum and five pectus carinatum patients who underwent operative repair. No changes could be documented in the pectus carinatum patients. The excavatum patients demonstrated a small improvement in total lung capacity (P less than .02) and a significant improvement in maximal voluntary ventilation (P less than .001). In addition, the exercise performances were improved for the excavatum patients postoperatively as quantitated both by the total exercise time and the maximal oxygen consumption (P less than .01). In addition, at any given work rate, the excavatum patient demonstrated a lower heart rate and higher minute ventilation after operation. These findings support the hypothesis that both the restricted cardiac stroke volume and the increased work of breathing that have been described in pectus excavatum patients can be ameliorated by operative intervention.

Adolescent↗
PubMed1989

Cardiac performance in children with pectus excavatum.

Fourteen children with pectus excavatum and 14 normal control patients underwent graded exercise testing using a cycle ergometer and the James protocol. All the subjects were preoperative. The children were exercised to exhaustion during the test. The pectus and control groups were broken down into subgroups consisting of subjects less than or equal to 10 years of age and subjects greater than or equal to 11 years of age (pectus and controls less than or equal to 10 years old, PI and CI, respectively; pectus and controls greater than or equal to 11 years old, PII and CII, respectively). Maximal workload, oxygen consumption, cardiac output, and stroke volume were not significantly different when comparing the total groups or when each of the respective subgroups were compared. However, maximal diastolic BP was significantly elevated when the entire pectus and control groups were compared. When the subgroups were compared, maximal diastolic BP was elevated only in the older pectus patients (PII) and remained so until five minutes after exercise. Additionally, left ventricular systolic time intervals were measured immediately after exercise in all the children. The ratio of preejection period to left ventricular ejection time (P/L) was significantly shortened in the total pectus group. When the subgroups were compared, the P/L ratio was significantly decreased only in the older patients. Also, the preejection period (PEP) was significantly shortened in the older pectus patients. It appears that children with pectus excavatum have a normal exercise tolerance and oxygen transport. However, older pectus children develop an increased diastolic BP and a shortened P/L and PEP in response to exercise.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1996

Continuous thoracic epidural infusions for postoperative analgesia after pectus deformity repair.

PURPOSE: To determine whether continuous epidural analgesia after repair of a pectus deformity is a viable and safe alternative to high-dose narcotics in children. METHODS: Data were collected prospectively for 19 children (4 to 17 years of age; 15 boys, 4 girls) who underwent pectus excavatum (14) or carinatum (5) repair between June 1, 1991 and July 1, 1994. Seventeen had a thoracic epidural catheter placed for postoperative pain control and two did not. The epidural catheter was routinely plead preoperatively by the anesthesiologist at the T3-T8 level, after induction of general anesthesia. Epidural catheters were test-dosed with local anesthesia alone or in combination with fentanyl, and afterward a continuous epidural infusion was maintained on the floor. Postoperative pain was assessed by nursing and house staff on the Wong-Baker scale, with adjustment of the dose rate or analgesic medication as appropriate. RESULTS: All patients had extubation before leaving the operating room and were sent to the general pediatrics ward after leaving the recovery room. The average duration of the epidural was 69 hours (range, 20 to 116 hours). Sixteen patients received their test epidural dose preoperatively, and one patient had his in the recovery room. Fifteen epidural initially were dosed with bupivicaine (1 to 2 mg/kg) alone or in combination with fentanyl (1 to 2 micrograms/kg). Two patients received initial doses of lidocaine (1 to 1.5 micrograms/kg). Ten of 17 patients received fentanyl (1 microgram/kg/h) with bupivicaine (0.5 to 1.0 mg/kg/h) in the epidural as their maintenance medication, and the remainder received bupivicaine alone at the same dosage rate. Eight of 17 patients required additional intermittent supplemental narcotics, with an average of two doses of intravenous morphine per day (0.1 mg/kg) over the first 3 postoperative days. In contrast, the two patients who did not have an epidural catheter for pain control required high-dose intravenous morphine (0.2 mg/kg) every 2 to 3 hours for the first 3 to 4 postoperative days. No catheter-related complications occurred. CONCLUSION: Thoracic epidural analgesia was completely successful in nine (53%) children who underwent repair of pectus deformity, and effectively reduced the intravenous narcotic demand in the other eight. Pain control was excellent, and no catheter-related complications were encountered. The data show that this method of analgesia in children is a safe and attractive alternative to intravenous narcotics, and eliminates the potential disadvantages of sedation and respiratory compromise.

Adolescent↗
PubMed1998

Operative management of asphyxiating thoracic dystrophy after pectus repair.

Pectus excavatum repair usually results in unchanged or improved pulmonary function. However, a small subset of patients will experience severely impaired pulmonary function after pectus repair caused by restrictive lung disease, and no adequate surgical approach has been described for this condition. A procedure is described that is a variation of an operation for Jeune's thoracic dystrophy, that resulted in marked respiratory improvement in this setting. A 14-year-old boy had undergone standard pectus excavatum repair at age 4, from which he recovered uneventfully. Beginning at age 10 to 12 years progressive restrictive pulmonary disease, recurrent pneumonia, and cor pulmonale developed, which resulted in almost constant shortness of breath and the need for continuous nasal positive pressure support. Pulmonary function test results were markedly abnormal and worsening. He underwent an operative procedure consisting of sternal split that was wedged open permanently with rib struts, opening of pleura bilaterally, and six rib resections bilaterally. His postoperative recovery was satisfactory, and his pulmonary functions have shown steady improvement. He is now completely off oxygen and pressure support, has improved exercise tolerance, and has returned to school. Severe restrictive lung disease after pectus repair can be successfully managed with aggressive operative procedures. Patients should have close follow-up after pectus repair for the development of this potentially debilitating disorder to allow earlier repair.

Adolescent↗
PubMed1999

Repair of pediatric pectus deformity and congenital heart disease as a combined procedure.

Coexisting pectus deformity and congenital heart disease is not uncommon. Traditionally, the approach to this problem has been to repair each one with a separate surgical procedure because of fear of increased complications from bleeding, infections, and anesthesia. More recently, many reports of successful combined repair have been published, particularly in adults with coronary artery or aortic pathology. The authors wished to determine the feasibility of this combined procedure in younger patients, particularly those with a severe pectus deformity. Three patients underwent repair, including a 17 year old with Marfan's syndrome and a severe pectus excavatum deformity. The postoperative course was smooth for these patients, and all had good short- and long-term (over 18 months) results.

Adolescent↗
PubMed2000

Why is exercise capacity reduced in subjects with pectus excavatum?

BACKGROUND: Exercise capacity is often limited in subjects with pectus excavatum (PE), but the mechanism is unknown. OBJECTIVES: We attempted to quantify exercise capacity and to investigate whether limitation of venous return to the heart contributes to exercise intolerance in PE. METHODS: A total of 13 patients with PE (mean age, 19 +/- 6 years) and 20 control subjects (mean age, 25 +/- 11 years) underwent sitting and supine incremental cycling and exercise Doppler stroke volume (SV) measurements. RESULTS: Supine peak oxygen uptake (V'O(2)max) for the patients (1351 +/- 345 mL/min) and control subjects (1505 +/- 330 mL/min) was not different. In contrast, sitting V'O(2)max was lower in the patients than in the control subjects, 1480 +/- 462 and 1994 +/- 581 mL/min, respectively (P =.02). Supine exercise SV was not different between groups. Moreover, only in the patients with PE was supine exercise SV, 70 +/- 18 mL, higher than sitting exercise SV, 55 +/- 14 mL (P =.015). The corresponding values for the control subjects were 70 +/- 18 mL and 65 +/- 19 mL (P = NS). CONCLUSIONS: Patients with PE exercising in the sitting position have reduced V'O(2)max and SV, whereas during supine exercise they approached the control values. The supine advantage in PE suggests that upright exercise capacity in this disease is affected by reduced filling of the heart in the non-supine position.

Adult↗
PubMed1975

The Coffin-Lowry syndrome: an inherited faciodigital mental retardation syndrome.

Eight patients in three families had mental retardation, characteristic facies and hands, and skeletal changes; the clinical features suggested to us that they had a syndrome previously thought to represent two entities described by Lowry and associates and by Coffin and associates, respectively. New findings include skeletal, orodental, and dermatoglyphic abnormalities and histopathologic changes suggesting that the syndrome is a heritable disorder of connective tissue. Severe expression in males and transmission through mildly affected females suggest X-linked or sex-influenced autosomal dominant inheritance.

Adolescent↗
PubMed1980

Association between pectus excavatum and segmental bronchomalacia.

Five children presented with recurrent respiratory illnesses and were noted to have pectus excavatum. In four patients subsequent investigation demonstrated a collapsible segment in the middle third of the left main bronchus, which appeared to lack cartilage support and caused gas trapping in the left lung. The remainder of the bronchial tree was normal. In the remaining child the clinical and physiologic pattern was very similar. In three children there were no other anomalities, but two had congenital heart disease. The association of bronchial anomalies and pectus excavatum may occur more often than suspected.

Abnormalities, Multiple↗