Epidemiology and Outcome of Major Congenital Malformations in a Large German County

Abstract

Introduction Congenital malformations are associated with substantial neonatal morbidity and mortality. Furthermore, only sparse data are available on the modalities of care provided to and the associated clinical outcomes in affected neonates. In this study, we focused on five malformations that require surgery during the neonatal period: duodenal stenosis and atresia (DA), gastroschisis (GA), omphalocele (OM), congenital diaphragmatic herniation (CDH), and esophageal atresia (EA).

Materials and Methods We reviewed the Hessian neonatal registry (2010–2015) to identify records including the ICD-10 (International Classification of Diseases, Tenth Edition) codes for the aforementioned diagnoses and identified 283 patients who were affected by at least one of these conditions. Multiple regression analyses were performed to further identify risk factors for mortality and extended length of hospital stay.

Results The incidence rates per 10,000 live births and inhospital mortality rates were as follows: DA: 1.79 and 3.6%; GA: 1.79 and 1.8%; OM: 1.60 and 24%; CDH: 1.32 and 27.5%; and EA: 2.67 and 11.1%, respectively. Thirty-three percent of the patients had not been born in a perinatal center in which corrective surgeries were performed. The following risk factors were significantly associated with early mortality: trisomy 13 and 18, congenital heart defects, prematurity, and high-risk malformations (OM and CDH). The predictors of length of stay were as follows: gestational age, number of additional malformations, and treatment in the center with the highest patient volume.

Conclusion Epidemiology and outcome of major congenital malformations in Hesse, Germany, are comparable to previously published data. In addition, our data revealed a volume–outcome association with regard to the length of hospital stay.

Keywords

congenital malformation - neonate - esophageal atresia - outcome - caseload

• References

• 1 Tennant PWG, Pearce MS, Bythell M, Rankin J. 20-year survival of children born with congenital anomalies: a population-based study. Lancet 2010; 375 (9715): 649-656
• 2 Calzolari E, Barišic I, Loane M. , et al. Epidemiology of multiple congenital anomalies in Europe: a EUROCAT population-based registry study. Birth Defects Res A Clin Mol Teratol 2014; 100 (04) 270-276
• 3 Stark AR. ; American Academy of Pediatrics Committee on Fetus and Newborn. Levels of neonatal care. Pediatrics 2004; 114 (05) 1341-1347
• 4 American Academy of Pediatrics Committee on Fetus And Newborn. Levels of neonatal care. Pediatrics 2012; 130 (03) 587-597
• 5 Becke K. Empfehlungen für die strukturellen Voraussetzungen der perinatologischen Versorgung in Deutschland. Anästhesiologie & Intensivmedizin 2016; 57: 465-471
• 6 Deutschland. Bekanntmachung eines Beschlusses des Gemeinsamen Bundesausschusses über eine Richtlinie über Maßnahmen zur Qualitätssicherung der herzchirurgischen Versorgung bei Kindern und Jugendlichen gemäß § 137 Absatz 1 Nummer 2 des Fünften Buches Sozialgesetzbuch (SGB V), (Richtlinie zur Kinderherzchirurgie): Erstfassung: Vom. Köln: Bundesanzeiger-Verl.-Ges; 2010
• 7 Gaynor JW, Stopp C, Wypij D. , et al; International Cardiac Collaborative on Neurodevelopment (ICCON) Investigators. Neurodevelopmental outcomes after cardiac surgery in infancy. Pediatrics 2015; 135 (05) 816-825
• 8 Reiss I, Schaible T, van den Hout L. , et al; CDH EURO Consortium. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium consensus. Neonatology 2010; 98 (04) 354-364
• 9 Statistisches Bundesamt. Anzahl der Geburten in Hessen von 1990 bis 2015. Available at: https://de.statista.com/statistik/daten/studie/588908/umfrage/anzahl-der-geburten-in-hessen/ . Accessed November 18, 2016
• 10 Tutdibi E, Gries K, Bücheler M, Misselwitz B, Schlosser RL, Gortner L. Impact of labor on outcomes in transient tachypnea of the newborn: population-based study. Pediatrics 2010; 125 (03) e577-e583
• 11 Kalder M, Reichelt J, Kyvernitakis I, Misselwitz B, Hadji P, Schmidt S. A Population-based study on trends in the mode of delivery subsequent to stillbirth, infant mortality and severe infant malformations in the state of Hesse, Germany between 1990 and 2012. Z Geburtshilfe Neonatol 2014; 218 (03) 113-121
• 12 Olney RS, Ailes EC, Sontag MK. Detection of critical congenital heart defects: review of contributions from prenatal and newborn screening. Semin Perinatol 2015; 39 (03) 230-237
• 13 Bremer S, Kiess W, Thome U. , et al. Pravalenz von Gastroschisis, Omphalozele, Spina bifida und orofazialen Spaltbildungen bei Neugeborenen im Zeitraum Januar 2000-Dezember 2010 in Leipzig, Sachsen, Sachsen-Anhalt und Deutschland [German]. Gesundheitswesen 2018; 80 (02) 122-128
• 14 EUROCAT. Cases and prevalence (per 10,000 births) of all congenital anomaly subgroups for all registries, from 2008 to 2012. Available at: http://www.eurocat-network.eu/ACCESSPREVALENCEDATA/PrevalenceTables . Accessed May 8, 2017
• 15 Haeusler MCH, Berghold A, Stoll C, Barisic I, Clementi M. ; EUROSCAN Study Group. Prenatal ultrasonographic detection of gastrointestinal obstruction: results from 18 European congenital anomaly registries. Prenat Diagn 2002; 22 (07) 616-623
• 16 Pedersen RN, Calzolari E, Husby S, Garne E. ; EUROCAT Working group. Oesophageal atresia: prevalence, prenatal diagnosis and associated anomalies in 23 European regions. Arch Dis Child 2012; 97 (03) 227-232
• 17 Centers for Disease Control and Prevention (CDC). Hospital stays, hospital charges, and in-hospital deaths among infants with selected birth defects--United States, 2003. Morb Mortal Wkly Rep 2007; 56 (02) 25-29
• 18 Wang Y, Hu J, Druschel CM, Kirby RS. Twenty-five-year survival of children with birth defects in New York State: a population-based study. Birth Defects Res A Clin Mol Teratol 2011; 91 (12) 995-1003
• 19 Sulkowski JP, Cooper JN, Lopez JJ. , et al. Morbidity and mortality in patients with esophageal atresia. Surgery 2014; 156 (02) 483-491
• 20 Sfeir R, Bonnard A, Khen-Dunlop N. , et al. Esophageal atresia: data from a national cohort. J Pediatr Surg 2013; 48 (08) 1664-1669
• 21 Wang B, Tashiro J, Allan BJ. , et al. A nationwide analysis of clinical outcomes among newborns with esophageal atresia and tracheoesophageal fistulas in the United States. J Surg Res 2014; 190 (02) 604-612
• 22 Götz D, Köhn A, Rißmann A, Spillner C, Vogt C. Jahresbericht des Bundeslandes Sachsen-Anhalt zur Häufigkeit von congenitalen Fehlbildungen und Anomalien sowie genetisch bedingten Erkrankungen 2013: Fehlbildungsmonitoring Sachsen-Anhalt an der Medizinischen Fakultät der Otto-von-Guericke-Universität Magdeburg. Available at: http://www.angeborene-fehlbildungen.com/monz_mm/Dokumente/Jahresberichte/Bericht2013_WEB.pdf . Updated February 16, 2017. Accessed April 8, 2017
• 23 Escobar MA, Ladd AP, Grosfeld JL. , et al. Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg 2004; 39 (06) 867-871
• 24 Chen QJ, Gao ZG, Tou JF. , et al. Congenital duodenal obstruction in neonates: a decade's experience from one center. World J Pediatr 2014; 10 (03) 238-244
• 25 Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA. Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg 1998; 133 (05) 490-496
• 26 Bradnock TJ, Marven S, Owen A. , et al; BAPS-CASS. Gastroschisis: one year outcomes from national cohort study. BMJ 2011; 343: d6749
• 27 Skarsgard ED, Claydon J, Bouchard S. , et al; Canadian Pediatric Surgical Network. Canadian Pediatric Surgical Network: a population-based pediatric surgery network and database for analyzing surgical birth defects. The first 100 cases of gastroschisis. J Pediatr Surg 2008; 43 (01) 30-34
• 28 Brindle ME, Flageole H, Wales PW. ; Canadian Pediatric Surgery Network (CAPSNet). Influence of maternal factors on health outcomes in gastroschisis: a Canadian population-based study. Neonatology 2012; 102 (01) 45-52
• 29 Springett A, Draper ES, Rankin J. , et al. Birth prevalence and survival of exomphalos in england and wales: 2005 to 2011. Birth Defects Res A Clin Mol Teratol 2014; 100 (09) 721-725
• 30 Brindle ME, Cook EF, Tibboel D, Lally PA, Lally KP. ; Congenital Diaphragmatic Hernia Study Group. A clinical prediction rule for the severity of congenital diaphragmatic hernias in newborns. Pediatrics 2014; 134 (02) e413-e419
• 31 Gallot D, Boda C, Ughetto S. , et al. Prenatal detection and outcome of congenital diaphragmatic hernia: a French registry-based study. Ultrasound Obstet Gynecol 2007; 29 (03) 276-283
• 32 Colvin J, Bower C, Dickinson JE, Sokol J. Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia. Pediatrics 2005; 116 (03) e356-e363
• 33 Jaillard SM, Pierrat V, Dubois A. , et al. Outcome at 2 years of infants with congenital diaphragmatic hernia: a population-based study. Ann Thorac Surg 2003; 75 (01) 250-256