Evaluation of aortopexy in the management of severe tracheomalacia after esophageal atresia repair.

Severe tracheomalacia (TM) is a difficult problem in esophageal atresia (EA) patients. We reviewed our experience with aortopexy and other interventions for severe TM in this population. With review ethics board approval, a retrospective review of TM in postoperative EA patients was conducted (1989-2010). Demographics, perinatal, and surgical information regarding EA repair was collected. TM infants were analyzed for symptomatology, clinical severity, investigations, interventions, and outcomes. Data are presented as proportions or median(range). One hundred and thirty-two EA patients were reviewed. Most had type C atresia (87.3%), and 18 patients (13.6%) died. Twenty-five patients (18.9%) had TM of whom five (20%) died. Median symptom onset was 18 days (0-729) after EA repair, with stridor (64%) or retractions/distress (44%) being most frequent. Four and two patients had airway obstruction or cardiorespiratory arrest, respectively. Median time from symptom onset to investigations was 11 days; these were most commonly rigid bronchoscopy (56%) and fluoroscopy (36%). Ten patients (40%) had severe TM on bronchoscopy. Six underwent aortopexy, one fundoplication, and three were treated medically. Length of hospital stay (LOS) post-aortopexy was 13 days (5-60), and ventilation time was 2 days (0-9). LOS was 60.5 (1-69) days postdiagnosis in non-aortopexy patients. Readmission rates for respiratory issues were significantly less in the aortopexy (median 0 vs. 5; P = 0.048) group over 2-year follow up after discharge. Complications of aortopexy included transfusion (1) and temporary diaphragmatic paresis (1), and one mortality secondary to severe congenital cardiac anomalies. Our experience suggests that aortopexy is safe and effective for the treatment of severe TM. It is associated with reduced LOS compared with other treatment strategies and few complications or long-term sequelae.

Thoracotomy for repair of esophageal atresia: not as bad as they want you to think!

Is it outdated now to do a thoracotomy to repair esophageal atresia (EA)? Our practices and the literature on the subject of thoracoscopic and open thoracotomy repair of EA were reviewed, seeking answers to the following questions: Is it correct to compare the new thoracoscopic approach for the repair of EA against the thoracotomy techniques of 15-30 years ago? Should post-thoracotomy scoliosis/thoracic deformity reported in up to 56% of patients be a significant current concern? Are the clips used to close the fistula in thoracoscopic repairs as safe as open suture closures? Is the leak and stricture rate similar with thoracoscopic surgery? Are the anesthesia, period of ventilation, pain, time to first feeding, and the length of hospital stay significantly different with current thoracotomy techniques compared with thoracoscopic methods? Is the cosmetic result of a thoracoscopic repair significantly better? Is the learning curve for EA thoracoscopic repair harming patients for minimal long-term benefit? These questions were scientifically unanswerable at this time. The limited EA thoracotomies currently performed have a track record of proven safety and minimal morbidity. The results published by surgeons who are pioneers in thoracoscopy may not be generalizable, and the complication rate from teams with less experience is likely underreported. In selected patients and with experienced teams, thoracoscopic EA repair is appropriate. However, EA repair via thoracotomy should, for now, remain as the 'gold standard'. Further registry-based, multicenter, comparative studies on EA repair methodologies and outcomes should provide important answers.

Refractory strictures post-esophageal atresia repair: what are the alternatives?

Esophageal strictures remain the most frequent complication after esophageal atresia (EA) repair despite refinements in operative techniques. With an incidence of anastomotic stricture between 8% and 49%, EA is the most frequent cause of benign esophageal stricture in children. The mainstay of treatment for esophageal stricture is dilatation with a 58-96% success rate. In order to relieve dysphagia, between 1 and 15 dilatations will be required in each EA patient with an esophageal stricture. However dilatations may lead to complications including perforation (0.1-0.4% of all esophageal benign strictures) and sociopsychological morbidity. Fifty percent of EA strictures will improve in 6 months. However, 30% will persist and require repeat dilatations. The present article explores the variety of non-surgical alternative treatments for anastomotic strictures after EA repair, focusing on triamcinolone acetonide, mitomycin C and esophageal stents. We propose an algorithm for a more standardized therapeutic approach, with the hope that an international panel of experts could meet and establish a consensus.

Surgical and metabolic aspects of liver transplantation for tyrosinemia.

Tyrosinemia is the diagnosis of a very small percentage of patients undergoing liver transplantation worldwide. Tyrosinemia is endemic within our referral area however, and fully one-third of the liver transplantations at our institution are done for this disease. Since 1986, 15 patients with tyrosinemia (TYR) and 31 patients with various other indications (non-TYR) have undergone a total of 51 liver transplantations. The 36-month actuarial survival for TYR patients is 87%, compared with 74% for non-TYR patients. Liver transplantation for hereditary tyrosinemia and other metabolic disorders without portal hypertension or previous portohepatic operations is notably easier to perform. Intraoperative blood loss was less, length of hospital stay was shorter, and incidence of infections was lower in TYR than in non-TYR patients. Less than 10% of TYR patients had foci of hepatocellular carcinoma at the time of transplantation. For this reason, and while most patients with tyrosinemia will eventually require liver transplantation, our results do not support systematic early transplantation before the age of two years.

Nonoperative management of biliary leaks after orthotopic liver transplantation.

Specific therapy should be instituted expeditiously once the diagnosis of a biliary leak has been made in patients who have undergone orthotopic liver transplantation. Controversy exists over whether to use nonoperative or operative management. The results of 325 consecutive orthotopic liver transplants in 297 adult and pediatric recipients were reviewed. The biliary tract was reconstructed using a choledochocholedochostomy anastomosis (254/325 or 78%) or a Roux-en-Y choledochojejunostomy anastomosis (71/325 or 22%). The incidence of biliary leaks was 23% (74/325). Overall, only 3% (10/325) of the orthotopic liver transplant recipients required operative repair of a biliary leak. Biliary leaks occurring in patients with Roux-en-Y choledochojejunostomy anastomoses (9/71 or 13%) commonly required operative repair (6/9 or 67%), whereas leaks that occurred in patients with choledochocholedochostomy anastomoses (65/254 or 26%) seldom required operative repair (4/65 or 6%). All choledochojejunostomy leaks occurred at the anastomosis, whereas choledochocholedochostomy leaks occurred either at the anastomosis (17/254 or 7%) or the T-tube insertion site (45/254 or 18%). Our study confirms that in centers with proficient endoscopic and interventional radiologic support, resolution of biliary leaks in orthotopic liver transplant patients can be achieved with nonoperative management.

Reversal of intrapulmonary shunting in cirrhosis after liver transplantation demonstrated by perfusion lung scan.

A young girl with biliary atresia leading to cirrhosis developed respiratory complications with hypoxemia. Intrapulmonary shunting was diagnosed with a 99mTc-MAA perfusion lung scan, which showed marked systemic activity. The shunting resolved after liver transplantation. The perfusion lung scan offers an efficient method to screen patients with cirrhosis in whom intrapulmonary shunts are suspected and to follow their progress.

Radiographic recognition of chest tube malposition in the major fissure.

Twelve patients with a chest tube positioned in the major fissure of either lung were collected prospectively in a two-year period. In eight of these 12, there was evidence of chest tube malfunction, with poor drainage of pleural air or fluid collections; in six of these eight, placement of additional tubes or tube repositioning resulted in improved pleural drainage. In all 12, the tube had been placed through the lateral chest wall. Although tube placement in the major fissure can sometimes be suspected on the anteroposterior portable chest roentgenogram because of the tube's characteristic course, in such cases a lateral roentgenogram is not usually obtained for confirmation of location unless there is evidence of tube malfunction.

Peripheral hepatic arterial embolization with crosslinked collagen fibers.

Hepatic artery embolization with a nonimmunogenic, crosslinked microfibrillar collagen preparation (Angiostat, Collagen for Embolization, Target Therapeutics, Los Angeles, CA) was studied in mongrel dogs. Flow-directed technique was used to achieve complete distal arterial occlusion. Serial liver function evaluation demonstrated marked alterations at 48 to 72 hours, partial correction at one week, and resolution of abnormalities by one month. Restoration of large vessel blood flow was angiographically demonstrable at one week. Follow-up arteriograms showed no persistent arterial occlusion. Collagen was demonstrated in vessels of 20 to 250 micron. Recanalization was achieved by migration of endothelial cells around the collagen, development of a new vascular channel within an endothelial cell cleft, and subsequent complete removal of the collagen over a three- to four-month period. Three months after embolization with a single dose, normal hepatic vascular and tissue anatomy and hepatic function were restored completely. Repeated embolization at two weekly intervals was well tolerated.

A new prognostic classification for esophageal atresia.

BACKGROUND: Waterston's risk categories have been extensively used in the past for the classification and management of neonates with esophageal atresia. Advances in neonatal care have affected the prognostic usefulness of the Waterston classification. The purpose of this study is to formulate a new classification of risk factors that would more accurately predict outcome. METHODS: We retrospectively reviewed 95 consecutive cases of esophageal atresia and/or tracheoesophageal fistula treated at the Montreal Children's Hospital. Fifteen patients were in the Waterston class A, with a 6.7% mortality; 35 patients, in class B with a 5.7% mortality; and 45 patients, in class C with a 26.7% mortality. Logistic regression analysis of the influence of each risk factor (weight, pulmonary status, and severity of associated anomalies) on mortality was performed. RESULTS: Birth weight was not found to independently influence mortality. Only severe pulmonary dysfunction with preoperative ventilator dependence and severe associated anomalies had a prognostic influence. We therefore revised the classification to include only significant factors. The new high-risk class II consists of patients with either life-threatening anomalies or both major anomalies and ventilator dependence; the low-risk class I includes all other patients. These criteria radically changed the stratification of both the number of cases and the mortality among classes: the 82 patients in class I had a 7.3% mortality; the 13 patients in class II had a 69.2% mortality. Logistic regression analysis confirmed the validity of this new classification by showing displacement of individual variables by the revised classification but not by Waterston's. CONCLUSIONS: This new "Montreal classification" can simplify and improve the stratification of patients with esophageal atresia. It also reflects the more favorable outcome of low birth weight neonates.

Diaphragmatic pacing in children with congenital central alveolar hypoventilation syndrome.

BACKGROUND: The purposes of this study were to study congenital central alveolar hypoventilation syndrome (CCAHS), to report the experience at our institution with this disorder, and to describe the surgical technique of diaphragmatic pacing. Three patients are in our diaphragmatic pacing program. They all have CCAHS, documented by means of lack of respiratory drive to hypercapnia and normal peripheral nerve and muscle studies. Two patients have associated Hirschsprung's disease. METHODS: The ages of patients at insertion of bilateral phrenic nerve pacemakers were 1, 2, and 5 years. A bilateral axillary thoracotomy in the third interspace was used in two patients, and anterior thoracotomy was used in one for insertion of electrodes on the phrenic nerves. Counterincisions in both flanks were used in all patients for insertion of the receivers in subcutaneous pockets. RESULTS: In all patients pacing was commenced within 1 week of the surgical procedure, because no primary pacemaker failures occurred. One patient has experienced no failure of the equipment and no infectious complications at 4 years. A second patient has had the pacemakers for 6 months without complications. The third patient underwent placement of bilateral pacemakers in 1984. In this patient a 10-year experience has resulted in subcutaneous implant failure on two separate occasions; also the presence of a staphylococcal empyema necessitated the temporary removal of one phrenic nerve electrode for 6 months, with successful reinsertion. All patients now receive mechanical assisted ventilation when sleeping and phrenic nerve pacing when awake. CONCLUSIONS: Pediatric surgeons should be aware of CCAHS because it may be treated with surgically implanted electrodes that allow for pacing of the diaphragm. The technique has an acceptable complication rate, and it can greatly decrease the impact of the disease on the lifestyle and activity of the patient. CCAHS also may be associated with Hirschsprung's disease.

Apnea threshold and breathing rhythmicity in newborn lambs.

This study was designed to determine the effect of the removal of chemical stimuli on breathing rhythmicity in awake newborn lambs; it was also designed to define the chemical threshold below which breathing would stop [arterial PCO2 (PaCO2) apnea threshold]. We used a technique of graded extracorporeal CO2 removal with apneic oxygenation in three groups of animals according to age and carotid body (CB) integrity: < 2 days, CB intact (n = 5); 12 days, CB intact (n = 7); and 12 days, CB denervated (CBD; n = 5). In all animals, whatever their age and CB status, suppression of the chemical drive resulted in sustained apnea. The study, performed at four constant levels of oxygenation (hyperoxia, normoxia, moderate hypoxia, and severe hypoxia), allowed precise determination of the PaCO2 apnea threshold. We found that this PaCO2 apnea threshold depended on the degree of postnatal maturation (it was higher in the younger lambs), the level of arterial oxygenation (it was lowered by hypoxia), and CB status (it was higher in CBD animals). Moreover, we found that the 12-day-old CBD lambs breathe at a level of PaCO2 set close to the point of apnea.(ABSTRACT TRUNCATED AT 250 WORDS)

Intraoperative ultrasound and preoperative localization detects all occult insulinomas; discussion 1025-6.

HYPOTHESIS: Preoperative invasive localization procedures with intraoperative ultrasound (IOUS) can result in successful surgical treatment of occult insulinomas when noninvasive imaging study results are equivocal or negative. DESIGN: Prospective study. SETTING: Tertiary care university hospital. PATIENTS: Thirty-seven consecutive patients with a biochemical diagnosis of insulinoma without multiple endocrine neoplasia (MEN). INTERVENTION: All patients underwent portal venous sampling (PVS) (n = 22) or calcium angiogram (n = 15) followed by surgery with palpation and IOUS (n = 37). MAIN OUTCOME MEASURE: Portal venous sampling, calcium angiogram, palpation, and IOUS were compared for accurate localization of insulinoma. RESULTS: All patients were cured of hypoglycemia after surgery. Portal venous sampling correctly localized tumors in 17 (77%) of 22 patients. Calcium angiogram was correct in 13 (87%) of 15 patients. Palpation identified 24 (65%) of 37 tumors, and IOUS found 35 (95%) of 37 tumors. The 2 tumors missed by IOUS were located in the tail of the pancreas and were resected based on regional localization alone. CONCLUSIONS: Intraoperative ultrasound is the single best localization study, but it will miss some tumors that regional localization can identify. Combining both modalities allowed surgical cure of all insulinomas in our study. Therefore, we recommend both IOUS and regional localization for insulinoma when preoperative imaging studies are equivocal.

Selective arterial embolization for the control of lower gastrointestinal bleeding.

BACKGROUND: Transcatheter embolization is accepted as a safe method for treating acute bleeding from the upper gastrointestinal (GI) tract. Hesitancy persists using this technique below the ligament of Treitz, based on the belief that the risk of intestinal infarction is unacceptably high, despite mounting clinical evidence to the contrary. METHODS: A series of 17 consecutive patients with angiographically demonstrated small intestinal or colonic bleeding was retrospectively reviewed. The success and complication rate of subselective embolization was assessed. RESULTS: Bleeding was stopped in 13 of 14 patients (93%) in whom embolization was possible, and in 13 of 17 patients (76%) where there was an intention to treat. Sufficiently selective catheterization to permit embolization could not be achieved in 3 patients. No clinically apparent bowel infarctions were caused. CONCLUSION: Subselective embolization is a safe treatment option for lower GI bleeding, suitable for many patients and effective in most. Careful technique and a readiness to abandon embolization when a suitable catheter position cannot be achieved are important.

The anterior sagittal approach to the treatment of anorectal malformations: evolution of the Mollard approach.

Mollard's anterior perineal approach has been used for more than 20 years for the treatment of anorectal malformations and has undergone several modifications. The anterior sagittal approach is a simplification of the Mollard approach. It allows a safe dissection and preservation of the puborectalis sling, a clear identification of the external sphincters and other striated muscle fibers caudal to the puborectalis, and prevents inadvertent damage to the nerve supply. The site of the rectourethral (or rectovaginal) fistula, containing the internal sphincter, is preserved for anastomosis with the anoderm. The rectal cul-de-sac is mobilized minimally to come through the puborectalis under some tension, while the anoderm and external sphincters are also brought up under tension to meet these structures, thereby creating a short anal canal more closely resembling the normal anatomy. This approach can be used for most types of anorectal malformations in both sexes, and usually is combined with a transverse suprapubic laparotomy for supralevator anomalies.

Extensive upper aerodigestive tract anomalies in 'VACTERL' Association.

Extensive upper aerodigestive tract anomalies are rare in newborns. When they occur, they are often accompanied by other congenital malformations. We present a case of a newborn who presented to the Montreal (Quebec) Children's Hospital with severe tracheoesophageal abnormalities, as part of vertebral, anal, cardiac, tracheal, esophageal, renal, and limb anomalies (VACTERL) association. In spite of surgical intervention, the patient finally succumbed to other congenital abnormalities. Management of infants with such conditions usually involve complex reconstructive efforts, while prognoses remain poor due to other congenital defects and cardiopulmonary complications.

Biliary access procedure in the management of oriental cholangiohepatitis.

The objective was to review the treatment of oriental cholangiohepatitis using a combined approach of surgical access to the biliary tree with a cutaneous choledochoenteric conduit and interventional radiology to remove intrahepatic stones and dilate biliary strictures. Design was a retrospective case series. Oriental cholangiohepatitis is a condition marked by intrahepatic strictures and extensive formation of pigmented stones leading to recurrent biliary sepsis and hepatic abscesses. It is a common condition in southeast Asia and is seen with increasing frequency in Western populations due to Asian immigration. Ten patients were treated at Tripler Army Medical Center from 1986 to 1994. Tripler is a 500 bed tertiary referral center located in Honolulu, Hawaii, and serves the military community in the Pacific basin as well as beneficiaries of the Trust Territories of Micronesia. Patients underwent cholecystectomy and formation of a Roux-en-Y choledochojejunostomy with a lateral limb that was brought out as a cutaneous stoma. After 4 weeks of healing, the intestinal conduit was used by the interventional radiologist to extract retained stones and dilate strictures using a variety of techniques. This was easily performed under light sedation. After completion of therapy, the stoma was closed and buried subcutaneously. This retains the option for accessing the conduit percutaneously or reopening the stoma if necessary for recurrence. Resolution of symptoms and radiologic clearance of intrahepatic stones, biliary strictures, and hepatic abscesses were the main outcome measures. Eight patients underwent the biliary access procedure and had clearance of stones and strictures after one to 10 interventional sessions. There was no major morbidity associated with treatment. No patient required liver resection, and there was resolution of the hepatic abscesses in all cases. The access procedure could not be completed in one patient due to extensive adhesions; this patient was successfully treated by endoscopic retrograde cannulation of the biliary duct. Another patient was treated by the same method on several occasions and never referred to surgery. A combined approach using surgical access to the biliary tree and interventional radiology offers effective treatment of oriental cholangiohepatitis without the need for hepatic resection.

Percutaneous biopsy of a posterior mediastinal mass through an extrapleural window created with dilute contrast.

Extrapleural administration of dilute contrast was used to facilitate biopsy of a posterior mediastinal mass.

Expandable metallic stents in benign tracheobronchial obstruction.

Expandable metallic stents offer advantages over previously available techniques for treating benign tracheobronchial stenosis or obstruction. Endoluminal stent placement offers a rapid and effective means of opening up narrowed airways, and results in excellent relief of symptoms and improvement in pulmonary function. Because they are delivered in a nonexpanded state using flexible over-wire systems, they can be placed using a flexible bronchoscope and can be located in second-order bronchial branches. Metallic stents have been used to treat benign airway obstruction caused by anastomotic narrowing after lung transplantation, infection, congenital lesions, tracheobronchial malacia, inflammatory conditions including relapsing polychondritis, Wegener granulomatosis, and acquired immunodeficiency syndrome, and external compression from benign mediastinal masses or fibrosis. The stents become epithelialized, which prevents migration and permits ciliary activity to continue. Significant complications can occur, including airway inflammation, stent migration, airway erosion, and stent fracture and collapse, but more serious complications are uncommon. Computed tomography is essential in imaging patients being considered for stent placement, as it allows 1) accurate representation of airway anatomy in three dimensions. 2) measurement of airway diameter, 3) evaluation of airway anatomy distal to a narrowed segment and invisible to bronchoscopy, 4) demonstration of dynamic changes in airway morphologic features during forced exhalation in patients with airway malacia, and 5) demonstration of focal or diffuse air trapping in lung peripheral to the abnormal airway. In patients who have had stent placement, computed tomography is valuable in assessing airway morphologic features and dynamics distal to the stent, and can be valuable in assessing stent dysfunction.

Pediatric surgeons' activities and future plans.

OBJECTIVE: The authors aim was to survey members of the Canadian Association of Paediatric Surgeons (CAPS) on their demographics, practice, and future plans. MATERIALS AND METHODS: A questionnaire was mailed to 86 members. RESULTS: We received 60 questionnaires (70%), with a return rate including 85% men and 15% women. Seventeen percent of the respondents do not have children, 8% did not answer the question pertaining to children, and 8% expect to have more children. Among the 75% of surgeons with children, 16% have reduced the number of hours worked, from 2 hours to more than 1 day per week, for a number of years. Younger surgeons and women are more likely to reduce their work load for their family life. Most surgeons practice in an academic (64%) or a mixed setting (25%), with only 12% involved in private practice. On average, 69% of their time is devoted to patient care; teaching and research each take an average of 10% of the surgeon's time, while 9% of their time is spent on administrative duties. Study respondents work an average of 57 hours per week, and 45 weeks per year. Age significantly influenced the number of hours worked per week, and the number of weeks worked on a yearly basis. Gender and type of practices did not significantly influence the number of hours or weeks worked, whereas location of practice did. Spouse activity also had an impact on the number of hours and weeks worked. When asked about their preference for the next 5 years, 30% of surgeons would opt for a decrease in their level of activity, and 15% wish to retire. Irrespective of the age group or the type of practice, surgeons would prefer to decrease their level of activity. A recurring theme submitted by respondents is the need for increased time for teaching and research and less administrative work. Finally, 60% of surgeons were very satisfied with their work, 27% were satisfied, and 12% were unsatisfied. CONCLUSION: Lifestyle and family commitment have an impact on pediatric surgeons' activity and should be considered when analyzing work force requirements.