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Between June 1990 and September 1995, 8 of 24 children with cystic fibrosis (CF) who were accepted either for combined transplantation or isolated liver transplantation died while waiting for a graft; 11 underwent transplantation and 5 are currently on the waiting list. Of the 11 children who had surgery, 7 (group 1) underwent one of the following procedures: heart-lung-liver (n = 4), sequential double lung-liver (n = 2), or bilateral lobar lung from a split left lung and reduced liver (n = 1). During the same period, the four other children (group 2) underwent isolated liver transplantation (three full-size livers, one partial liver). There was one perioperative death in each group. Pulmonary infection was the most common cause of morbidity in group 1. Other complications in group 1 included tracheobronchial stenosis (n = 2), biliary stricture (n = 2), and severe ascites (n = 2). All were successfully treated. Obliterative bronchiolitis developed in three patients. This was treated with FK 506. In group 2, pulmonary function tests improved or remained stable after liver transplantation. Surgical complications in group 2 included severe ascites (n = 1), biliary stricture (n = 1), and abscess of the liver (n = 1). Actuarial survival was 85.7% +/- 2% in group 1 at 1 year; it remained unchanged at 3 years and was 64.2% at 5 years.

Individualization of the segment as the functional anatomical unit of the liver permits the performance of surgical segmentectomies. These segmental resections are “reglees” because the plane of cleavage of the hepatic parenchyma follows the anatomical scissurae. From the technical point of view, liver segmentectomies are characterized by an exclusive transparenchymatous approach to the vascular pedicles of the segment to be removed. We have performed this type of surgery in 22 patients with no mortality. These operations are indicated in: (1) some benign tumors; (2) some liver trauma; (3) biliary operations above the hilus, where anterior resection of segment IV can be necessary; and (4) carcinomas of the gallbladder discovered histologically after cholecystectomy. Liver segmentectomies can also be indicated for malignant tumors when the liver is cirrhotic, or when an extended resection is likely to expose the patient to the risk of liver failure. Indeed, one of the main advantages of liver segmentectomies is that they permit an economical but safe surgical resection of the hepatic parenchyma.