A multivariable risk factor analysis of the portoenterostomy (Kasai) procedure for biliary atresia: twenty-five years of experience from two centers.

OBJECTIVE: The authors investigated risk factors for failure after portoenterostomy for biliary atresia using univariate and multivariable methods. SUMMARY BACKGROUND DATA: Kasai's portoenterostomy has gained worldwide acceptance as the initial surgical therapy for infants with biliary atresia. Although extended survival has been achieved for many patients, factors influencing outcome have not been defined clearly. METHODS: The authors analyzed risks for failure in 266 patients treated from 1972 to 1996 by the Kaplan-Meier product limit estimate and Cox proportional hazards model. Failure was defined as death or transplant. RESULTS: Age at surgery, surgical decade, and anatomy of atretic bile ducts were identified as independent risk factors. Five-year survival was 49% and median survival was 15 years when bile drainage was achieved. Sixty-five patients had liver transplants. Mean age at transplant was 5.4 years. CONCLUSIONS: The outcome after portoenterostomy for biliary atresia is determined by age at surgery and anatomy of the atretic extrahepatic bile ducts. Liver transplant will salvage patients with failed Kasai with 10-year posttransplant survival of 71%.

Biliary atresia.

Biliary atresia, a progressive obliterative process involving the bile ducts, has its onset in the newborn period. It is characterized by worsening cholestasis, hepatic fibrosis, and cirrhosis, which lead to portal hypertension and a decline in hepatic synthetic function. Untreated, the outcome is uniformly fatal. The two major milestones toward improved treatment of this disease have been the Kasai portoenterostomy and orthotopic liver transplantation. There has been discussion regarding transplantation as primary therapy, but portoenterostomy remains the standard of care as first-line intervention. Hepatic transplantation, done more frequently for biliary atresia than for any other cause of liver failure in the pediatric population, offers improved survival and quality of life to those for whom the Kasai operation fails. The etiology of biliary atresia remains poorly understood. Working toward a better understanding of this disease, recent investigations target more precise characterization of the hepatic pathology and seek to identify possible causative agents and predictors of favorable outcome. Recent advances in the understanding of biliary atresia published between December 1995 and November 1996 are the focus of this review.

Update on the analysis of the need for pediatric surgeons in the United States.

UNLABELLED: Accurate estimations of pediatric surgical manpower needs are necessary if this specialty is to avoid the consequences of under- or oversupply, and reasonable decisions must be made relative to the number of training programs needed. METHODS: Fifteen, 10, and 5 years ago, pediatric surgeons (PSs) in 62 standard metropolitan statistical areas (SMSAs) having a population of at least 200,000 were asked to estimate the number of PSs needed in their localities. A computer program analogous to the SOSSUS program was designed to project the number of PSs that would result from various numbers of trainee graduates per year. The program has been updated for comparison. Known input data included the present number and age of PSs, age range of trainees, current US population projections to the year 2025, and the average retirement age. RESULTS: These PSs estimated that 88 additional PSs are needed in the next 10 years. Currently, 26 programs in the United States graduate an average of 24 trainees per year, and six programs in Canada graduate six trainees per year. The previous projection indicated that 20 trainees per year would result in 525 PSs in 1993, and the actual number is 559; so the figures indicate that 27 or 28 PSs are entering practice each year. The apparent increase in numbers is related to entry of Canadian trainees primarily, and a few others, into practice. The current computer projection indicates that 20 graduate trainees per year would result in an absolute increase of 0.55% per year, and 25 per year would result in an increase of 1.43% per year, to 2020, while the increases in the US population would be 1.02% per year for all ages and 0.52% for 0 to 15 year olds. If all programs currently being considered for approval are certified, as many as 36 trainees per year--or 7 times the rate of the 0-15-year population increase--will result. CONCLUSION: Although an average of 20 graduates per year entering practice would keep pace with the pediatric population, 25 to 27 graduates per year--or 3.5 to 4 times the rate of the 0- to 15-year population increase--can be accommodated now into the current system of delivery of pediatric surgical care on the basis of estimated need. Many more graduates than this would create an excess of surgeons before long.

Cervical lymphadenitis in infants and children.

One of the more common cervical lesions seen in children is cervical lymphadenitis. Pediatricians handle the vast majority of these children who present with an acute infection. The pediatric surgeon becomes involved only when the presumptive infection does not respond to antibiotic therapy, or if the lump becomes chronic and persists for weeks or months. In this situation the specter of malignancy looms, but benign causes still predominate. The infectious agents range from common bacteria such as staphylococci and hematologic staphylococci to atypical mycobacterium, fungi, and other much less common organisms. A broad discussion of the numerous causes of cervical lymphadenitis is presented together with a pragmatic diagnostic and therapeutic approach.

The jaundiced newborn.

Evaluation of the newborn with hyperbilirubinemia is an important and commonly encountered problem in all clinical pediatric-based practices. Cholestatic jaundice is always pathologic and warrants prompt assessment. The timely evaluation and directed treatment of any jaundiced newborn are essential to prevent serious metabolic consequences and irreversible hepatic damage.

Bladder management in children with genitourinary sarcoma.

Between 1977 and 1991, 14 patients were treated for genitourinary sarcoma (mean age, 7.4 years). The primary site was bladder in six patients, prostate in five, and vagina in three. Histological study showed embryonal rhabdomyosarcoma in 12 and leiomyosarcoma in 2 children. Initial therapy included biopsy followed by chemotherapy in all patients. Subsequently, five patients had anterior exenteration, four underwent partial cystectomy, and one patient had a radical prostatectomy; four patients were treated with chemotherapy and external beam pelvic irradiation (4,000 to 6,000 rads) alone. Overall survival for the group is 100% (follow-up 6 to 168 months). Two of four patients undergoing partial cystectomy had bladder augmentation at the time of surgery. All patients having partial cystectomy had negative surgical margins, are tumor free, and have volitional voiding. Two of four patients (50%) initially treated with chemotherapy and radiotherapy alone have had significant bladder deterioration requiring bladder reconstruction. There has been an evolution toward less radical, initial surgical intervention in pediatric genitourinary sarcoma; however, surgical resection continues to be the primary curative modality. Partial cystectomy with or without primary reconstruction may be preferable to exenteration for selected patients. Primary reconstruction at the time of partial cystectomy leaves a functional bladder and excellent long-term results. Children treated with chemotherapy and radiotherapy protocols alone must be monitored for late bladder deterioration.

Contemporary management of congenital diaphragmatic hernia.

Despite advances in infant transport and intensive care, congenital diaphragmatic hernia (CDH) still poses a serious threat to life during the neonatal period. Only with the introduction of extracorporeal membrane oxygenation (ECMO) has mortality declined significantly. Understanding the pathophysiology of CDH and its devastating effect on the lungs' bronchial and vascular development assists the clinician in rational management during the perioperative period. Successful treatment demands prompt diagnosis, informed preoperative assessment and preparation, carefully timed surgical repair, and aggressive postoperative intensive care with a thorough understanding of the role of ECMO. Improvements in ventilatory support and pharmacologic management of pulmonary hypertension have allowed surgeons to delay repair until circulatory hemodynamics, electrolytes, and oxygenation can be optimized, even resorting to preoperative ECMO in selected cases if needed. Patients with severe bilateral pulmonary hypoplasia pose serious dilemmas in terms of selection for ECMO, as well as for surgical repair. Long-term follow-up of patients after CDH repair reveals persistent hypoperfusion of the involved lung but no debilitating pulmonary disease. Future therapies may include early fetal intervention or lung transplantation, although these areas remain controversial and experimental, and clinical experience is limited.

Choledochal cyst.

The most commonly encountered variant of choledochal cyst (type I) is characterized by fusiform dilatation of the bile duct. Patients usually come to medical attention within the first decade of life. The diagnosis is readily established by ultrasound or computed tomography. Choledochal cyst has been diagnosed in the fetus by maternal ultrasound. Treatment is by surgical excision.

Three pediatric patients with extension of prostatic embryonal rhabdomyosarcoma anterior to the bladder into the space of Retzius.

Invasive embryonal rhabdomyosarcoma of the prostate often extends superiorly into the bladder base requiring partial cystectomy as part of excision of the primary tumor. Three patients were studied with ultrasonography CT and MRI and showed the extension of the tumor superiorly and anterior to the bladder in the prevesical space of Retzius. Complete excision with bladder sparing was successful in two of the patients. The imaging of pelvic tumors in children (although well studied with ultrasonography, CT) is helped by MRI in the sagittal plane and/or lateral films of intravenous pyelograms and cystograms to establish the relation of the tumor to the bladder.

Acquired coarctation of the aorta.

Coarctation of the aorta is usually caused by a congenital narrowing of the aorta. This report describes two children who developed hypertension secondary to an acquired coarctation of the aorta. In one patient the coarctation was temporally related to umbilical artery catheterization and was associated with thrombosis and aneurysmal dilatation of the aorta. In the second patient, the coarctation occurred after surgical aortotomy during the removal of an intrathoracic neuroblastoma. Patients who have interventional damage to the aorta should be periodically examined for the appearance of a coarctation. Although an acquired coarctation of the aorta is an infrequent complication of invasive or surgical procedures, it should be identified since it represents a remediable cause of hypertension in children.

Obstruction of the Roux limb after portoenterostomy for biliary atresia: a delayed complication.

We report the case of a 5-year-old girl who underwent a Kasai portoenterostomy for extrahepatic biliary atresia. The conduit was exteriorized until 11 months of age. She was doing well, with stable portal hypertension until she suddenly developed jaundice, acholic stools, and bacteremia not responsive to a course of steroids and intravenous antibiotics. Suspecting obstruction at the site of the previously exteriorized anastomosis, a percutaneous cannulation of the conduit was performed. Catheterization of the conduit obstruction unkinked it and reestablished bile flow. She has remained anicteric with stable liver function.

Right pneumonectomy syndrome: a lethal complication of lung resection in a newborn with cystic adenomatoid malformation.

A newborn girl underwent a right pneumonectomy for cystic adenomatoid malformation. She subsequently developed compression of the trachea and left mainstem bronchus by a combination of aortic arch, pulmonary artery, and ductus arteriosus. She died while planning aortic suspension, and the anatomic relationships were confirmed by autopsy. This problem is potentially treatable by a variety of means.

Biliary atresia and noncardiac polysplenic syndrome: US and surgical considerations.

Ten percent of children with biliary atresia have an associated complex of anomalies, including polysplenia, azygous continuation of the inferior vena cava, preduodenal portal vein, hepatic arterial anomalies, and bilaterally bilobed lungs. These abnormalities will not be detected if the preoperative workup is limited to hepatobiliary nuclear scanning. Ultrasonography is important in the preoperative evaluation of patients suspected of having biliary atresia. It is important to identify the associated abnormalities preoperatively because they have an impact on the initial portoenterostomy and may preclude subsequent orthotopic liver transplantation.

Delayed diagnosis of esophageal perforation by aluminum foreign bodies.

Two pediatric patients presented with long-term respiratory or gastrointestinal complaints after swallowing aluminum poptops. The delay in diagnosis was due in part to poor appreciation of the relative radiolucency of aluminum.

Potential errors in the diagnosis and surgical management of neonatal jaundice.

Despite advances and refinements in diagnostic techniques, distinguishing between cholestatic syndromes and extrahepatic biliary atresia is not always possible. Because there are inherent errors in all diagnostic studies including nuclear scanning, ultrasonography, and liver biopsy some infants presumed to have biliary atresia will come to surgery when, in fact, the bile ducts are partially or completely patent. The decision to proceed with a portoenterostomy or to terminate the procedure depends upon the appearance of the liver and histology of the biopsy.