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

Surgically treated ovarian lesions in preadolescent girls

Introduction The purpose of this study was to investigate the epidemiology and characteristics of surgically treated ovarian lesions in preadolescent girls. Material and methods This was a retrospective cohort study including all 0- to 11-year-old girls operated at a single center from 1999 to 2016 for ovarian cysts, neoplasms or torsions. Patient charts were reviewed for symptoms, preoperative radiological imaging, operative details and histopathology. Results We identified 78 girls, resulting in a population-based incidence of 4.2/100 000. Infants (n = 44) presented with benign cysts (42/44, 95%, one bilateral), a benign neoplasm (1/44, 2%) and a torsion without other pathology (1/44, 2%). Torsion was found in 25/29 (86%) ovaries with complex cysts and in 3/15 (21%) ovaries with simple cysts in preoperative imaging (P <0.001). Most infants were symptomless. Lesions in 1- to 11-year-old girls (n = 34) included benign neoplasms (n = 21/34, 62%), malignant neoplasms (n = 5/34, 15%), a cyst with torsion (n = 1/34, 3%) and torsions without other pathology (n = 7/34, 21%). Torsion was more common in benign (17/21, 81%) than in malignant neoplasms (1/5, 20%) (P <0.020). Ovarian diameter did not differ between ovaries with or without torsion (P = 0.238) or between benign and malignant neoplasms (P = 0.293). The duration of symptoms in lesions with or without torsion was similar. Conclusions The majority of surgically treated ovarian lesions in preadolescent are benign lesions with torsion. Surgery should be ovary-preserving and performed without delay.Peer reviewe

Ruokatorviatresia - hoito, komplikaatiot ja pitkäaikaisongelmat

English summaryPeer reviewe

Dimensions of random affine code tree fractals

We calculate the almost sure Hausdorff dimension for a general class of random affine planar code tree fractals. The set of probability measures describing the randomness includes natural measures in random $V$-variable and homogeneous Markov constructions.Comment: 22 page

Outcomes of fundoplication in oesophageal atresia associated gastrooesophageal reflux disease

Aim of the study: Conservative management of gastrooesophageal reflux (GORD) in oesophageal atresia (OA) is sometimes inefficient, and fundoplication is required. We assessed the outcomes of fundoplication among OA patients from 1980 to 2016. Methods: After ethical consent, hospital records of 290 patients, including 22 referred patients, were reviewed. Included were 262 patients with end-to-end repair. Excluded were patients who underwent oesophageal reconstruction (n = 23) or no repair (n = 5). Primary outcome measures included survival, retaining the native oesophagus, resolution of GGORD symptoms, failure of fundoplication, and long-term endoscopic results. Main results: Gross types of OA in 262 patients were A (n = 12), B (n = 2), C (n = 217), D (n = 10), E (n = 19), and F (n = 2). Eighty-six (33%) patients, type A (n = 12, 100%), B (n = 2, 100%), C (n = 69, 31%), D (n = 3, 30%), and F (n = 1, 50%), underwent fundoplication at the median age of 5.4 (IQR 3.1-16) months. Main indications included recalcitrant anastomotic stenosis (RAS) in 41 (48%), respiratory symptoms in 16 (19%), and acute life threatening events (ALTE) in 15 (17%) of patients. Associated tracheomalacia in 25 (29%) patients were treated with aortopexy. Median follow-up was 7.5 (IQR 1.8-15) years. RAS resolved in 30 (73%) patients, whereas 11 (27%) with unresolved RAS underwent oesophageal resection (n = 8) or replacement (n = 3). Six (7%) patients died of heart failure (n = 4), bolus impaction (n = 1), and ALTE (n = 1). Fundoplication failed in 27 (31%) patients, and 13 (15%) underwent redo fundoplication. Fundoplication failure was predicted by long-gap OA RR = 3.8 (95% CI = 1.1-13), P = 0.04. In total GORD associated symptoms persisted in 7 (8%) patients, including one with permanent feeding jejunostomy. Latest endoscopy showed moderate or severe oesophagitis in 7% of fundoplicated and in 3% nonfundoplicated patients and intestinal metaplasia in 3% and 1% (p = 0.20-0.29). Conclusion: Fundoplication provided a safe and relatively effective control of OA associated symptomatic GORD and oesophagitis. The failure rate of fundoplication was high in those with long-gap OA. Type of study: Treatment study. Level of evidence: IV (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Combined management of perianal rhabdomyosarcoma with chemotherapy, radical surgery, and irradiation : A series of three consecutive children

Background and aim: We describe a series of three successive patients with perianal rhabdomyosarcoma (PRMS) from 2014 to 2017 managed with combined chemotherapy, radical surgery, and radiotherapy. Methods: Ethical consent was obtained. Data including tumor presentation, treatment, and survival was collected from hospital reports. Results: Two girls aged 15 and 16 years (patient #1 and #2) and one boy aged five years (patient #3) were referred because of a suspected perianal abscess. MRI showed large perianal tumors from 7 to 12 cm in diameter that surrounded or infiltrated the anal sphincters and were inconsistent with abscess. Tumor biopsies showed RMS of alveolar (#1 and #2) and embryonal (#3) types. Patient #1 had lymph node and bone metastases, patient #2 lymph node metastases, and patient # 3 no metastases. Pretreatment staging, IRS Clinical Group, and Risk Groups were: Stage 4, II, high; Stage 3, GII, intermediate; and Stage 3, I, low, respectively. All underwent colostomy before neoadjuvant chemotherapy (CWS-RMS 2009 program). Neoadjuvant chemotherapy failed to clear the tumors from anal sphincters preventing anus-saving surgery, and all patients underwent abdominoperineal excision. All removed specimens had freemargins with negative lymph nodes. After adjuvant chemotherapy and local radiation, the patients were tumor free after 48, 13, and 18 months. Conclusion: In PRMS local surgical control required abdominoperineal excision. Confusion between PRMS and abscess may cause unnecessary delay in management. (C) 2018 Elsevier Inc. All rights reserved.Peer reviewe

Risk factors and outcomes of tapering surgery for small intestinal dilatation in pediatric short bowel syndrome

Background: In remains unclear why in some short bowel syndrome (SBS) patients, the remaining small bowel (SB) dilates excessively leading to requirement of tapering surgery. Methods: Among SBS children, we retrospectively analyzed risk factors for tapering surgery with logistic regression and compared the outcome of operated patients (n = 16) to those managed conservatively (n = 44) with Cox proportional hazards regression. Results: SBS was caused by necrotizing enterocolitis (NEC) (n = 31), SB atresia (SBA) (n = 13), midgut volvulus (n = 12), or gastroschisis (n = 4). Patients with spontaneous symptomatic SB dilatation unable to wean parenteral nutrition (PN) underwent tapering surgery at median age of 1.04 (interquartile range 0.70-3.27) years. Missing ICV was related to an 8-fold (p = 0.003) increased risk while SBA diagnosis was related to a 13-fold risk of tapering surgery (p <0.001). Increasing SB length and NEC diagnosis were protective of tapering (p = 0.027-0.004). Of operated patients, 75% reached enteral autonomy during follow-up and their postoperative adjusted PN weaning rate was similar to nonoperated children (p = 0.842). Conclusion: SBS children with short remaining SB, missing ICV, and SBA etiology are more likely while NEC patients are less likely than others to necessitate tapering surgery. Postoperative PN weaning rates were comparable to patients who initially had more favorable intestinal anatomy and adapted without surgery. (C) 2017 Elsevier Inc. All rights reserved.Peer reviewe

Altered Bile Transporter Expression and Cholesterol Metabolism in Children With Cholesterol and Pigment Gallstones

Objectives: We elucidated pathophysiology of pediatric gallstone disease by assessing liver expression of bile transporters in relation to bile acids and surrogates of cholesterol absorption and synthesis in serum and gallstones. Methods: RNA expression of canalicular bile transporters in liver biopsies from 32 pediatric gallstone patients and from 6 liver donors (controls) was measured by qRT-PCR (quantitative real-time reverse transcription polymerase chain reaction). Concentrations of cholesterol and precursors, plant sterols and bile acids in gallstones, and in serum of the patients and 82 healthy children were measured. Primary outcomes were the difference in RNA expressions and serum sterol profiles between patients and controls. Results: Cholesterol stones (CS; n = 15) contained cholesterol >42% and pigment stones (PS; n = 17)Peer reviewe