Management of anterior mediastinal masses in children.

BACKGROUND: Children with anterior mediastinal masses are at risk for life-threatening airway compromise during anesthesia, and can present a diagnostic and management challenge for pediatric surgeons. METHODS: We performed a retrospective chart review of all children presenting with an anterior mediastinal mass from 1994-2009. Parameters studied included demographics, historical and physical findings at diagnosis, radiographic evidence of airway compression, diagnostic studies, diagnosis, and complications. RESULTS: There were 26 patients with anterior mediastinal masses over a 15-year period. The mean age was 11.3 years, and there were no gender differences. The diagnoses were lymphoma (62%, 16/26), leukemia (15%, 4/26), and other (23%, 6/26). Diagnosis was made by CBC/peripheral smear in 2/4 patients with leukemia, by bone marrow biopsy in 2/4 patients with leukemia, by thoracentesis in 3/16 patients with lymphoma, by lymph node biopsies in 6/16 patients with lymphoma, and by biopsy of a mediastinal mass in 7/16 patients with lymphoma and in 6/6 patients with other diagnoses. Radiographic evidence of airway compression was seen in 62% (16/26) of children. Only 12% (3/26) had a tracheal cross-sectional area (TCA) <50%. Correlation of symptoms with anatomical airway obstruction or complications was poor. Pulmonary function studies were obtained in 38%, 10/26 children. Only 2 children had a PEFR (peak expiratory flow rate) <50% predicted. This data also correlated poorly with anatomical airway obstruction or complications. 3 patients had anesthesia-related complications: one desaturation during induction prior to median sternotomy, one with significant desaturation and bradycardia during biopsy under local anesthesia with minimal sedation, and one with prolonged (5 days) mechanical ventilation after general anesthesia. 2 of these patients had a TCA <50%, and 2 had SVC obstructions. There were no anesthesia-related deaths, and the overall survival was 85% (22/26). CONCLUSION: Anterior mediastinal masses in children should be approached in a step-wise fashion with multi-disciplinary involvement, starting with the least invasive techniques and progressing cautiously. The surgeon should have a well-defined and preoperatively established contingency plan if these children require general anesthesia for diagnosis.

Outcome of congenital diaphragmatic hernia repair depending on patch type.

INTRODUCTION: Patch repair of a congenital diaphragmatic hernia is associated with a much higher rate of recurrence than when primary repair is feasible. The biosynthetic options for the repair materials continue to expand. We therefore reviewed our experience to benchmark complication rates as we progress with the use of new materials. METHODS: A retrospective review was conducted of all patients who underwent repair of congenital diaphragmatic hernia from January 1994 to May 2009. RESULTS: Of the 155 patients included in the study, 101 patients had primary closure and 54 received a diaphragmatic patch. The rates of recurrence, Small Bowel Obstruction (SBO), and subsequent abdominal operation were all significantly higher in the group of patients requiring patch repair. There were 3 types of patch repairs: 37 patients received a SIS patch, 12 had a nonabsorbable patch, and 5 received an AlloDerm patch. The incidence of SBO in patients with a nonabsorbable mesh was 17% and was associated with a 50% recurrence rate and 67% re-recurrence rate. SIS was associated with 19% incidence of SBO, a recurrence rate of 22% and a 50% re-recurrence rate, whereas AlloDerm had a 40% incidence of SBO, 40% recurrence rate, and 100% re-recurrence rate. DISCUSSION: As we move towards the next generation of materials, these data do not justify the continued comparison with nonabsorbable patches. We do not have enough comparative data to define a superior biosynthetic material, but we plan to use our data on SIS to benchmark our experience with future generation materials.

Bowel obstruction after treatment of intra-abdominal tumors.

BACKGROUND: Tumors of the solid viscera are one of the most common types of pediatric malignancies. Due to the intra-abdominal location of many of these neoplasms, laparotomy and/or bowel resection are often necessary, predisposing patients to postoperative intestinal obstruction. Additionally, chemotherapy and radiation therapy may lead to acute and chronic bowel injury, also potentially predisposing these patients to postoperative bowel obstruction. We reviewed our data over an eleven-year period to identify the incidence of obstruction as well as factors associated with its development. METHODS: A retrospective data analysis of all patients diagnosed with intra-abdominal Wilms' tumor, rhabdomyosarcoma, neuroblastoma, and Hodgkin's and non-Hodgkin's lymphoma in a single institution from 1997 to 2007 was conducted. Data collected included demographic factors, operations, incidence of small bowel obstruction (SBO) and the use of adjuvant or neoadjuvant chemoradiation therapy. Patients who developed SBO were compared to those who did not develop obstruction. Data comparisons were analyzed statistically using Fisher's exact test, 2-tailed Student's t-Test, or chi-square proportional analysis with significance reported for p<0.05. RESULTS: A total of 291 patients were identified during the study period. Mean age at diagnosis was 8.1+/-5.8 years. Males accounted for 57% of all patients. Tumor distribution was as follows: Wilms' tumor: 56 (19%); non-Hodgkin's lymphoma: 71 (24%); Hodgkin's lymphoma: 64 (22%); rhabdomyosarcoma: 32 (11%); and neuroblastoma: 68 (24%). There were a total of 12 bowel obstructions in 11 patients (3.7%). Mean follow-up for all patients was 3.6+/-2.7 years. Children with bowel obstruction were more likely to be male (4.5:1, p=0.061) and younger (4.2 years versus 8.1 years; p=0.087). Wilms' tumor accounted for 45% of patients with bowel obstruction, but made up only 19% of the study population. The incidence of bowel obstruction in patients with Wilms' tumor was 8.9% compared to an overall incidence of 3.8% (p=0.043). CONCLUSION: Bowel obstruction is relatively uncommon after intra-abdominal malignancies in children. Wilms' tumor, rhabdomyosarcoma and Burkitt's lymphoma appear to be associated with the highest risk of bowel obstruction.

Review of the Lynch syndrome: history, molecular genetics, screening, differential diagnosis, and medicolegal ramifications.

More than one million patients will manifest colorectal cancer (CRC) this year of which, conservatively, approximately 3% (approximately 30,700 cases) will have Lynch syndrome (LS), the most common hereditary CRC predisposing syndrome. Each case belongs to a family with clinical needs that require genetic counseling, DNA testing for mismatch repair genes (most frequently MLH1 or MSH2) and screening for CRC. Colonoscopy is mandated, given CRC's proximal occurrence (70-80% proximal to the splenic flexure). Due to its early age of onset (average 45 years of age), colonoscopy needs to start by age 25, and because of its accelerated carcinogenesis, it should be repeated every 1 to 2 years through age 40 and then annually thereafter. Should CRC occur, subtotal colectomy may be necessary, given the marked frequency of synchronous and metachronous CRC. Because 40-60% of female patients will manifest endometrial cancer, tailored management is essential. Additional extracolonic cancers include ovary, stomach, small bowel, pancreas, hepatobiliary tract, upper uroepithelial tract, brain (Turcot variant) and sebaceous adenomas/carcinomas (Muir-Torre variant). LS explains only 10-25% of familial CRC.

Carrier risk status changes resulting from mutation testing in hereditary non-polyposis colorectal cancer and hereditary breast-ovarian cancer.

CONTEXT: In hereditary cancer syndrome families with an identified cancer associated mutation, mutation testing changes the carrier risk status of the tested person and may change the carrier risk status of relatives. OBJECTIVE: This study aimed to describe the change in the distribution of carrier risk status resulting from testing in hereditary breast-ovarian cancer (HBOC) and hereditary non-polyposis colorectal cancer (HNPCC) families. DESIGN: This was an observational cohort study. PATIENTS: The cohort included members of 75 HBOC and 47 HNPCC families. Of the 10 910 cohort members, 1408 were tested for a mutation and learned their test results. OUTCOME MEASURE: Carrier risk for all cohort members was assessed before and after mutation testing. RESULTS: There was a change in carrier risk status in 2906 subjects after testing of 1408 family members. The most common type of carrier risk change, from at risk to non-carrier status, accounted for 77% of the risk changes; 12% were a change to known carrier status from a lower risk. Sixty percent of persons with a carrier risk status change were not themselves tested; their risk status changed because of a relative's test result. CONCLUSIONS: Carrier risk status changes from uncertainty to certainty (that is, to carrier or to non-carrier) account for 89% of risk changes resulting from testing. These risk changes affect cancer prevention recommendations, most commonly reducing their burden. Current practices do not ensure that untested family members are informed about changes in their carrier risk status which result from mutation testing of their relatives.

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.

Semiquantitative polymerase chain reaction in RNase-producing tissues: Analysis of the developing pancreas.

BACKGROUND/PURPOSE: Many studies in pediatric surgical research use a quantitative analysis of gene expression in microscopic quantities of tissue. The authors describe an analysis of the beta-tubulin mRNA content of the embryonic pancreas, which contains abundant endogenous RNases. A detailed analysis of this RNase-containing system will provide a good template for analysis of other potentially simpler systems. METHODS: Embryonic mouse pancreases were harvested at serial gestational ages. DAPI nuclear staining allowed for counting of cells. cDNA was amplified using a fluoresceinated primer and the normalized fluorescence determined. Known numbers of molecules were amplified in parallel as a standard control. RESULTS: The number of cells increased from 38,000 to 2,700,000 between embryonic day 10.5 (E10.5) and E18.5. mRNA for beta-tubulin did not increase proportionately. Assuming a yield of 100% at E10.5 when no RNases are present, the yield of expected mRNA was 65.3% at E12.5, 13.8% at E15.5, and 0.9% at E18.5, presumably because of the appearance of RNases. CONCLUSIONS: Several parameters must be considered in performing semiquantitative reverse transcription polymerase chain reaction: (1) the yield of RNA based on the projected amount of mRNA, (2) the number of cells in the tissue, and (3) a known number of template molecules amplified in parallel.

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.

Effect of pediatric surgical practice on the treatment of children with appendicitis.

OBJECTIVE: Acute appendicitis in children is managed by both general surgeons (GSs) and pediatric surgeons (PSs). Our objective was to investigate the economics of surgical care provided by either GSs or PSs for appendicitis. METHODS: The outcome of children within our state who underwent operative treatment for appendicitis (January 1994 to June 1997) by board-certified GSs were compared with the results of PSs. Data were sorted according to patient age and diagnosis according to the International Classification of Diseases, Ninth Revision. Analysis of variance was performed on continuous data, and chi(2) analysis was performed on nominal data; data are depicted as mean +/- standard error of the mean. RESULTS: GSs (n = 2178) managed older children when compared with PSs (n = 1018; 11.0 +/- 0.1 vs 9.1 +/- 0.1 years) and less frequently treated perforated appendicitis (18.8% vs 31.9%). Independent of diagnosis (simple or perforated appendicitis), younger children (0-4 years, 5-8 years, and 9-12 years) who were treated by PSs had a significantly shorter hospital stay and/or decreased hospital charge when compared with those who were treated by GSs. However, older children (13-15 years) seemed to have comparable outcomes. CONCLUSIONS: Younger children with appendicitis have reduced hospital days and charges when they are treated by PSs.

Multiple gastrointestinal atresias with cystic dilatation of the biliary duct.

A term newborn developed signs and symptoms of an upper-intestinal obstruction. A palpable transverse upper-abdominal mass was identified. An ultrasound examination demonstrated a cystic mass with massive dilatation of the biliary tree. At laparotomy, dozens of segmental intestinal atresias (IA) from the jejunum to the rectum were identified. The cystic mass was the duodenum, obstructed distally from the atresias and proximally from an antral web. The biliary dilatation was due to normal drainage into a closed-loop duodenal obstruction, and was relieved by division of the web. The multiple atresias were explored, but establishment of intestinal continuity was impossible. The entity of multiple segmental IAs in association with cystic biliary dilation has been previously described, but there are no recorded survivors; 35 patients have been reported in the world literature. Our patient was the longest survivor; she finally died after 2 years of gastrostomy-tube drainage and total parenteral nutrition while awaiting intestinal transplantation. Only 5 patients other than ours had documented immune-system abnormalities. We review the findings and management of this rare syndrome.

Nonoperative management of newborn splenic injury: a case report.

Traumatic injury of the spleen is rare in newborns. Nonoperative management of pediatric splenic injuries is now recognized as the treatment of choice, but there is scant experience with the problem in neonates. The authors report their experience with a neonatal splenic rupture, managed nonoperatively.

Outcome analysis for gastroschisis.

BACKGROUND/PURPOSE: Several factors are reportedly associated with an adverse outcome in gastroschisis, including mode of delivery, in utero diagnosis, type of closure, concurrent anomalies, intestinal atresia, and necrotizing enterocolitis (NEC). Since 1969, the authors have treated 185 patients who had gastroschisis. The authors analyzed their database to identify variables associated with increased morbidity and mortality. METHODS: A retrospective study of all patients with gastroschisis treated at our institution in the last 30 years was performed. The characteristics of survivors and nonsurvivors were compared. A logistic regression analysis was performed, with survival as the dependent variable, and the following parameters as independent variables: in utero diagnosis, mode of delivery, gestational age and birth weight, era of repair, type of closure, presence of other associated anomalies, intestinal atresia, and development of necrotizing enterocolitis. Further logistic regression analysis was performed, with various indicators of morbidity as dependent variables. These included development of sepsis, bowel obstruction, and complications related to the closure or to the silo. No attempt at long-term follow-up was made. RESULTS: A total of 185 infants with gastroschisis were treated at our institution from 1969 to 1999. Mean gestational age was 36.6 weeks, and the mean birth weight was 2,501 g. A total of 21 infants had intestinal atresia. NEC developed in 8 infants. Six infants had other serious anomalies. The overall survival rate was 91%. Survival improved in last 2 decades (94%). There were no differences in gestational age, birth weight and mode of delivery, method of closure, or presence of intestinal atresia between survivors and nonsurvivors. Only the era of repair (P = .002), presence of necrotizing enterocolitis (P = .044), and presence of other major anomalies (P < .001) correlated with mortality in the logistic regression analysis. Sepsis, bowel obstruction, and closure complications accounted for most of the morbidity. Analysis of these three morbidity factors identified low gestational age (P = .038) and development of necrotizing enterocolitis (P = .020) as independent predictors of sepsis. Closure complications were only associated with lower birth weight (P = .006). No predictors of bowel obstruction were identified. CONCLUSIONS: Mode of delivery, method of closure, birth weight and gestational age, and the presence of intestinal atresia do not appear to correlate with survival in infants with gastroschisis. Only the presence of another major anomaly, the era of repair, and the development of necrotizing enterocolitis were associated with increased mortality. Degree of prematurity and development of enterocolitis were associated with an increased incidence of septic complications. Low birth weight was a marker for closure complications. Type of delivery (vaginal or cesarean section) had no influence on either morbidity or mortality.

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.

Glans approximation procedure urethroplasty for the wide, deep meatus.

OBJECTIVES: To assess the reliability, cosmesis, and complication rate of the glans approximation procedure (GAP). METHODS: We reviewed 37 consecutive GAP urethroplasties performed at the Children's Mercy Hospital in Kansas City, Missouri over a 5-year period, performed by three different pediatric surgeons. All patients selected had a large, deep ventral groove, typically with a wide open urethral meatus. RESULTS: The mean age was 18 months, with a mean follow-up of 28 months. Of the 37, there was one urethrocutaneous fistula that has since been easily repaired with good results. The parents have been very pleased with the results in all cases, with a straight and strong urinary stream. There was one episode of transient erythema, possibly representing an infection, which resolved after 3 days of oral antibiotics. CONCLUSIONS: The GAP is technically easy, reliable, and offers a relatively low complication rate for the repair of a very select group of patients with hypospadias with a deep ventral glanular groove and wide-mouthed meatus.

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.