Long-term Outcomes and Health Perceptions in Pediatric-onset Portal Hypertension Complicated by Varices

Objectives: Outcomes of pediatric-onset portal hypertension are poorly defined. We aimed to assess population-based long-term outcomes of pediatric-onset portal hypertension complicated by varices. Methods: All children with esophageal varices (n = 126) were identified from 14,144 single nationwide referral center endoscopy reports during 1987 to 2013, and followed up through national health care and death registers. A questionnaire was sent to survivors (n = 94) of whom 65 (69%) responded. Results: Nineteen underlying disorders included biliary atresia (35%), extrahepatic portal vein obstruction (35%), autosomal recessive polycystic kidney disease (7%), and other disorders (23%). During median follow-up of 15.2 (range 0.5-43.1) years patients underwent median 9 (1-74) upper gastrointestinal endoscopies. Esophageal varices were first observed at a median age of 4.0 (0.3-18.2) years, 112 (89%) patients underwent median 6 (1-56) sclerotherapy/banding sessions, and 61 (48%) experienced median 2 (range 1-20) variceal bleeding episodes. Forty-eight surgical shunt procedures were performed to 41 (36%) patients and 38% underwent liver transplantation. Portal hypertensive biliopathy was diagnosed in 4 patients. Hepatopulmonary syndrome necessitated liver transplantation in 2 patients, hepatic encephalopathy in 2, and hepatorenal syndrome in 1. No patient died of variceal bleeding. Patient-reported perception of health on a scale of 1 to 10 was 9 (range 4-10), and 86% reported no current symptoms attributable to esophageal varices. Conclusions: Pediatric-onset portal hypertension is a heterogeneous disease with significant long-term morbidity, requiring multimodal approach with considerable resources and continuation of follow-up in adulthood. Although mortality to variceal bleeding was avoided, bleeding episodes recurred also in adulthood, while patient-reported health of long-term survivors was encouraging.Peer reviewe

Giant inguinal hernia in a preterm child : Technical challenges and long-term outcome

Giant inguinal hernias are rare in children, and they represent a unique challenge for pediatric surgeons. The sparse literature includes variable definitions of the disease and different surgical techniques without significant difficulties with the decrease of abdominal domain. We present a prematurely born boy with a unilateral giant inguinal hernia which developed promptly after birth. This case highlights the multiple possible challenges related to giant inguinal hernias in children. We also discuss the possible treatment options and the advantages and disadvantages based on the current literature.publishedVersionPeer reviewe

Reduced Number of Pediatric Orthopedic Trauma Requiring Operative Treatment during COVID-19 Restrictions: A Nationwide Cohort Study

Cited by: 28; All Open Access, Green Open AccessPeer reviewe

Reduced Number of Pediatric Orthopedic Trauma Requiring Operative Treatment during COVID-19 Restrictions: A Nationwide Cohort Study

Cited by: 28; All Open Access, Green Open AccessPeer reviewe

Long-term outcomes after pediatric splenectomy

Background. Splenectomy is performed frequently for various and primarily hematologic indications in children and adolescents. We analyzed the long-term outcome after splenectomy (median, 8.7 years) focusing on sepsis, portal vein thrombosis (PVT), and retained accessory spleen. Methods. In total, 141 consecutive children after open (n = 89; 63 %) or laparoscopic (n = 52; 37%) splenectomy from 1991 to 2010 were followed up through nationwide registries for septic infections, PVT, and causes of death. Sixty-six patients (58% of survivors) answered a structured questionnaire on infections, abdominal symptoms, and general health, and 64 (laparoscopic n = 26, open n = 38) consented to ultrasonography of the portal venous system. Results. Median operation age was 8.8 years (range, 1.0-22). Reoperations were required for bleeding after open procedures (n = 1) and retained accessory spleen after laparoscopic procedures (n = 3). Postsplenectomy sepsis occurred after a median of 1.7 years (range, 0.2-5.9) in 11 patients (8%), of whom 10 had an underlying immunodeficiency. No cases of PVT were observed, although the median portal vein flow was 1,130 mL/min (range, 440-2200) and diameter was 9.9 mm (range, 7-15) at a median follow-up of 9.5 years (range, 2.0-22) after splenectomy. Twenty-seven patients (19%) died after 8.7 years (0.03-23.00). The most common cause of death was the underlying malignancy (n = 15), with sepsis being an additional cause of death in 5 patients. Conclusion. Postsplenectomy sepsis was associated almost exclusively with an underlying immunodeficiency with a high mortality rate. No PVT was observed. The overall risk of retained accessory spleen was around 7%, and was slightly greater after laparoscopic operation,.Peer reviewe