The effect of pharmacogenetic profiling with a clinical decision support tool on healthcare resource utilization and estimated costs in the elderly exposed to polypharmacy.

OBJECTIVE: To compare healthcare resource utilization (HRU) and clinical decision-making for elderly patients based on cytochrome P450 (CYP) pharmacogenetic testing and the use of a comprehensive medication management clinical decision support tool (CDST), to a cohort of similar non-tested patients. METHODS: An observational study compared a prospective cohort of patients ≥65 years subjected to pharmacogenetic testing to a propensity score (PS) matched historical cohort of untested patients in a claims database. Patients had a prescribed medication or dose change of at least one of 61 oral drugs or combinations of ≥3 drugs at enrollment. Four-month HRU outcomes examined included hospitalizations, emergency department (ED) and outpatient visits and provider acceptance of test recommendations. Costs were estimated using national data sources. RESULTS: There were 205 tested patients PS matched to 820 untested patients. Hospitalization rate was 9.8% in the tested group vs. 16.1% in the untested group (RR = 0.61, 95% CI = 0.39-0.95, p = 0.027), ED visit rate was 4.4% in the tested group vs. 15.4% in the untested group (RR = 0.29, 95% CI = 0.15-0.55, p = 0.0002) and outpatient visit rate was 71.7% in the tested group vs. 36.5% in the untested group (RR = 1.97, 95% CI = 1.74-2.23, p < 0.0001). The rate of overall HRU was 72.2% in the tested group vs. 49.0% in the untested group (RR = 1.47, 95% CI = 1.32-1.64, p < 0.0001). Potential cost savings were estimated at $218 (mean) in the tested group. The provider majority (95%) considered the test helpful and 46% followed CDST provided recommendations. CONCLUSION: Patients CYP DNA tested and treated according to the personalized prescribing system had a significant decrease in hospitalizations and emergency department visits, resulting in potential cost savings. Providers had a high satisfaction rate with the clinical utility of the system and followed recommendations when appropriate.

Minimally invasive bar repair for 'redo' correction of pectus excavatum.

BACKGROUND/PURPOSE: A small percentage of patients who have undergone traditional, "Ravitch-type" pectus excavatum repair present with unsatisfactory results and require a second procedure for correction. Reoperative open surgery for pectus excavatum has been associated with extensive dissection and substantial blood loss. The minimally invasive (MIS) bar repair for the correction of pectus excavatum has been gaining acceptance. This study evaluates the authors results with patients who have undergone the MIS bar repair for redo correction of their pectus excavatum. METHODS: A retrospective chart review of all patients undergoing MIS bar repair between December 1997 and August 2001 was performed. Information about demographics, deformity, operative course, complications, and early outcome was recorded. RESULTS: Ninety-two patients underwent MIS repair during this period. Ten patients had redo MIS bar repair for unsatisfactory prior open correction. Operating time was 52 minutes for standard patients and 70 minutes for the redo patients (P <.001). Blood loss and postoperative hospitalization were similar between groups. CONCLUSION: The minimally invasive pectus repair can be performed safely with minimal blood loss and short operating time in patients who have undergone prior unsatisfactory open repair of pectus excavatum and can be an alternative approach to reoperative open repair in these patients.

Minimally invasive repair of pectus excavatum: a single institution's experience.

BACKGROUND: The Nuss repair of pectus excavatum is a relatively new, minimally invasive surgical (MIS) alternative to the traditional open "Ravitch-type" operation. We have one of the larger single-center experiences to date, and we conducted this clinical study to evaluate our early experience, emphasizing initial outcome and technical modifications designed to minimize complications. METHODS: A retrospective chart review was performed on 112 patients who underwent 116 pectus excavatum repairs between January 1995 and January 2001. The Nuss procedure was performed in 80 patients, and open repair was performed in 32 patients. Information about demographics, deformity, operative course, complications, and early outcome was recorded. RESULTS: Operative duration was 143 minutes for the open group and 53 minutes for the Nuss MIS group (P <.001). Blood loss was 6 mL/kg for the open group and 0.5 mL/kg for the MIS group (P <.001). Postoperative hospitalization was 3.2 days for the open group versus 3.7 days for the MIS group (P<.05). CONCLUSIONS: The MIS pectus repair can be performed safely with minimal blood loss and reduced operative time. Short-term analysis of the quality of repair, including absence of preoperative symptoms, patient satisfaction, and cosmetic appearance are encouraging.

Management of intestinal atresia in patients with gastroschisis.

BACKGROUND/PURPOSE: Intestinal atresia occurs in approximately 10% to 20% of children with gastroschisis and may be missed at the initial closure if a thick peel obscures the bowel. Some investigators have identified intestinal atresia as a significant contributor to morbidity and mortality. The authors reviewed their experience with gastroschisis and intestinal atresia in an attempt to answer the following questions. What is the incidence of this association? How often is the intestinal atresia unrecognized as a result of the peel? What is the optimal management for infants with atresia and gastroschisis, and does the atresia affect morbidity or mortality? METHODS: The hospital charts and medical records of all patients with gastroschisis treated at our institution from 1969 to present were reviewed thoroughly. Parameters analyzed included gestational age (GA), birth weight (BW), antenatal diagnosis, mode of delivery, type of closure, era of repair, presence of other major anomalies, and development of necrotizing enterocolitis. Morbidity and mortality rates were examined. Characteristics of patients with and without atresia were compared. Chi-squared was used for crosstabular analysis. Sample parameters were compared with Student's t test. P values of less than.05 were considered significant. RESULTS: A total of 199 babies had gastroschisis and 25 (12.6%) had intestinal atresia. Intestinal atresia was initially unrecognized in 3 patients. Most patients (80%) underwent primary closure of the abdominal wall. Initial stoma formation and delayed anastomosis was performed in 12 (48%) patients, none of whom required prosthetic material for abdominal wall closure. Initial stomas were avoided in 5 patients who required SILASTIC (Dow Corning, Midland, MI) silos. Skin closure alone was used in 2 babies. The level of the atresia was most commonly jejunoileal (20 of 25, 80%). Mean hospital stay was increased in babies with intestinal atresia, 36.2 versus 63.1 days (P <.001). CONCLUSIONS: Although patients with intestinal atresia did have feeding delays, an increased incidence of adhesive intestinal obstruction, and prolonged hospitalization, neither chi(2) nor logistic regression analysis showed any correlation with mortality. Intestinal repair at the first operation is sometimes possible and depends on the severity of the peel. Delayed repair of the atresia after a period of bowel decompression and parenteral nutrition is preferred, but in certain situations (colonic atresia, necrotic intestine, complicated atresia) may not be possible. The combination of stomas and prosthetic material can be avoided in almost all patients. A management algorithm for patients with atresia and gastroschisis is discussed.

Vascular anomalies and tracheoesophageal compression: a single institution's 25-year experience.

BACKGROUND: Vascular rings are uncommon anomalies in which preferred strategies for diagnosis and treatment may vary among institutions. In this report, we offer a description of our approach and a review of our 25-year experience. METHODS: A retrospective review was conducted of all pediatric patients with symptomatic tracheoesophageal compression secondary to anomalies of the aortic arch and great vessels diagnosed from 1974 to 2000. RESULTS: Thirty-one patients (38%) of eighty-two patients (mean age, 1.7 years), were identified with double aortic arch, 22 patients (27%) with right arch left ligamentum, and 20 patients (24%) with innominate artery compression. Our diagnostic approach emphasized barium esophagram, along with echocardiography. This regimen was found to be reliable for all cases except those with innominate artery compression for which bronchoscopy was preferred, and except those with pulmonary artery sling for which computed tomography or magnetic resonance imaging, in addition to bronchoscopy, were preferred. Left thoracotomy was the most common operative approach (70 of 82; 85%). Ten patients (12%) had associated heart anomalies, and 6 (7%) patients underwent repair. Complications occurred in 9 (11%) patients and led to death in 3 (4%) patients. CONCLUSIONS: In our practice, barium swallow and echocardiography are sufficient in diagnosing and planning the operative strategy in the majority of cases, with notable exceptions. Definitive intraoperative delineation of arch anatomy minimizes the risk of misdiagnosis or inadequate treatment.

Thoracotomy for persistent bronchopleural fistula in the very low birth weight infant.

Although conservative treatment is appropriate for most very low birth weight infants with bronchopleural fistulas, early surgical closure may improve survival in properly selected patients. We report our experience with successful surgical closure in 3 consecutive neonates weighing <800 g.

The diagnosis of cor triatriatum sinistrum in children: a continuing dilemma.

Cor triatriatum (CT) is a rare congenital cardiac anomaly. The salient clinical, roentgenographic, electrocardiographic, echocardiographic, and hemodynamic findings are presented in two asymptomatic children and one with nonspecific dyspnea on exertion. Two male children had a classical form of cor triatriatum with normal physical and inconsequential roentgenographic and electrocardiographic findings. One of the male patients had surgery for a large atrial septal defect ostium secundum (ASD 2 degrees) and pulmonary hypertension in infancy. The female patient had CT with a communicating accessory chamber to right atrium and a rare patent foramen ovale. Her clinical findings confirmed an atrial level shunt. All patients had excision of the fibromuscular membrane from the right and left atrial transseptal approach with excellent results and with no recurrence during 2 to 4 year follow-up. We report the dilemma encountered in the clinical diagnosis of CT in children and the pivotal role played by echocardiography in the diagnosis of this anomaly.

Survival of patients with esophageal atresia: influence of birth weight, cardiac anomaly, and late respiratory complications.

PURPOSE: The aim of this study was to determine the influence of various prognostic factors on the outcome of esophageal atresia patients. METHODS: The authors reviewed 240 charts of patients admitted with esophageal atresia or tracheoesophageal fistula (EA-TEF) in a single institution. A logistic regression model was used with survival as the dependent variable and era of repair, birth weight, and cardiac anomaly as independent variables. RESULTS: Era was statistically significant (P = .011); 87% (117 of 134) of patients survived in the era from 1980 through 1997 compared with 78% (83 of 106) from 1960 through 1979. Cardiac anomaly (CHD) was a significant risk factor (P = .001); 88% (176 of 199) survived without cardiac anomaly, whereas only 59% (24 of 41) survived with cardiac disease. Eighty-four percent (185 of 219) of infants with a birth weight (BW) of more than 1,500 g survived, compared with 71% (15 of 21) of infants with a birth weight of less than 1,500 g. This was not statistically significant (P = .59). Early hospital deaths were primarily cardiac and chromosomal (61%). Late deaths were primarily respiratory (59%; two-tailed Fisher's Exact test, P = .004). CONCLUSIONS: (1) Survival of patients with esophageal atresia has significantly improved in the recent years. (2) Low birth weight (<1,500 g) does not seem to affect survival. (3) Associated cardiac and chromosomal anomalies are significant causes of death, particularly for early demise. (4) Late death from respiratory disease (tracheomalacia, reactive airway disease, reflux, and aspiration) warrants attention, and a close follow-up of postoperative patients is suggested.

Ping-pong ball plombage for right postpneumonectomy syndrome in children.

BACKGROUND/PURPOSE: Right postpneumonectomy syndrome is a rare complication of pneumonectomy characterized by exertional dyspnea resulting from mediastinal shift into the empty hemithorax. Historically, this problem has been treated with thoracoplasty, muscle flap transposition, pericardial fixation, and plombage using a variety of materials. Significant postoperative complications have been reported with each of these methods. In an effort to avoid the problems known to be associated with other plombage materials, we have used sterilized ping-pong balls as plombage to treat the postpneumonectomy syndrome. METHODS: Two children with pneumonectomy syndrome were treated with ping-pong ball plombage. RESULTS: Symptoms improved immediately in both patients and postoperative chest x-rays in each showed a normal cardiac position. Neither child had a postoperative complication. Six-year follow-up chest films in both children have demonstrated some fluid filling of the ping-pong balls, but no clinical problems attributable to them have been noted. CONCLUSION: The authors conclude that ping-pong ball plombage appears to be a viable management option in children with right postpneumonectomy syndrome.

Inferior vena caval obstruction from a horseshoe kidney: report of a case with operative decompression.

Horseshoe kidney is a common anomaly with frequent associated anomalies. The confluence of the lower poles of the kidneys lies anterior to the inferior vena cava. The authors were unable to find any reports in the world's literature of a vena caval obstruction caused by the presence of a horseshoe kidney. The authors report such a case presenting as bilateral lower leg swelling.

Surgical treatment of gastroesophageal reflux in children: a combined hospital study of 7467 patients.

OBJECTIVE: To review retrospectively the combined clinical experience with the surgical treatment of persistently symptomatic gastroesophageal reflux (SGER) in childhood from seven large children's surgery centers in the United States. DESIGN: During the past 20 years, 7467 children <18 years of age underwent antireflux operations for SGER at the seven participating hospitals. Fifty-six percent were neurologically normal (NN) and 44% were neurologically impaired (NI). The most frequent diagnostic studies were upper gastrointestinal series (68%), esophageal pH monitoring (54%), gastric emptying study (32%), and esophagoscopy (25%). The age at operation was under 12 months in 40% and 1 to 10 years in 48%. The type of fundoplication was Nissen (64%), Thal (34%), and Toupet (1.5%). A gastric emptying procedure was performed on 11.5% of NN patients and 40% of NI patients. Laparoscopic fundoplication was performed on 2.6% of patients. RESULTS: Good to excellent results were achieved in 95% of NN and 84.6% of NI patients. Major complications occurred in 4.2% of NN and 12.8% of NI patients. The most frequent complications were recurrent reflux attributable to wrap disruption (7.1%), respiratory (4.4%), gas bloat (3.6%), and intestinal obstruction (2.6%). Postoperative death occurred in 0.07% of NN and 0.8% of NI patients. Reoperation was performed in 3.6% of NN and 11.8% of NI patients. The results and complications were similar among the participating hospitals and did not seem related to the type of fundoplication used. CONCLUSION: The excellent results (94% cure) and low morbidity with gastroesophageal fundoplication with or without a gastric emptying procedure from a large combined hospital study indicate that operation should be used early for SGER in NN children and to facilitate enteral feedings and care in NI children.

Comparison of muscle-sparing thoracotomy and thoracoscopic ligation for the treatment of patent ductus arteriosus.

PURPOSE: We reviewed our experience with the treatment of patent ductus arteriosus (PDA), and compared two therapeutic techniques; muscle-sparing thoracotomy (MST) and thoracoscopic PDA ligation (TPDAL). METHODS: We reviewed the clinical records and operative reports of 19 nonnewborn patients who were treated at our institution for PDA. Eleven patients underwent TPDAL and eight patients MST. The TPDAL patients underwent thoracoscopic clipping (ligation) of the PDA, whereas the MST group had a complete division of the ductus. A two-tailed Student's t test was used to calculate the 95% confidence intervals for length of operation, number of doses of intravenous narcotics and hospital stay. Costs were also compared. RESULTS: All patients underwent diagnostic echocardiography in the evaluation of an asymptomatic murmur. Both groups were similar in age (average, 4 years) and gender. All procedures were performed electively. Two thoracoscopic attempts were aborted, one for bleeding and the other for inadequate clip size. The length of the procedure averaged 1.3 hours +/- 0.330 SD for TPDAL versus 1.4 hours +/- 0.335 SD for MST. Five of the nine successful TPDAL patients were admitted to the Pediatric Intensive Care Unit (PICU) with an average length of stay of 20 hours, and two of eight MST patients stayed in the PICU for average of 18 hours. All patients were extubated after the procedure. Chest tubes were placed in two TPDAL patients and three MST patients. However, two patients who underwent TPDAL required tube thoracostomy for persistent postoperative pneumothorax. Average number of intravenous narcotics administered for the TPDAL was 1.2 doses per patient and for MST, 1.75 doses per patient. Mean hospital stay for TPDAL was 1.33 +/- 0.71 SD days and for MST 1.8 +/- 0.83 SD days. Ninety-five percent (95%) confidence intervals for the difference in means demonstrated no difference between the two groups for length of operation, hospital stay, or number of doses of intravenous narcotics administered. CONCLUSIONS: The authors were unable to identify any benefit to thoracoscopic patent ductus arteriosus ligation versus muscle-sparing thoracotomy in terms of hospital stay, length of operation, or morbidity. Additionally, with MST there is a complete division of the PDA theoretically decreasing the risk of recurrence in comparison with clip ligation.

Balloon extraction of esophageal foreign bodies in children.

BACKGROUND: Whereas esophageal foreign bodies are commonly removed with rigid esophagoscopy under general anesthesia, selected foreign bodies also can be removed using a Foley catheter balloon under fluoroscopic control without anesthesia. The authors prefer to initially attempt removal of smooth, radiopaque esophageal foreign bodies using the balloon technique and then proceed to rigid esophagoscopy if unsuccessful. METHODS: The authors reviewed their 7-year experience with this approach in 276 children with esophageal foreign bodies. RESULTS: One hundred seventy-three children underwent attempted balloon extraction, and 146 of these underwent successful removal with this method. Fifteen remained lodged in the esophagus and were subsequently removed by esophagoscopy, and 12 passed into the stomach and were observed. The only complication was one episode of epistaxis that resolved. Success of the balloon method was not related to patient age, foreign body duration, location, or presenting symptoms. The use of more than 5 minutes of fluoroscopy time was associated with a low likelihood of successful extraction. CONCLUSION: The balloon extraction technique is a safe and effective alternative to rigid esophagoscopy for the removal of selected esophageal foreign bodies in children.

Efficacy of partial wrap fundoplication for gastroesophageal reflux after repair of esophageal atresia.

Gastroesophageal reflux (GER) often develops in children who have undergone prior repair of esophageal atresia/tracheoesophageal fistula (EA/TEF). Fundoplication is necessary in many of these children. The complete wrap (Nissen) fundoplication is often used in this setting. However, poor results have been noted, with a mean failure rate of 30% reported in four recent studies. A partial wrap fundoplication for GER associated with EA/TEF is theoretically attractive, because the poor esophageal motility and diminished acid clearance (already physiologically present) is exacerbated by a complete wrap fundoplication. The authors reviewed their extensive experience with partial wrap (Thal) fundoplication in EA/TEF to determine if the failure rate was better than that reported for the Nissen fundoplication. In the past 18 years, the authors performed 1,467 fundoplication procedures. During the same period, 143 children underwent repair of EA/TEF. Fifty-nine children underwent fundoplication after a previous EA/TEF repair. Most of the fundoplications (58 of 59, 98%) were Thal procedures. Defining failure strictly as a need for reoperation, the failure rate in our series was 15% (9 of 59 children). Compared with the failure rate in the 1,408 non-EA/TEF patients (61 of 1408, 4.3%), results were significantly worse for the EA/TEF group (P > .001). The failure rate of Thal fundoplication performed for GER in the EA/TEF population is substantially higher than the non-EA/TEF patients. The same factors responsible for the development of reflux in these children (poor acid clearance, altered motility, esophageal shortening) may contribute to the higher failure rate. Although partial wrap fundoplication frequently failed (15%), the results were still substantially better than those reported for Nissen fundoplication in these children (30% failure rate).