Chiara Grimaldi

Long-term outcome of home parenteral nutrition in patients with ultra-short bowel syndrome.

Objective: The patients with ultra-short bowel syndrome (U-SBS) have been considered potential candidates for a preemptive/rehabilitative intestinal transplantation owing to the high risk of death from the underlying disease. We hypothesized that children with U-SBS, in the absence of intestinal failure-associated liver disease (IFALD), could also have a good rate of survival on home parenteral nutrition (HPN). Methods: A prospective database from the “Bambino Gesù” Artificial Nutrition and Intestinal Failure Program was used to evaluate outcomes and morbidities of consecutive patients with ⩽10 cm of small bowel enrolled since 2000. Results: Eleven patients were identified with a median bowel length of 7.5 (3–9) cm. Eight patients developed IFALD, which reversed in 7 of them; the IFALD progressively worsened in 1 patient until death. One patient underwent isolated intestinal transplantation and 1 patient is no longer receiving parenteral nutrition (PN) and both are fully enterally fed. The other patients remained at least partially dependent on HPN. The number of days of inpatient care decreased in all of the patients except for the 1 who had repeated episodes of central line infections. Conclusions: The survival of patients with U-SBS receiving HPN was good. Although IFALD was frequent, it had been manageable in most of the patients, but in a single complex case, it led to death. The multidisciplinary management warranted to these patients to approach the school age, to grow, and to maintain the oral intake. Patients with U-SBS are rare, and to better understand their long-term survival, further studies, including more large patient populations, are required.

Intestinal transplantation for total intestinal aganglionosis: a series of 12 consecutive children

BACKGROUND Management of patients with total intestinal aganglionosis (TIA) is a medical challenge because of their dependency on parenteral nutrition (PN). Intestinal transplantation (ITx) represents the only alternative treatment for patients with irreversible intestinal failure for achieving intestinal autonomy. METHODS Among 66 patients who underwent ITx in our center, 12 had TIA. They received either isolated ITx (n = 4) or liver-ITx (LITx, n = 8) after 10 to 144 months of total PN. All grafts included the right colon. RESULTS After a median follow-up of 57 months, the survival rate was 62.5% in the LITx group and 100% in the ITx patients. The graft survival rate was 62.5% in the LITx group and 75% in the ITx group. All the surviving patients were fully weaned from total PN, after a median of 57 days. Pull through of the colon allograft was carried out in all patients. Fecal continence is normal in all but one of the surviving children. CONCLUSION These results suggest that ITx with colon grafting should be the preferred therapeutic option in TIA. Early referral to a transplantation center after diagnosis of TIA is critical to prevent PN-related cirrhosis and thereby to permit ITx, which is associated with a good survival rate.

Meso-Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives

de Ville de Goyet J, Lo Zupone C, Grimaldi C, D’Ambrosio G, Candusso M, Torre G, Monti L. Meso‐Rex bypass as an alternative technique for portal vein reconstruction at or after liver transplantation in children: Review and perspectives.

Surgical management of portal hypertension in children

The management of children with portal hypertension has dramatically changed during the past decade, with an improvement in outcome. This has been achieved by improved efficiency of endoscopic variceal control and the success of liver transplantation. Emergency surgical shunt procedures are rarely required, with acute bleeding episodes generally controlled endoscopically or, occasionally in adults, by interventional radiological procedures. Portosystemic shunts may be considered as a bridge to transplant in adults but are rarely used in this context in children. Nontransplant surgery or radiological interventions may still be indicated for noncirrhotic portal hypertension when the primary cause can be cured and to allow normalization of portal pressure before liver parenchyma is damaged by chronic secondary changes in some specific diseases. The meso-Rex bypass shunt is used widely but is limited to those with a favorable anatomy and can even be performed preemptively. Elective portosystemic shunt surgery is reserved for failure to respond to conservative management in the absence of alternative therapies.

Non-alcoholic fatty liver disease and hepatocellular carcinoma in a 7-year-old obese boy: coincidence or comorbidity?

Hepatocellular carcinoma (HCC) may develop from non‐alcoholic fatty liver disease (NAFLD) either in the presence or in the absence of established cirrhosis. Non‐cirrhotic patients with NAFLD‐related HCC are usually adult, male and obese. However, this association has not been reported yet in younger age groups. Objectives In the present study, the clinical case of a 7‐yaer‐old obese boy with steatosis and HCC is presented.

Congenital intrahepatic portohepatic shunt managed by interventional radiologic occlusion: a case report and literature review

Congenital intrahepatic portosystemic shunts are rare hepatic vascular anomalies that often lead to severe secondary conditions. A 6-year-old boy was referred for assessment of severe hypoxia, and a large liver mass was diagnosed with such a malformation and was managed by direct closure of the venous fistula by interventional radiology. Follow-up assessment shows normalization of the respiratory condition and a progressive reduction of the vascular liver lesion. Review of literature suggests that radiologic interventions are currently the criterion standard for managing these conditions, with surgery reserved for patients who are not eligible for radiologic procedure and those requiring liver transplantation.

Portal hypertension in children

The main causes of intrahepatic portal hypertension in children are cirrhosis and congenital hepatic fibrosis. Non cirrhotic portal hypertension in children is mostly due to extrahepatic portal vein obstruction. In half of cases, no underlying disorder is found. The meso-Rex bypass is the preferred treatment, when it is possible. The closest to the portal vein the obstruction, the highest the risk of esophagogastric varices.

Splitting livers: Trans‐hilar or trans‐umbilical division? Technical aspects and comparative outcomes

Controversy remains about the best line of division for liver splitting, through Segment IV or through the umbilical fissure. Both techniques are currently used, with the choice varying between surgical teams in the absence of an evidence‐based choice. We conducted a single‐center retrospective analysis of 47 left split liver grafts that were procured with two different division techniques: “classical” (N = 28, Group A) or through the umbilical fissure and plate (N = 19, Group B). The allocation of recipients to each group was at random; a single transplant team performed all transplantations. Demographics, characteristics, technical aspects, and outcomes were similar in both groups. The grafts in Group A, prepared with the classical technique, were procured more often with a single BD orifice compared with the grafts in Group B; however, this was not associated with a higher incidence of biliary problems in this series of transplants (96% actual graft survival rate [median ± s.d. follow‐up: 26 ± 20 months]). Both techniques provide good quality split grafts and an excellent outcome; surgical expertise with a given technique is more relevant than the technique itself. The classical technique, however, seems to be more flexible in various ways, and surgeons may find it to be preferable.

Focal congenital hyperinsulinism managed by medical treatment: a diagnostic algorithm based on molecular genetic screening

Congenital hyperinsulinism (CHI) requires rapid diagnosis and treatment to avoid irreversible neurological sequelae due to hypoglycaemia. Aetiological diagnosis is instrumental in directing the appropriate therapy. Current diagnostic algorithms provide a complete set of diagnostic tools including (i) biochemical assays, (ii) genetic facility and (iii) state‐of‐the‐art imaging. They consider the response to a therapeutic diazoxide trial an early, crucial step before proceeding (or not) to specific genetic testing and eventually imaging, aimed at distinguishing diffuse vs focal CHI. However, interpretation of the diazoxide test is not trivial and can vary between research groups, which may lead to inappropriate decisions. Objective of this report is proposing a new algorithm in which early genetic screening, rather than diazoxide trial, dictates subsequent clinical decisions.

Intrahepatic portal venous systems in children with noncirrhotic prehepatic portal hypertension: anatomy and clinical relevance.

BACKGROUND Children with extrahepatic portal hypertension typically present with cavernomatous transformation of the portal vein and a poorly defined intrahepatic portal vein system on conventional imaging. With the Meso-Rex Bypass becoming the gold-standard intervention for a cure, a precise assessment of the intrahepatic portal vein system provides helpful data for deciding whether a Meso-Rex Bypass is feasible or not. METHODS All children with extrahepatic portal hypertension were prospectively assessed by wedged hepatic venous portography. Venous anatomy was categorized into five subtypes (A to E), depending on the presence of thrombosis in the Rex recessus, or not, and its extension within the intrahepatic portal venous system. RESULTS Eighty-nine children entered the study. Previous umbilical vein catheterization is usually associated with Rex thrombosis, while the Rex recessus and the intrahepatic portal venous system are patent in idiopathic cases, thus allowing for the performance of a Meso-Rex Bypass with a good outcome. CONCLUSIONS Wedged hepatic venous portography is a very effective tool for detailed preoperative assessment and identification of children being considered for Meso-Rex Bypass surgery. An anatomic-radiological classification is useful in selecting patients for Meso-Rex Bypass with anticipation of a high rate of success.