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Biomedical subjects

J M Laberge

Publications and source records attributed to J M Laberge.

At least 55 records · Page 3Linked to original sources

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.

Anastomosis, Roux-en-Y↗

Vascular complications after pediatric liver transplantation.

From February 1986 to July 1994, 81 hepatic transplantations were performed in 73 children, with an overall patient survival rate of 83%. Forty-two patients received whole-liver grafts (WLG) and 39 had reduced-size grafts (RSG). The mean patient weight was 19.7 kg, with 29 patients weighing less than 10 kg. Seventeen vascular complications (21%) occurred in 13 children: 8 (10%) had hepatic artery thrombosis (HAT), 5 (6%) had portal vein thrombosis (PVT), 1 had both HAT and PVT (1%), and 3 (4%) had aortic conduit perforation (ACP). There was no significant difference in the incidence of HAT between RSG (5%) and WLG (14%) or between children weighing less than 10 kg (10%) and those weighing more than 10 kg (10%). The site of arterial reconstruction, end-to-end to the recipient common hepatic artery or end-to-side to the infrarenal aorta, had no significant effect on the occurrence of HAT (7% v 8%), but HAT occurred in 2 of 6 cases (33%) in which an aortic conduit was used. PVT documented in 5 cases (6%) was associated with technical complications (2), preduodenal portal vein (2), and a circulating cardiolipid antibody (1), and required thrombectomy, with no graft loss. Combined HAT and PVT was found in one patient 2 years postretransplantation for HAT. Although graft function is normal, portal hypertension persists. The aortic conduit, used in six patients, led to arterial perforation (3), HAT (2), and death (2). Of the 8 cases of HAT, 1 was diagnosed during autopsy and 7 occurred within 30 days and required retransplantation (6) or thrombectomy with rearterialization (1).(ABSTRACT TRUNCATED AT 250 WORDS)

Anastomosis, Surgical↗

Reversible tracheal obstruction in the fetal sheep: effects on tracheal fluid pressure and lung growth.

Congenital diaphragmatic hernia (CDH) continues to carry high morbidity and mortality. A number of treatment modalities including extracorporeal membrane oxygenation and in utero repair have improved the mortality rate only minimally. With this condition, there is often insufficient lung mass at birth and persistent pulmonary hypertension postnatally. Experimental fetal tracheal ligation has been shown to increase lung growth in utero and to reduce the hernial contents in CDH. The purpose of this study was to determine the effect of reversible fetal tracheal occlusion on tracheal pressure and lung development. Nine fetal sheep were divided into two groups. Group 1 had intratracheal balloons placed, and the balloons were left inflated for 21 to 28 days. Group 2 consisted of littermates that served as controls. They either had uninflated balloons placed or were left unoperated. Tracheal pressure measurements were recorded periodically, and the amniotic fluid pressure served as a reference. The animals were killed near term, and the lungs, heart, and liver were weighed and corrected for body weight. Standard morphometry was used to compare the lungs further, and the lung DNA and protein content were measured. Tracheal damage from the balloon catheter also was assessed. The tracheal pressure was 3.85 (+/- .49 SE) mm Hg in experimental animals, and it averaged -0.27 (+/- .27 SE) mm Hg in controls (P < .0001). Tracheal occlusion increased lung weight and volume by two to three times (P < .0001 and P < .0006, respectively) while heart and liver weights remained similar to those of controls.(ABSTRACT TRUNCATED AT 250 WORDS)

Amniotic Fluid↗

Timing of repair of congenital diaphragmatic hernia requiring extracorporeal membrane oxygenation support.

Treatment of congenital diaphragmatic hernia (CDH) has undergone a revolutionary change in philosophy, from previous urgent repair to the present practice of stabilization and delayed repair. However, when extracorporeal membrane oxygenation (ECMO) is required, many people believe that the risk of postoperative pulmonary hypertension (PPHN) mandates hernia repair while on ECMO. This report details the experience in two ECMO centers with stabilization, ECMO if required, and CDH repair post-ECMO. All CDH patients symptomatic in the first hour of life with a gestational age of at least 34 weeks during the period were reviewed retrospectively. Standard criteria were used to select patients for ECMO. High-frequency jet or oscillating ventilators and nitric oxide were not routinely available throughout the study period, but were used in some of the more recent patients. A total of 60 patients presented to the two centers; 24 cases were stabilized with conventional management, repair of the CDH was done elective, and survival was 100%. Eight patients were referred after having repair elsewhere; six survived (75%). The two deaths were attributable to associated lethal lesions--complex cyanotic heart disease and alveolar capillary dysplasia. Eight patients who required ECMO were managed with the intention of repairing the defect on ECMO. Four survived (50%). Two patients died before repair. Twenty patients were managed with ECMO, with the intention of repairing the defect after decannulation. Overall survival was 13 (65%), deaths were caused by pre-ECMO hypoxia, pulmonary insufficiency, and associated cardiac disease. No patient had recurrent pulmonary hypertension after late repair.(ABSTRACT TRUNCATED AT 250 WORDS)

Capillaries↗

Repair of congenital diaphragmatic hernia after weaning from extracorporeal membrane oxygenation.

Stabilization and delayed operation for patients with congenital diaphragmatic hernia (CDH) is now widely accepted. When preoperative extracorporeal membrane oxygenation (ECMO) is needed, most centers have CDH repaired on ECMO to minimize the risk of postoperative deterioration. The authors adopted a policy of weaning from ECMO before repair in an effort to avoid hemorrhagic risks. They reviewed their experience with CDH patients who required ECMO for stabilization before repair but for whom post-ECMO repair was planned. The records of all high-risk CDH patients with a gestational age of at least 34 weeks were reviewed. Eighteen patients were identified. None of the eight who were stabilized and operated on without ECMO required bypass postoperatively; all survived. Ten were placed on bypass, nine for stabilization before repair. Of the nine, seven (78%) were weaned from ECMO to conventional ventilation. Repair of the diaphragmatic defect was performed an average of 3.8 days later; none of these patients had severe pulmonary hypertension postoperatively, and all survived. Two could not be weaned before repair, one of whom had a complex congenital heart defect. This patient died. The other patient had repair on ECMO because of intrathoracic gastric volvulus. Severe blood loss prompted decannulation, and the patient died. One patient who was placed on bypass was transferred 10 days after having had repair elsewhere (at 4 hours of age). Pulmonary hypertension did not resolve, and the postmortem examination showed alveolar capillary dysplasia, with focal misalignment of the pulmonary vessels.(ABSTRACT TRUNCATED AT 250 WORDS)

Extracorporeal Membrane Oxygenation↗

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.

Artificial Organs↗

Reoperation for cysts of the thyroglossal duct.

OBJECTIVE: To determine the causes of recurrence of thyroglossal duct cysts (TDCs). DESIGN: A case series. SETTING: A university-affiliated children's hospital. PATIENTS: Between 1974 and 1990, 121 children were treated for TDCs. They ranged in age from 1 month to 18 years. Of these 121 children, 23 had recurrent TDC. INTERVENTIONS: Simple aspiration of the cyst, incision and drainage, cystectomy without hyoidectomy and the Sistrunk operation. MAIN OUTCOME MEASURES: Initial procedures that led to recurrence and procedures that resulted in cure. RESULTS: Six children had recurrence of TDCs after simple cystectomy without hyoidectomy; all were cured by the Sistrunk operation. Fourteen children with infected cysts initially underwent a variety of procedures with recurrence and were eventually managed by the Sistrunk operation. TDCs recurred in eight of these. Three children without infected cysts had recurrence after the Sistrunk operation. Of 34 TDCs infected initially, 40% recurred, whereas of 87 TDCs that were not infected initially, only 8% recurred. CONCLUSION: Simple cystectomy without hyoidectomy and pre-existing infection are the main causes of recurrence of TDCs.

Adolescent↗

Sequential sac ligation for giant omphalocele.

The authors describe a technique for the management of giant omphalocele in which the sac is not resected, but used to sequentially reduce the contents into the abdominal cavity. Three patients were treated in this fashion. Delayed primary fascial closure was achieved in two. The third patient did not have complete reduction after 3 weeks, and temporary placement of a silastic sheet was required. The sac remained intact in all three cases, and neither infection nor injury to the abdominal contents occurred. This technique has the advantages of maintaining an intact membrane and avoiding the initial operative placement of a prosthetic silo in these sometimes critically ill neonates.

Female↗

Aortoesophageal fistula: congenital and acquired causes.

Aortoesophageal fistula (AEF) is a rare but frequently lethal cause of upper gastrointestinal bleeding. This is a report of the authors' experience with four cases--two from congenital and two from acquired causes. A review of the pediatric cases reported in the literature is included. The mortality from AEF is high, and can be decreased in two ways. AEF can be prevented by avoiding prolonged nasogastric intubation in patients with known vascular rings, and by promptly removing esophageal foreign bodies and promptly treating periesophageal abscesses. Once an AEF occurs, it presents with typical large-herald upper gastrointestinal bleeding of bright red blood, and cardiovascular collapse. Recognition of this pattern, with vigorous resuscitation and aggressive surgical management, will improve the survival rate.

Aortic Diseases↗

Gastroesophageal reflux associated with large diaphragmatic hernias.

Extracorporeal membrane oxygenation (ECMO) support has improved the outlook for some infants who have large diaphragmatic hernias (CDH). This has resulted in a subset of survivors of CDH with typically larger defects, more severe pulmonary hypoplasia, and more associated pathologies. This report describes the authors' experience with gastroesophageal reflux in patients with large diaphragmatic hernias who require ECMO. Contrary to previous reports, this reflux was severe and intractable. There appears to be a component of associated gastric dysmotility. In treating this reflux, medical therapy and anterior fundoplication were not successful, and Nissen fundoplication combined with pyloroplasty was required to control reflux and to allow gastric feeding. On the basis of the authors' experience, it is recommended that patients who have gastroesophageal reflux after CDH repair, for whom medical management has failed, be managed aggressively by surgery, with early Nissen fundoplication, and pyloroplasty and insertion of a gastric feeding tube.

Animals↗

Biliary tract complications in pediatric orthotopic liver transplantation.

Biliary tract complications are reported in 15% to 30% of orthotopic liver transplantations (OLTs). Since 1986, 53 OLTs were done in 48 children with a mean age and weight of 5.3 years and 18.9 kg, respectively. Twenty-seven transplantations (51%) were reduced liver grafts (RLG) and 26 (49%) were whole liver grafts (WLG). Since 1988, 70% of transplantations have been RLG. Choledochocholedochostomy (mean weight, 25 kg) with a T-tube (CC) or choledochojejunostomy (CJ) (mean weight, 14.5 kg) were done in 24 (45%) and 29 (55%) cases, respectively. The overall mortality was 19% but none of the deaths were related to biliary problems. There were 13 biliary tract complications (24.5%) in 11 patients including 7 leaks, 5 obstructions, and 1 intrahepatic biloma. Leaks leading to bile peritonitis were managed with simple suture and drainage and were related to the T-tube (4), to the Roux-en-Y loop (2), and to the transection margin of a RLG (1). Obstruction was documented in 5 cases with none associated with hepatic artery thrombosis (HAT). Stenosis after CC reconstruction (2) required conversion to CJ. Two patients had revision of CJ because of kinking of the common bile duct after a left lateral segment graft and an anastomotic stricture 46 months after OLT. The last patient developed a vanishing bile duct syndrome 4 months posttransplant and is awaiting retransplantation. One patient had multiple episodes of cholangitis after HAT and was retransplanted. Neither the type of grafts (RLG 25.9% v WLG 23.1%) nor the type of biliary reconstruction (CC 25% v CJ 24%) influenced the rate of biliary complications.(ABSTRACT TRUNCATED AT 250 WORDS)

Amino Acid Metabolism, Inborn Errors↗

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.

Adolescent↗

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.

Adolescent↗