Complex lymphatic malformations in pediatrics: a review of treatment options.

INTRODUCTION: Lymphatic malformations (LMs) are low-flow lesions resulting from abnormalities in the development of lymphatics. The management of these lesions is complex and involve the collaboration of many specialties. The purpose of this review is to summarize current knowledge regarding the different therapeutic options used in complex lymphatic malformations, analyzing their indications, efficacy and complications. EVIDENCE ACQUISITION: A search was made using the algorithm: "(lymphatic abnormality OR lymphatic malformation OR lymphangioma OR cystic hygroma) AND (extensive OR giant OR complex) AND (therapeutics OR treatment) AND (child OR children)". Of the 120 articles found, 53 were included. EVIDENCE SYNTHESIS: Historically, surgery was the treatment of choice for this type of lesions. However, excision was often incomplete, associated with high rates of recurrence and severe complications. The use of sclerotherapy emerged as a minimal invasive option appropriate in selected cases as a single or adjuvant therapy. Inhibitors of the mammalian target of rapamycin, such as sirolimus, now play a central role in the treatment of complex malformations resistant to sclerotherapy, recurrent after surgery or more extensive malformations that affect vital structures. Other therapeutic options as sildenafil and laser ablation are also recognized as effective in selected cases. CONCLUSIONS: Looking through the literature over the last decade authors realize that surgery had gradually been replaced by less invasive options such as sirolimus with or without adjuvant sclerotherapy. In conclusion, each treatment option seems to have its own indications and characteristics, which must be considered in therapeutic decision and individualized for each patient.

Congenital Shunts of the Portal Venous System: Case-series of Uncommon Shunts.

BACKGROUND AND AIMS: Congenital shunts of the portal venous system are rare entities that can present in children with clinical heterogeneity. To evaluate the clinical course of children with uncommon shunts presenting to our institution and examine the available literature on this topic. Medical records of children with rare forms of congenital shunts were retrospectively reviewed for demographics, symptoms, management, and outcome between 2003 and 2016. RESULTS: Three female patients with congenital shunts, including a congenital mesenterico-portal Rex shunt (n = 1) and congenital portosystemic shunts (CPSS) (n = 2), were referred for surgical evaluation between ages 4 and 9. Median follow-up was 8 years (range, 6-13 years). One asymptomatic patient did not require treatment and remained disease-free during long-term follow-up. The other 2 patients with CPSS and unusual symptoms, including liver focal nodular hyperplasia (FNH) in infancy (n = 1) and bleeding from esophageal varices (n = 1), showed subsequent progression to liver nodules that were managed by endovascular shunt occlusion. One patient showed symptom resolution and the other showed stable lesions at last follow-up. Literature yielded descriptions of two cases of congenital mesenterico-portal Rex shunt, one case of coincident CPSS and FNH in infancy, but zero reports of bleeding from esophageal varices. CONCLUSIONS: This case series examines each distinct patient's presentation, discusses the diagnosis, management and outcome and compares findings while discussing literature on this topic. A high index of suspicion and familiarity with unusual forms and treatment options is required to allow timely diagnosis and appropriate treatment.

Hydroxyethyl starch 130/0.4 attenuates early hepatic damage in ischemia/reperfusion injury.

PURPOSE: Ischemia/reperfusion injury (IRI) remains a clinical challenge. We tested the hypothesis that fluid therapy using hydroxyethyl starch (HES) 130/0.4 during the early phase of IRI in rat liver decreases markers of hepatic injury. METHODS: We induced liver IRI in three groups of rats anesthetized with ketamine and chlorpromazine by means of 60 min of segmental hepatic ischemia followed by 120 min of reperfusion. At the onset of reperfusion, Group 1 (IRI + HES; n = 12) was given 13 mL.kg(-1) of HES; Group 2 (IRI + HS; n = 12) received the same volume of 7.5% saline (HS), and Group 3 (IRI-only; n = 12) received no fluid. Three other groups of 12 animals each were sham-operated and received the same fluid as the test groups. We euthanized the animals after three hours, drew blood for alanine aminotransferase (ALT) quantification, and took ischemic liver samples for histomorphological study. RESULTS: Serum ALT activity was greater in all of the IRI groups than in the sham-operated animals. The ALT activity was 1,081 +/- 575 IU.L(-1) in IRI + HES Group 1; 2,363 +/- 1,839 IU.L(-1) in IRI + HS Group 2; and 2,866 +/- 2,491 IU.L(-1) in IRI-only Group 3. There was a statistically significant difference between the IRI + HES and the IRI-only groups (P = 0.001), but not between the IRI + HS and the IRI-only groups (P > 0.05). Likewise, histological scores were greater in all IRI groups compared with the sham-operated animals. Scores were higher in the IRI-only group (median 3.5) than in the groups receiving fluid (IRI + HES median 2; IRI + HS median 3). The difference between IRI + HES and IRI-only was statistically significant (P = 0.008) but not so between IRI + HS and IRI-only (P > 0.05). CONCLUSIONS: Giving HES 130/0.4 attenuates rat liver IRI compared with no fluid, while giving HS does not. This suggests a role for HES in hepatoprotection associated with liver IRI.