Preface

This issue of Seminars in Pediatric Surgery devoted to hepatobiliary and pancreatic disorders is the third concerning this topic since this publication started 13 years ago. The first, edited by R. Peter Altman, was published in 1992, and the second, edited by Takeshi Miyano, appeared in 2000. The considerable advancements in knowledge, technology, and skills in this area in the course of the last decade justify, the present update.

Pediatric surgeons learned to treat biliary atresia and some forms of familial cholestases rather efficiently but without understanding much about their etiology and pathogenesis. In the last few years, some breakthroughs in the study of these diseases have clarified their molecular origins and the potential role of treatments, particularly surgery. In the article by Dr. Alvarez and coworkers, this knowledge is clearly summarized and updated.

The limits of minimally invasive surgery have been expanded almost daily. In the article by Dr. Martinez Ferro and coworkers, the successful application of laparoscopic procedures to the treatment of biliary atresia and choledocal cyst is described in detail. The advantages of magnification for identifying and handling the liver hilar structures are obvious. Transplantation is ultimately required in most patients with biliary atresia at one moment or another, and the dense and highly vascular postoperative adhesions due to portal hypertension often make recipient hepatectomy a nightmare for the surgeon. The interest and potential advantage of the laparoscopic approach are clearly pointed out in this article.

The treatment of extra-hepatic portal hypertension remains a controversial topic mainly because many pediatric surgeons, and particularly those belonging to groups used to transplant, reduce, or divide even tiny livers and to perform anastomoses in small vessels, do not find it totally justified to maintain a conservative approach at this time. Repeated endoscopic sclerotherapy or waiting until spontaneous shunts develop during adolescence may not be a better alternative than shunting the obstructed portal venous system to the systemic venous system while preserving the spleen and even preserving liver portal flow using mesenterico-left portal shunts. In the article by Professor Gauthier, the different types of shunts and the indications and limits of each one are carefully reviewed and illustrated.

The need for adequate preoperative evaluation of the vascular anatomical map in liver, renal, or cardiovascular diseases prompted the use of the current sophisticated imaging devices, and particularly of magnetic angioresonance, for this purpose. These issues are reviewed by Dr. Fernandez-Cuadrado and coworkers in their article.

Refinements in surgical skills and improvements in chemotherapy together with cooperative trials and protocols allow total removal of hepatoblastomas and (to a lesser extent) hepatocarcinomas in a high proportion of affected children obtaining improved long-time survival. However, the extent of some of these tumors at diagnosis makes removal with clean margins impossible. The introduction of total hepatectomy followed by transplantation has radically changed the former dismal prognosis of this particular group of patients. Survival is achieved in around 80% of patients with resection and chemotherapy, matching those obtained in less extensive tumors. Professor Otte and coworkers contribute an update and analysis of the available experience in this aspect of pediatric surgery.

Finally, Dr. Stringer summarizes the current knowledge on surgical pancreatic disorders. The pancreas is an organ rarely frequented by pediatric surgeons, and the changes in management in the last decade are analyzed in his chapter.

This editor believes that keeping the more sophisticated skills required for the treatment of hepatobiliary and pancreatic diseases within our specialty is mandatory. If the increasing complexity of the surgical procedures in children requiring liver transplantation, pancreatic surgery, or complex laparoscopy is left to surgeons that mainly treat adult patients and are only marginally involved in children’s care, the contents of Pediatric Surgery as a specialty will be seriously affected. If we allow this part of our specialty to be usurped, other conditions like severe liver or pancreatic trauma or tumors may follow.

The size, population, or organization of health care delivery of each country may impose alternative models of managing these complex conditions, but it is imperative for survival of our specialty that we acquire, develop, and expand the skills necessary to treat even the most complex patients. Since the more complex techniques of liver transplantation, like liver reduction or splitting, were developed to a great extent by pediatric surgeons, there is no reason for us not to be responsible for the treatment of all liver and pancreatic surgical disorders in children.