Prenatal detection of BA is a rare situation, found on 20 patients over 15 years in our center (with about 20 pts/year referred for BA). Prenatal signs on ultrasound performed at the second trimester of gestation include:

Great care should be deserved for the prenatal counseling regarding the likelihood of BA, although a complete and early postnatal assessment should be performed before the second week of life.

Infants with BA present with a conjugated jaundice, dark urine, and pale stools. These signs are usually present from birth. Initially the infants feed normally though their weight gain is subnormal due to their inability to digest fat and soluble vitamins. The liver is usually enlarged and firm in older infants (e.g., more than 10 weeks), and there may be ascites and or splenomegaly indicating the onset of portal hypertension along with cirrhosis.

The diagnosis of BA is an operative diagnosis, but is accurate when performed preoperatively in more than 90 % of cases. In our institution, this is achieved by exclusion of various medical causes of conjugated jaundice (Fig. 26.3). Ultrasonography is equivocal of BA if it excludes intrahepatic bile duct dilatation (found in choledochal malformation) and the absence, irregular, or very small gallbladder after a 4 hours long fasting, and the specific triangular cord sign (which corresponds to the fibrotic remnant at the hilum) is found in about 80 % of cases [38] (Fig. 26.4). In our center, ERCP is indicated if there is any doubt regarding the color of stools or the presence of a gallbladder without the triangular cord sign on US. ERCP showing stained bile at the papilla or intrahepatic bile ducts excludes BA [39].

Cholangitis occurs most commonly in the days following primary surgery but can be delayed. It is recorded in about 40–50 % of cases in most series and almost always occurs in children with at least some degree of bile flow, not those with early failure. Clinically, it is characterized by worsening jaundice, fever, and acholic stools. The diagnosis may be confirmed by blood culture, but it is important to treat suspected cases early with broad-spectrum antibiotics effective against gram-negative organisms.

Inguinal hernia, usually occurring in about 10 % of these infants, should be checked before discharge and repaired especially if the child has a successful Kasai [43].

As for all patients presenting with abdominal trauma, ABC should be performed at the arrival in the trauma room [51]. Two intravenous accesses is the rule. At the end of this first assessment, you should be able to know what exam to perform, either a FAST evaluation or a total body CT scan. Based on 136 papers accepted among 900 on pediatric abdominal trauma, Sang-Woo Pak speculated in 2013 that if the clinical exam shows abdominal wall or lower chest bruising, abdominal pain or tenderness, and/or low blood pressure without shock, a FAST scan and a blood work should be performed searching for liver enzyme increase. In case of anomalies, a CT scan can be discussed. If not, the patient can be admitted to the ward for monitoring [52].

The liver has a tremendous capacity of healing, and the success rate has been reported to be between 82 and 100 % [53]. The treatment is mainly guided by the patient’s hemodynamic status more than by the liver injury scale [54]. The initial management should follow the Advanced Trauma Life Support (ATLS) principles of aggressive fluid resuscitation, guided by monitoring of central venous pressure and urinary output [51]. Actually, the only indication of surgery is the need for blood transfusion above 40 ml/kg (half body mass index). Nonoperative management involves admission to a unit and the monitoring of vital signs, with strict bed rest, frequent monitoring of hemoglobin concentration, and repeated abdominal examinations [55]. In case of failure, angio-embolization can be an interesting option. A perfect collaboration between the anesthesiologist, the interventional radiologist, and the surgeon is therefore crucial. Angio-embolization can be necessary either in a high-grade liver injury (grade IV and V) associated to unstable hemodynamic or after a first surgical procedure such as a packing when the patient remains unstable [56]. Success has been reported to be between 68 and 87 % [56]. Complications can be related to the nonoperative management (liver hematoma, false aneurysm, biloma, or biliary ascites) or to angio-embolization (hepatic necrosis, liver abscess). Incidence increases accordingly with the grade of injury. In a series of 337 patients with liver injury grades III–V treated nonoperatively, those with grade III had a complication rate of 1 %, grade IV 21 %, and grade V 63 % [57]. Patients with grades IV and V injuries are more likely to require surgery and to develop complications of nonoperative treatment.

If the patient requires surgery because of instability, laparoscopic approach has no place. Through a midline incision, after a complete exploration of the abdominal cavity, the surgeon should be able to see the liver fracture and starts by packing with surgical dress. Indeed, severe coagulopathy, acidosis, and hypothermia can occur in case of important hemorrhage and make the resuscitation challenging to save the patient. Initial control of hemorrhage and contamination followed by packing and temporary abdominal closure, ICU restoration of normal physiology, and delayed definitive repair is also well known as “the damage control” technic. The group at the University of Pennsylvania has popularized this technic in 1993 [58]. If the patient remains unstable, an attempt to see where the bleeding comes from should be done carefully, especially in case of hepatic vein rupture. Indeed, if the surgeon tries to bring down the liver with his hand, the risk of aggravating the hepatic vein lesion is major. The next step would be the Pringle maneuver, which consists in clamping the whole hepatic pedicle. This maneuver is controversial, but it can be maintained up to 1 h without compromising the blood supply to the liver. Despite this, if the patient continues to be unstable, the next step is to clamp the inferior vena cava. After this, surgeon should consider performing a hepatic resection in case of continuous bleeding or repair a hepatic vein or vena cava injury.

The treatment of a bile leakage is most of the time a conservative treatment. A simple drainage of the ascites or a cholecystostomy can be done if the leak is heavy with a considerable amount of ascites. Stenting the bile duct through radiologist intervention is another interesting and valid option [59].

For isolated liver injury, a guideline with the number of hospitalization days has been published depending on the grade of the injury from 2 to 5 days for grade I to grade IV [60]. An even shorter hospital stay has been also reported with a discharge at day 1 or 2 after the trauma [61, 63]. Considering the risk of secondary bleeding, it could be safer to keep the patient to the hospital longer especially in case of high-grade liver injury.

Hepatoblastoma (HBL) is the most common malignant hepatic tumor in children typically seen in the first 3 years of life. Incidence is about 1.2–1.5 cases per million [62]. Those that occur after 5 years of age, formerly called transitional hepatocellular carcinoma (HCC), tend to have a worse prognosis, probably because their behavior is similar to a HCC [63]. Most HBLs do not have an abnormal genetic background, but predisposing factors include trisomy 18, Beckwith-Wiedemann syndrome, familial adenomatous polyposis, and prematurity [64].

The most common presentation is an isolated abdominal mass. As some HBL found to have an HCG excretion, mild signs of virilization should be looked for. They rarely present signs of rupture, i.e., acute abdominal pain with guarding or contracture.

The chemotherapy of hepatoblastoma is based on platin family and anthracyclines. Neoadjuvant chemotherapy has been administered to all patients in SIOPEL protocols as compared to the North American protocol, although this may change in the future. The type and frequency of drug administration are reported in Fig. 26.7, as well as the timing of surgery.

Careful preoperative assessment is mandatory before surgery. Patients should be referred to a radiologist and a surgeon with expertise in liver resection and imaging. Early referral after diagnosis improves surgery planning. After the chemotherapy, preoperative assessment includes CT scan and post-neoadjuvant chemotherapy (POSTEXT) evaluation coupled with operative or preoperative Doppler ultrasound to carefully identify the limits of the tumor with the main hepatic vessels. α-FP decrease should be monitored and documented. Echocardiography is mandatory in order to assess both anthracycline-related toxicity and the tolerance to a potential hepatic vascular exclusion. For these large tumors with a poor prognosis in case of local relapse, both minimally invasive surgery and wedged surgical excision are not recommended. Transverse or subcostal laparotomy is performed. The abdominal cavity is inspected. The hepatic pedicle is encircled by a tape in prevision of a Pringle maneuver, triangular ligaments are sectioned, and total vascular exclusion of the liver is prepared. Anatomic resection of the liver is performed via vascular ligature of the inflow to the involved part of the liver. Transection is done with the help of either thermic or ultrasound sealing devices. The aim is complete microscopic resection of the tumor with minimum blood loss and avoiding air embolism via the main hepatic veins. This is why, in our experience, we perform a brief total vascular exclusion of the liver (i.e., less than 30 min) when the tumor borders are in the vicinity of the hepatic veins. Morbidity of this procedure is low given that the remaining liver parenchyma is healthy.