Fibropolycystic liver disorders (FLD) arise from abnormal development of the ductal plate and are classified according to the size of the affected hepatobiliary duct. Congenital hepatic fibrosis (CHF) has small duct involvement characterized by a variable degree of periportal fibrosis and hyperplasia without affecting the liver's architecture. Caroli's disease (CD) is a rare autosomal recessive disorder with a prevalence of one case per 1,000,000 people and is characterized by cystic dilation of large intrahepatic ducts. When the disease presents with congenital hepatic fibrosis, it is referred to as Caroli's syndrome (CS). Patients are usually diagnosed around the age of 20 with episodes of cholangitis, portal hypertension or hepatomegaly. We present the case of a two-year-old male with a previous history of autosomal recessive polycystic kidney disease (ARPKD) who presented to the emergency room with variceal bleeding secondary to portal hypertension. The physical examination showed an acutely ill-looking boy, with evident paleness and distended abdomen. Past medical history was negative for previous gastrointestinal bleeding or episodes of cholangitis. An upper gastrointestinal endoscopy was performed, showing esophageal varices secondary to portal hypertension. Imaging studies revealed hepatosplenomegaly, alterations in liver echogenicity, and dilated saccular bile ducts affecting both liver lobes without observing any apparent obstruction, highly suggestive of CD. A liver biopsy revealed nodular liver tissue with marked fibrosis between nodules, which confirmed the presence of CHF. Both kidneys were increased in size, hyperechoic and with loss of corticomedullary differentiation. FLD commonly present with coexisting hepatobiliary and renal alterations. Therefore, starting at the time of initial diagnosis, all patients with ARPKD should be evaluated to detect liver abnormalities due to the high association. Despite the rarity of CS, especially in early childhood, the association between ARPKD and FLD is well documented. So if this clinical presentation arises, CS should be suspected.

Background Machete injuries constitute a major cause of morbidity in Honduras. In this study, we aimed to determine the incidence, initial management, surgical treatment, and follow-up patterns for machete injuries at the national public hospital in Honduras. Microsurgery in Honduras is currently in transition with limitations at multiple levels. This study aims to provide critical information to better prepare visiting surgeons and establishes a blueprint to improve microsurgical reconstruction. Methods A retrospective chart review was performed to identify patients with machete injuries to the upper extremity (UE) who presented to the Hospital Escuela Universitario (HEU) for treatment from 2015 to 2017. Additional microsurgical data was obtained by personal communication with members of the plastic surgery department at the HEU. Results Complete data was retrieved for 100 patients who presented to the HEU with a UE machete wound. The cohort was male dominated (93%), employed as farmers (47%), and had a mean age of 32.1 years. Violence was the most common mechanism of injury (p < 0.001). The majority of UE machete injuries involved tendon (70%), nerve (28%), and an open fracture (55%). Of the 76% of patients who were scheduled for a follow-up visit, only 25% attended. Within the last calendar year, one replantation, 10 revascularizations at the wrist and forearm level, three microvascular free tissue transfers, and 175 nerve repairs were performed. Conclusion Management of UE machete injuries in Honduras is challenging and requires early recognition of possible injuries to multiple anatomical systems. The majority of injuries required operative intervention. Only a small percentage of patients presented for follow up. A program to streamline care starting at injury recognition up to final follow-up is currently unavailable and needs to be developed to optimize microsurgical care.