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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.

The recent developments in pediatric surgical research as well as new concepts and clinical applications in the specialty are emphasized in this report. There is no attempt to make this review all inclusive. Rather, it focuses on specific areas in which new data or techniques will influence future investigations or the management of patients. EXTRACORPOREAL MEMBRANE OXYGENATION No area has commanded more investigational effort than extracorporeal membrane oxygenation (ECMO). By oculopneumoplethysmography Foglia et al1 assessed the effects of right internal carotid artery ligation on cerebral perfusion in lambs during ECMO. Postnormoxic lambs had good right hemispheric perfusion after right internal carotid artery ligation, and posthypoxic animals showed marked initial depression of ipsilateral hemispheric perfusion which improved progressively and was comparable to the normoxic model at 20 hours. Foglia et al postulated a loss of autoregulation and/or intracranial edema, both of which were reversible. It is known that ECMO is associated with an early increase in inflammatory mediators. Simoni et al2 documented a second peak of complement activation and prostaglandin synthesis after 72 hours of high-flow ECMO. They reported increased levels of endotoxin, free radical activity, and tumor necrosis factor, all markers of systemic sepsis. Early weaning, meticulous technique, and periodic replacement of the ECMO circuit for the extended run are emphasized. Crombleholme and co-workers3 proposed using ECMO as a bridge to transplantation with reduced-size lung grafts. They demonstrated the technical feasibility of the procedure in immature swine recipients. The graft was ventilated preferentially and accepted the entire cardiac output. Ongoing studies by Stolar and Dillon4 demonstrated a biphasic prostanoid response in newborn lambs undergoing ECMO perfusion.

Background Kasai portoenterostomy (KP) remains the initial surgical therapy for biliary atresia (BA). Liver transplantation (LTx) is offered after a failed KP or if KP is not feasible. The timing of LTx in these children is not well established. We attempted to define factors that may help choose the optimal timing for LTx in children with BA managed by a multidisciplinary team including a pediatric surgeon, hepatologist, and liver transplant surgeon. Methods Records of children who underwent LTx for BA at our institution between January 1998 and December 2006 were reviewed. Clinical data such as pre-LTx pediatric end-stage liver disease (PELD) score, location of KP, and outcome were evaluated. Results Seventy one children underwent 77 liver transplants for BA at an average age of 25 months (range, 3-216 months). Sixty-one had a previous KP, 30 at our institution. Ten had LTx without KP. The overall patient survival was 94.4% and overall graft survival was 87% at median follow-up of 58 months (range, 6-111 months). Four patients died, 1 because of vascular thrombosis despite repeat LTx, 1 because of fungal infection after LTx, and 2 because of causes unrelated to LTx. Six children required retransplantation. Living donor liver transplantation was performed in 32 of these children with 91% patient and graft survival. Fifty-three children had a PELD score of 10 or higher with patient and graft survivals of 92% and 86%, respectively. Eighteen children had a PELD score of less than 10 with patient and graft survivals of 100%. For the 30 children who underwent KP at our institution, the median age at LTx was 9 months (range, 3-168 months), and patient and graft survival were both 93%. Conclusions Outcome of LTx for BA is excellent. Children with higher PELD scores (≥10) at LTx may have worse outcome. Children with a PELD score of less than 10 survived with their original grafts. In children with BA, the PELD score should be monitored and may help stratify patients for eventual LTx. When a child with BA is deemed a candidate for LTx, the PELD score should be determined. A PELD score that approaches 10 should trigger discussion of LTx and living donor liver transplantation with the family.

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.

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.

The 5th edition of Swenson's text is a hybrid constructed from contributions from faculty inside and outside the Children's Memorial Hospital (Chicago, Ill). In some measure, the book reflects the philosophy and practice of the editor, John G. Raffensperger, and many chapters carry his imprimatur. Too much of the first section is devoted to generic, obligatory, and somewhat superficial considerations of topics relevant to pediatric surgery. There are notable exceptions. Wherever experts are enlisted, the book is succinct and informative. Dr Nadler's discussion of genetics, the chapters on respiratory distress, and the pharmacologic management of shock provide contemporary insights. There are distracting inconsistencies in style. The scholarly exposition correlating embryogenesis and clinical disease is in stark contrast to the purely descriptive chapter on head and neck immediately preceding it and the following anecdotal consideration of foreign bodies in the gastrointestinal tract. The fundamental pediatric surgical subjects, including gastrointestinal and oncology

RANSPLANTATION OF THE LIVER as an auxiliary organ has intrigued investigators for over a decade. The prime advantages of this procedure are the avoidance of the technically hazardous job of host hepatectomy and immediate and total dependence on homograft function as is required in orthotopic (replacement) transplantation. The functional reserve of the patient’s own liver may also permit survival during impaired homograft function subsequent to rejection crises. In addition, and particularly in children, the handicap of providing a comparable-sized organ is eliminated. The scarcity of donors of appropriate size has imposed severe limitations on the number 01 children able to have orthotopic transplantation. In auxiliary liver transplantation, advantage may be taken of the organ-donating potential of stillborns and newborn infants with anomalies incompatible with life. These features have prompted extensive laboratory investigation of hetero.. topic hepatic transplantation. It is now unequivocably established that portal inflow from the host splanchnic bed must be supplied to the homograft. Lack of such provision results in atrophy of the transplant and its consequent failure to support life. Unfortunately, the penalty for total diversion of portal blood to the graft is involution and impairment of the functional integrity of the native liver. The specific requirements for auxiliary liver transplantation have been, for the most part. worked out in the dog. Anatomically and physiologically the porcine liver more closely resembles that of the human than does the canine livet.‘.’ Furthermore, the privileged immunologic status reportedly3-5 enjoyed by orthotopic porcine liver homografts suggests that the pig would be a more suitable model for investigation of auxiliary transplantation since immunologic rejection might be less of an obscuring factor in subsequent physiologic analysis. Finally, the portal vein in the pig is unique in that separation of the common mesenteric vein from the portal vein is technically feasible. This permits selective redirection of the intestinal and pancreatic venous blood into either the transplant or host liver while the other liver in the dual system is perfused with gastric and splenic venous effluent. Thus, simultaneous, albeit constitutionally different, portal inflow may be provided to both livers. The purpose of this communication is to report our preliminary results with the “split” portalRow model of heterotopic hepatic transplantation in the pig. The specific fac~rorrr thr Surgical Research Lahomtor~. Researzh b‘oundaiion o/ the t‘hddren’,\ Hospital o/ th(, Di.7tric.t of’ Columbia. Washington. D.C and the Division of Pediatric, Stugerv. Departnwnt I)/ .SurServ. L,‘mversii!, of Colorado Medicul Center. Lknvrr. COLI.

It has been suggested that the quantitative estimation of serum alpha-1-fetoprotein may help in distinguishing the neonatal hepatitis syndrome from biliary atresia. We measured the serum AFP concentration in 52 neonates and infants with various hepatobiliary disorders, including neonatal hepatitis syndrome (group I), biliary atresia (group II), and other hepatopathies such as choledochal cyst (group III). The mean serum AFP concentration in patients with neonatal hepatitis was significantly greater than the mean concentration in the other two groups. There was no significant difference between the mean serum AFP concentrations in patients with biliary atresia and in group III patients. Patient age was noted to be an important factor: Serum AFP levels greater than 35 microgram/ml in infants one to four months of age suggpst the diagnosis of neonatal hepatitis syndrome. Serum AFP levels below 10 microgram/ml in infants less than four months of age suggest the diagnosis of biliary atresia or hepatopathies other than neonatal hepatitis. However, the variable and significant overlapping of serum AFP values between 10 and 35 microgram/ml limit the diagnostic value of this test.

Anastomotic revision/resection procedures were carried out in five patients in whom refractory cholangitis jeopardized an apparently favorable surgical outcome after portoenterostomy for biliary atresia. Mobilization and displacement of the liver improves exposure of the porta hepatis. All five patients remain jaundice-free seven months to 4 yr after reoperation.

Hepatobiliary scintigraphy with technetium 99m-labeled p-isopropylacetanilido iminodiacetic acid (99mTc-PIPIDA) was used to evaluate 22 neonates with mixed jaundice. Ten patients were proved to have biliary atresia; ten others were diagnosed as having neonatal hepatitis. In the remaining two, jaundice was secondary to prolonged hyperalimentation. Initial studies in all ten patients with biliary atresia showed no evidence of excretion of the tracer into the intestinal tract. Following three to seven days of oral administration of phenobarbital, repeat studies were performed in six of the ten patients. None showed evidence of excretion. Initial studies of the 12 patients with intrahepatic cholestasis showed definite excretion in five, questionable evidence of excretion in two, and no demonstrable excretion in five. Studies after phenobarbital therapy in five of the seven patients with questionable or no excretion on the initial studies showed definite excretion in four. Only in one patient who had poor hepatic extraction did the phenobarbital therapy not change the scintigraphic pattern. The authors conclude that hepatobiliary scintigraphy with 99mTc-PIPIDA after three to seven days of phenobarbital therapy is a highly accurate test for differentiating biliary atresia from other causes of neonatal jaundice.

Plasma ammonia, arginine, citrulline and urine ammonia, total nitrogen, and orotic acid were measured in shunted and control dogs. The progressive rise in plasma ammonia in the experimental group could not be explained solely by shunt hemodynamics. Similarity of control and experimental urinary nitrogen does not implicate nitrogen load. Urea synthesis was similar in experimental and control liver slices and was augmented by ornithine only in the experimental slices. Additionally, elevated urine orotic acid suggests urea cycle compromise at the ornithine transcarbamylase step.

Fifty-six patients with esophageal atresia and distal tracheoesophageal fistula (Vogt-Gross type C) have been treated at our institution since 1966. The methods of treatment have been individualized according to gestational age, birth weight, pulmonary status, and coexistence of other major anomalies. Three distinct approaches have evolved: Immediate primary repair is reserved for infants weighing more than 2 kilograms who have no major anomalies and satisfactory pulmonary status. Delayed primary repair is used in infants of adequate weight (more than 2 kilograms) who have significant but reversible pulmonary changes, with anticipated recovery within I week, and in those in whom several days are required for evaluation of an associated congenital anomaly; we temporize by upper pouch suction, gastrostomy, and antibiotics. Staged repair is used in premature and severely distressed infants as well as in those with cardiac lesions of surgical priority; staging consists of upper pouch suction, gastrostomy, retropleural division of the fistula, or gastric division, with subsequent transpleural repair in 4 to 8 weeks. The surgical approach to each of these infants has been selected after assessment of all aspects of their clinical condition. Flexibility in treatment, which is predicated upon previously established criteria, seems superior to any single surgical plan.

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.

The surgical aspects of pediatric hepatobiliary disease concern a unique set of diagnoses, disease processes, diagnostic issues, and treatment problems. In this context, this article discusses features of biliary atresia, choledochal cyst, gallbladder disease, and liver abscess in the infant, child, and adolescent.

Hepatobiliary scintigraphy with 99m Tc-IDA derivatives was used to evaluate 40 neonates with mixed jaundice. Fourteen patients proved to have biliary atresia. The remaining 26 patients had intrahepatic cholestasis with patent extrahepatic ducts. Sixteen of the 40 patients underwent examinations without phenobarbital stimulation. Sixteen patients had two examinations, one before and one after 3–7 days of phenobarbital therapy. The remaining 8 patients had their initial examinations after phenobarbital therapy. The results of this study show that administration of phenobarbital in a dose of 5mg/kg/day for at least 5 days prior to the examination enhances and accelerates biliary excretion of IDA compounds and thereby significantly increases the accuracy of 99m Tc-IDA scintigraphy in differentiating extrahepatic biliary atresia from neonatal hepatitis. Its routine use in the evaluation of neonatal jaundice is therefore highly recommended.

The surgical management of infants and children with hepatobiliary disorders has undergone some important advances in recent years. Careful assessment over long periods will be necessary to determine if the new procedures discussed will endow these children with a normal or near-normal life span, free of disease.

Thirty-one patients were treated by portoenterostomy procedures for extrahepatic biliary atresia. In 20, there was unequivocal evidence of bile drainage. Eleven of these 20 were restudied from 3 to 23 mo postoperatively by liver biopsy. The clinical condition of most of these infants was satisfactory. Despite absence of clinical jaundice, normal rose bengal scan, and normal liver function studies, eight showed progressive degrees of hepatic fibrosis when compared to the results of the biopsy taken at the time of portoenterostomy. Two patients showed stable histology and one, after progressing on a second biopsy, had normal liver histology on a third study. The implications of progressive hepatic histopathology despite bile drainage are discussed.

Pediatric surgical research continues to expand along technical avenues while addressing issues of basic science.

of the plasma volume with lowering of the hematocrit. The red cell deficit is replaced later, since stimulation of new red cell production begins well after the whole blood volume has been restored by increase in plasma volume. These responses can be measured in the adult. and an understanding of them provides a rational basis for the diagnosis and treatment of blood loss. In order to assess the magnitude of blood loss and replacement reqlliremchnts in the infant, it has been necessary to rely on uncontrolled clinical observations alone or to extrapolate from experimental data obtained in adult subiects. It is obviously not safe to assume that physiologic responses in the infant and in the adult differ only in magnitlldti or that they are qualitatively unaffected by the processes of maturation. .4s with manv phvsiologic mechanisms which concern the young surgical patient. 110 csp&im&tal c~valuntions of the response to hr,morrhage in the yomng ha1.c been reported in the literature. This report concerns such an evaluation in puppies, The pl~ppy has been srleckd as the experimental animal becauscb abundant hemorrhage data have already been obtained in this and other laboratories “.a in cAzperimc,nts \\-ith the adult dog and are available for comparison . and bc~ause controlled experimental data are not readily obtainable in the hllman infant. T’xperiments in the dog and in man have shoL1.n thcb expansion of the plasma to occur continuously o\‘er a 36 to 18 hour period.:‘,” Diffcrencr~s iri rate, and magnitude of the process in man and in the dog hn\~a heen reported

Summary Conservative treatment for hyperthyroidism achieves permanent cure in only 50% of children. The special circumstances of extended longevity and the exclusion of 131 I therapy modifies the selection of thyroid surgery in this age group. Recurrence of hyperthyroidism can be anticipated in 15% of children having subtotal thyroid resection. Because of the hazards attending reoperation, and since the risks of total thyroidectomy are comparable to those of subtotal thyroidectomy, we have evaluated total thyroidectomy as the initial surgical procedure in eight children with medically intractable Graves' disease. Complications following total thyroidectomy have been minimal and temporary. All children are euthyroid on thyroid extract and have resumed normal activity for age. We present total thyroidectomy as a surgical alternative in the management of Graves' disease in children.

Since Kasai et al1 demonstrated convincingly that biliary atresia was a treatable, and in some instances curable condition, the approach to the jaundiced infant has changed dramatically. Efforts to identify infants with this disease were greatly accelerated as the "hands off" philosophy, predicated on the assumption that attempts to correct biliary atresia would be futile, gave way to a more aggressive approach. Inevitably, this led to a proliferation of diagnostic studies, each attempting to discriminate between intrahepatic cholestasis and extrahepatic atresia of the bile ducts. Many of these studies purported to be helpful are, in fact, of little value. Undoubtedly, this is because biliary atresia is not exclusively a condition afflicting the bile ducts, ultimately resulting in obstruction.

Cervical lymphadenopathy due to atypical mycobacteria was an easily identifiable clinical entity in 45 children. A 3-wk history of lymphadenopathy associated with positive atypical acid-fast mycobacterium skin test is highly suggestive of the diagnosis. Surgical extirpation is recommended for cure. The results of operative excision in 40 children were excellent.

This communication reviews the surgical experience with re-operations performed for correction of extrahepatic biliary atresia upon 22 patients at the Children's Hospital National Medical Center between 1972 and 1978.

The technical limitations of peritoneovenous shunting in small children were overcome by placing the valve in the iliac fossa. Both collecting and venous tubing are readily positioned from this approach. The valve is easily accommodated in this location.

Hepatic (cirrhosis) accounts for a higher percentage of portal hypertension than extrahepatic portal vein obstruction. Some patients with extrahepatic portal hypertension (portal vein obstruction) have a more favorable clinical status. Nonoperative management (balloon tamponade, vasopressin) will usually succeed in controlling bleeding (both groups). Most patients can be stabilized before surgical procedure. Results of devascularization procedures are encouraging. Central splenorenal shunt was most commonly employed (both groups). The Warren shunt is gaining popularity. There are no clear advantages of any particular method of portosystemic shunting in either group. The results of shunt therapy in cirrhotic patients are comparable to those observed in patients with extrahepatic portal hypertension.

Currently, no single area in pediatric surgery arouses more heated controversy than does the surgical treatment of biliary atresia. Because of reports (few have been published) of spontaneous resolution of biliary atresia, only limited operations have been recommended to avoid damage to unrecognized ductal structures. Conversely, because of other reports of correctable lesions "missed" at operation and subsequently discovered at autopsy, a thorough surgical exploration has been urged. The dilemma has not been helped any by communications from Japan about another operation for biliary atresia in which the extrahepatic bile ducts are removed entirely. For a while, most American pediatric surgeons simply ignored the Japanese communications. This action, or lack of action, was subsequently believed fully justified after the initial pessimistic report in the United States by Campbell and associates. 1 After experiencing ten outright failures in ten successive patients, the authors legitimately concluded "... that hepatic portoenterostomy appears

A formal examination is an effective device for assessing certain aspects of a resident's competence. Participation by outside examiners is advisable. Generalizations on the basis of 2 yr of experience may be premature, however, the oral examination, strengthened by outside examiners and complimented by a written clinical problem-solving test, has provided insight into the candidates' abilities and into their progress from the first to the second year. In addition, this process has identified strengths and weaknesses in the training program, which has led to modifications of the surgical curriculum. It is concluded that comprehensive oral and written examination of pediatric surgical trainees at the completion of their first year and again at the end of their second year yields information that is of importance to the trainee and to the faculty.

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.

Differentiating intrahepatic cholestasis from extrahepatic biliary tract obstruction may be difficult. Four patients with intraoperative cholangiographic evidence of extrahepatic ductal atresia who underwent hepatoportoenterostomy are described. All were ultimately shown to have arteriohepatic dysplasia with hypoplastic but patent extrahepatic ductal systems. The difficulty in establishing an accurate diagnosis, hazards associated with hepatoportoenterostomy, and suggestions for evaluation and management are discussed.

We are grateful to Byrne et al for their comments and for their willingness to share their experience using 99mTc- PIPIDA in the evaluation of neonatal jaundice. We are sympathetic to their concern regarding the total number of patients we reported; however, we felt compelled to report our early encouraging results so as to invite comments and to stimulate other groups to use phenobarbital therapy prior to scintigraphy. We have now expanded our prospective study to 40 patients, and the results reinforce our initial conclusion.

Malgre les resultats imparfaits de l'operation de Kasai qui n'assure pas une fonction hepatique satisfaisante dans la moitie des cas, elle doit neanmoins etre pratiquee dans l'attente d'une transplantation hepatique

• This report provides clinical information, diagnostic criteria, management, and outcome of 153 cases of mycobacterial lymphadenitis; 22 patients (14%) had Mycobacterium tuberculosis (TB) and 131 patients (86%) had nontuberculous mycobacterial (NTM) disease. Correct diagnosis of TB v NTM disease is essential, since antituberculous chemotherapy was effective for TB adenitis, while excisional biopsy was the treatment of choice for NTM adenopathy. Dual (PPD-NTM, PPD-T) Mantoux tests discriminated between TB and NTM adenitis in 151 (99%) of 153 patients, while dual (PPD-Battey [B], PPD-T) tests differentiated between NTM and TB adenitis in 135 (88%) of 153 cases. A PPD-T reaction of 1 to 14 mm suggested either an NTM or TB infection, whereas a PPD-T of 15 mm or greater was strongly associated with TB disease. We recommend the use of PPD-B and PPD-T antigens as reliable diagnostic discriminators between TB and NTM adenitis. ( AJDC 1984;138:917-922)