Eur J Pediatr Surg 2020; 30(01): 051-058
DOI: 10.1055/s-0039-1698768
Original Article
Georg Thieme Verlag KG Stuttgart · New York

Novel Risk Score for Fetuses with Congenital Diaphragmatic Hernia Based on Ultrasound Findings

Keita Terui
1   Department of Pediatric Surgery, Chiba University, Chiba, Japan
,
Kouji Nagata
2   Department of Pediatric Surgery, Reproductive and Developmental Medicine, Graduate School of Medical Sciences, Kyushu University, Fukuoka, Japan
,
Masahiro Hayakawa
3   Division of Neonatology, Center for Maternal-Neonatal Care, Nagoya University Hospital, Nagoya, Japan
,
Hiroomi Okuyama
4   Department of Pediatric Surgery, Osaka University Graduate School of Medicine, Suita, Osaka, Japan
,
Shoichirou Amari
5   Division of Neonatology, National Center for Child Health and Development, Setagaya-ku, Tokyo, Japan
,
Akiko Yokoi
6   Department of Pediatric Surgery, Kobe Children's Hospital, Kobe, Japan
,
Kouji Masumoto
7   Department of Pediatric Surgery, Tsukuba Daigaku, Tsukuba, Ibaraki, Japan
,
Naoto Urushihara
8   Department of Pediatric Surgery, Shizuoka Children's Hospital, Shizuoka, Japan
,
Tadaharu Okazaki
9   Department of Pediatric Surgery, Juntendo University Urayasu Hospital, Chiba, Japan
,
Noboru Inamura
10   Department of Pediatrics, Kinki University, Higashiosaka, Osaka, Japan
,
Katsuaki Toyoshima
11   Departments of Neonatology, Kanagawa Childrens Medical Center, Yokohama, Kanagawa, Japan
,
Keiichi Uchida
12   Department of Gastrointestinal and Pediatric Surgery, Mie University Graduate School of Medicine, Tsu, Mie, Japan
,
Taizo Furukawa
13   Department of Pediatric Surgery, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto, Japan
,
Manabu Okawada
14   Department of Pediatric General and Urogenital Surgery, Juntendo University School of Medicine, Tokyo, Japan
,
Yasunori Sato
15   Department of Preventive Medicine and Public Health, Keio University, Minato-ku, Tokyo, Japan
,
Noriaki Usui
16   Department of Pediatric Surgery, Osaka Women's and Children's Hospital, Izumi, Japan
› Author Affiliations
Funding This study was funded by the Ministry of Health, Labor, and Welfare of Japan (Grant/Award Number: “H24-Nanchi-Ippan-034”).
Further Information

Publication History

07 May 2019

02 September 2019

Publication Date:
10 October 2019 (online)

Abstract

Introduction We aimed to establish and validate a risk score for fetuses with congenital diaphragmatic hernia (CDH) using only prenatal ultrasound findings.

Material and Methods Derivation (2011–2016, n = 350) and validation (2006–2010, n = 270) cohorts were obtained from a Japanese CDH study group database. Using a logistic regression analysis, we created a prediction model and weighted scoring system from the derivation dataset and calculated the odds ratio of an unsatisfactory prognosis (death within 90 days of life or hospitalization duration exceeding 180 days). Five adverse prognostic factors obtained using prenatal ultrasound, including an observed/expected lung area-to-head circumference ratio (o/eLHR) <25%, liver herniation occupying more than one-third of the thoracic space, thoracic stomach, right-side CDH, and severe malformations, were used as predictors. The obtained model was validated using the validation cohort.

Results The unsatisfactory prognosis prediction model was obtained based on the adjusted odds ratios. The C statistics of the model were 0.83 and 0.80 in the derivation and validation datasets, respectively. The five variables were weighted proportionally to their adjusted odds ratios for an unsatisfactory prognosis (o/eLHR <25%, 1 point; liver herniation occupying more than one-third of the thoracic space, 1 point; thoracic stomach, 1 point; right-side CDH, 2 points; and severe malformations, 3 points). Unsatisfactory prognosis rates for the low- (0–2 points), intermediate- (3–5 points), and high-risk (6–8 points) groups were 17, 46, and 100%, respectively (p < 0.001), in the validation cohort.

Conclusion Our simple risk score effectively predicted the prognosis of fetuses with CDH.

 
  • References

  • 1 Skari H, Bjornland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 2000; 35 (08) 1187-1197
  • 2 Berman L, Jackson J, Miller K, Kowalski R, Kolm P, Luks FI. Expert surgical consensus for prenatal counseling using the Delphi method. J Pediatr Surg 2018; 53 (08) 1592-1599
  • 3 Puligandla PS, Grabowski J, Austin M. , et al. Management of congenital diaphragmatic hernia: a systematic review from the APSA outcomes and evidence based practice committee. J Pediatr Surg 2015; 50 (11) 1958-1970
  • 4 Senat MV, Bouchghoul H, Stirnemann J. , et al; Center for Rare Diseases: Congenital Diaphragmatic Hernia. Prognosis of isolated congenital diaphragmatic hernia using lung-area-to-head-circumference ratio: variability across centers in a national perinatal network. Ultrasound Obstet Gynecol 2018; 51 (02) 208-213
  • 5 Usui N, Kitano Y, Okuyama H. , et al. Reliability of the lung to thorax transverse area ratio as a predictive parameter in fetuses with congenital diaphragmatic hernia. Pediatr Surg Int 2011; 27 (01) 39-45
  • 6 Ruano R, Lazar DA, Cass DL. , et al. Fetal lung volume and quantification of liver herniation by magnetic resonance imaging in isolated congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 2014; 43 (06) 662-669
  • 7 Kitano Y, Nakagawa S, Kuroda T. , et al. Liver position in fetal congenital diaphragmatic hernia retains a prognostic value in the era of lung-protective strategy. J Pediatr Surg 2005; 40 (12) 1827-1832
  • 8 Kitano Y, Okuyama H, Saito M. , et al. Re-evaluation of stomach position as a simple prognostic factor in fetal left congenital diaphragmatic hernia: a multicenter survey in Japan. Ultrasound Obstet Gynecol 2011; 37 (03) 277-282
  • 9 Duess JW, Zani-Ruttenstock EM, Garriboli M, Puri P, Pierro A, Hoellwarth ME. Outcome of right-sided diaphragmatic hernia repair: a multicentre study. Pediatr Surg Int 2015; 31 (05) 465-471
  • 10 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
  • 11 Gentili A, Pasini L, Iannella E. , et al. Predictive outcome indexes in neonatal congenital diaphragmatic hernia. J Matern Fetal Neonatal Med 2015; 28 (13) 1602-1607
  • 12 Nagata K, Usui N, Kanamori Y. , et al. The current profile and outcome of congenital diaphragmatic hernia: a nationwide survey in Japan. J Pediatr Surg 2013; 48 (04) 738-744
  • 13 Terui K, Nagata K, Hayakawa M. , et al. Growth assessment and the risk of growth retardation in congenital diaphragmatic hernia: a long-term follow-up study from the Japanese Congenital Diaphragmatic Hernia Study Group. Eur J Pediatr Surg 2016; 26 (01) 60-66
  • 14 Snoek KG, Reiss IK, Greenough A. , et al; CDH EURO Consortium. Standardized postnatal management of infants with congenital diaphragmatic hernia in Europe: the CDH EURO Consortium Consensus—2015 Update. Neonatology 2016; 110 (01) 66-74
  • 15 Congenital Diaphragmatic Hernia Study Group. Estimating disease severity of congenital diaphragmatic hernia in the first 5 minutes of life. J Pediatr Surg 2001; 36 (01) 141-145
  • 16 Schultz CM, DiGeronimo RJ, Yoder BA. ; Congenital Diaphragmatic Hernia Study Group. Congenital diaphragmatic hernia: a simplified postnatal predictor of outcome. J Pediatr Surg 2007; 42 (03) 510-516
  • 17 Richardson DK, Corcoran JD, Escobar GJ, Lee SK. SNAP-II and SNAPPE-II: simplified newborn illness severity and mortality risk scores. J Pediatr 2001; 138 (01) 92-100
  • 18 Lally KP, Lasky RE, Lally PA. , et al; Congenital Diaphragmatic Hernia Study Group. Standardized reporting for congenital diaphragmatic hernia—an international consensus. J Pediatr Surg 2013; 48 (12) 2408-2415
  • 19 Terui K, Nagata K, Kanamori Y. , et al. Risk stratification for congenital diaphragmatic hernia by factors within 24 h after birth. J Perinatol 2017; 37 (07) 805-808
  • 20 Ruano R, Takashi E, da Silva MM, Campos JA, Tannuri U, Zugaib M. Prediction and probability of neonatal outcome in isolated congenital diaphragmatic hernia using multiple ultrasound parameters. Ultrasound Obstet Gynecol 2012; 39 (01) 42-49
  • 21 Neff KW, Kilian AK, Schaible T, Schütz EM, Büsing KA. Prediction of mortality and need for neonatal extracorporeal membrane oxygenation in fetuses with congenital diaphragmatic hernia: logistic regression analysis based on MRI fetal lung volume measurements. Am J Roentgenol 2007; 189 (06) 1307-1311
  • 22 Putnam LR, Harting MT, Tsao K. , et al; Congenital Diaphragmatic Hernia Study Group. Congenital diaphragmatic hernia defect size and infant morbidity at discharge. Pediatrics 2016; 138 (05) e20162043
  • 23 Peetsold MG, Huisman J, Hofman VE, Heij HA, Raat H, Gemke RJ. Psychological outcome and quality of life in children born with congenital diaphragmatic hernia. Arch Dis Child 2009; 94 (11) 834-840