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Singapore Med J 2013; 54(8): 432-436; http://dx.doi.org/10.11622/smedj.2013149. Addendum in: Singapore Med J 2013; 54(11): 661.
Antenatally diagnosed congenital diaphragmatic hernia in Singapore: a five-year series
Lee SY, Tan KH
Correspondence: Dr Lee Shu Yi Sonia, sonia.lee@mohh.com.sg
ABSTRACT
INTRODUCTION We aimed to investigate the epidemiology, diagnosis, prognostication, follow-up care and outcomes of antenatally diagnosed congenital diaphragmatic hernia (CDH) in KK Women’s and Children’s Hospital (KKH), Singapore. The objective of this study was to identify trends in current practice, and evaluate and improve the management of CDH.
METHODS We retrospectively reviewed cases of antenatally diagnosed CDH from 2006 to 2010.
RESULTS A total of 22 cases of CDH were found, bringing its incidence in KKH to 3.6 per 10,000 live births. The mean gestational age at diagnosis was 22.7 weeks, with 14 (63.6%) cases diagnosed at < 22 weeks and 8 (36.4%) diagnosed at ≥ 22 weeks. All cases were left-sided – 15 (68.2%) were isolated CDH and 7 (31.8%) were associated with other anomalies. Of the 22 cases, counselling about the prognosis of pregnancy based on the lung-to-head ratio was provided in 9 (40.9%). Overall, 10 (45.5%) cases resulted in termination of pregnancy (TOP), 9 (40.9%) resulted in live birth and 1 (4.5%) in stillbirth; 2 (9.1%) cases were lost to follow-up prior to delivery. Of the 9 live births, 3 (33.3%) resulted in neonatal death. In the 10 births, 5 (50.0%) were delivered by normal vaginal delivery, 4 (40.0%) by emergency Caesarean section and 1 (10.0%) by elective Caesarean section.
CONCLUSION CDH is a challenging perinatal problem with a low overall survival rate. Almost half of the cases opt for TOP. The data in this study can help clinicians better undertake the task of adequately counselling parents with qualitative and quantitative prognostic factors, using an evidence-based approach.
Keywords: antenatal diagnosis, congenital diaphragmatic hernia, epidemiology, outcome, prognosis
Singapore Med J 2013; 54(8): 432-436; http://dx.doi.org/10.11622/smedj.2013149
REFERENCES
| 1. Skari H, Bjomland K, Haugen G, Egeland T, Emblem R. Congenital diaphragmatic hernia: a meta-analysis of mortality factors. J Pediatr Surg 2000; 35:1187-97. http://dx.doi.org/10.1053/jpsu.2000.8725 | ||||
| 2. Thébaud B, Mercier JC, Dinh-Xuan AT. Congenital diaphragmatic hernia. A cause of persistent pulmonary hypertension of the newborn which lacks an effective therapy. Biol Neonate 1998; 74:323-36. | ||||
| 3. Fitzgibbons RJ, Greenburg AG, eds. Nyhus and Condon's Hernia. 5th ed. Philadelphia: Lippincott Williams & Wilkins, 2002. | ||||
| 4. Morgagni G. The Seats and Causes of Diseases Investigated by Anatomy. London: Millar and Cadell, 1960. | ||||
| 5. Game E, Haeusler M, Barisic I, et al. Congenital diaphragmatic hernia: evaluation of prenatal diagnosis in 20 European regions. Ultrasound Obstet Gynecol 2002; 19:329-33. http://dx.doi.org/10.1046/j.1469-0705.2002.00635.x | ||||
| 6. Grisaru-Granovsky S, Rabinowitz R, Ioscovich A, Elstein D, Schimmel MS. Congenital diaphragmatic hernia: review of the literature in reflection of unresolved dilemmas. Acta Paediatr 2009; 98:1874-81. http://dx.doi.org/10.1111/j.1651-2227.2009.01436.x | ||||
| 7. Beck C, Alkasi O, Nikischin W, et al. Congenital diaphragmatic hernia, etiology and management, a 10-year analysis of a single center. Arch Gynecol Obstet 2008; 277:55-63. http://dx.doi.org/10.1007/s00404-007-0407-4 | ||||
| 8. Romero R. Routine obstetric ultrasound. Ultrasound Obstet Gynecol 1993; 3:303-7. http://dx.doi.org/10.1046/j.1469-0705.1993.03050303.x | ||||
| 9. Tan KH, Tan TY, Tan J, et al. Birth defects in Singapore: 1994-2000. Singapore Med J 2005; 46:545-52. | ||||
| 10. Jani J, Keller RL, Benachi A, et al. Prenatal prediction of survival in isolated left-sided diaphragmatic hernia. Ultrasound Obstet Gynecol 2006; 27:18-22. http://dx.doi.org/10.1002/uog.2688 | ||||
| 11. Wright JC, Budd JL, Field DJ, Draper ES. Epidemiology and outcome of congenital diaphragmatic hernia: a 9-year experience. Paediatr Perinat Epidemiol 2011; 25:144-9. http://dx.doi.org/10.1111/j.1365-3016.2010.01172.x | ||||
| 12. Stege G, Fenton A, Jaffray B. Nihilism in the 1990s: the true mortality of congenital diaphragmatic hernia. Pediatrics 2003; 112:532-5. http://dx.doi.org/10.1542/peds.112.3.532 | ||||
| 13. Dillon E, Renwick M. Antenatal detection of congenital diaphragmatic hernias: the northern region experience. Clin Radiol 1993; 48:264-7. http://dx.doi.org/10.1016/S0009-9260(05)81015-3 | ||||
| 14. Kaiser JR, Rosenfeld CR. A population-based study of congenital diaphragmatic hernia: impact of associated anomalies and preoperative blood gases on survival. J Pediatr Surg 1999; 34:1196-202. http://dx.doi.org/10.1016/S0022-3468(99)90151-3 | ||||
| 15. Yang W, Carmichael SL, Harris JA, Shaw GM. Epidemiologic characteristics of congenital diaphragmatic hernia among 2.5 million California births, 1989-1997. Birth Defects Res A Clin Mol Teratol 2006; 76:170-4. http://dx.doi.org/10.1002/bdra.20230 | ||||
| 16. Dott MM, Wong LY, Rasmussen SA. Population-based study of congenital diaphragmatic hernia: risk factors and survival in Metropolitan Atlanta, 1968-1999. Birth Defects Res A Clin Mol Teratol 2003; 67:261-7. http://dx.doi.org/10.1002/bdra.10039 | ||||
| 17. Levison J, Halliday R, Holland AJ, et al for Neonatal Intensive Care Units Study of the NSW Pregnancy and Newborn Services Network. A population-based study of congenital diaphragmatic hernia outcome in New South Wales and the Australian Capital Territory, Australia, 1992-2001. J Pediatr Surg 2006; 41:1049-53. http://dx.doi.org/10.1016/j.jpedsurg.2006.01.073 | ||||
| 18. Colvin J, Bower C, Dickinson JE, Sokol J. Outcomes of congenital diaphragmatic hernia: a population-based study in Western Australia. Pediatrics 2005; 116:356-63. http://dx.doi.org/10.1542/peds.2004-2845 | ||||
| 19. Shaw KS, Filiatrault D, Yazbeck S, St-Vil D. Improved survival for congenital diaphragmatic hernia, based on prenatal ultrasound diagnosis and referral to a combined obstetric-pediatric surgical center. J Pediatr Surg 1994; 29:1268-9. http://dx.doi.org/10.1016/0022-3468(94)90821-4 | ||||
| 20. Gallot D, Coste K, Francannet C, et al. Antenatal detection and impact on outcome of congenital diaphragmatic hernia: a 12-year experience in Auvergne, France. Eur J Obstet Gynecol Reprod Biol 2006; 125:202-5. http://dx.doi.org/10.1016/j.ejogrb.2005.06.030 | ||||
| 21. Okuyama H, Kitano Y, Saito M, et al. The Japanese experience with prenatally diagnosed congenital diaphragmatic hernia based on a multi-institutional review. Pediatr Surg Int 2011; 27:373-8. http://dx.doi.org/10.1007/s00383-010-2805-0 | ||||
| 22. Geary MP, Chitty LS, Morrison JJ, et al. Perinatal outcome and prognostic factors in prenatally diagnosed congenital diaphragmatic hernia. Ultrasound Obstet Gynecol 1998; 12:107-11. http://dx.doi.org/10.1046/j.1469-0705.1998.12020107.x | ||||
| 23. Safavi A, Lin Y, Skarsgard ED; Canadian Pediatric Surgery Network. Perinatal management of congenital diaphragmatic hernia: when and how should babies be delivered? Results from the Canadian Pediatric Surgery Network. J Pediatr Surg 2010; 45:2334-9. http://dx.doi.org/10.1016/j.jpedsurg.2010.08.026 | ||||
| 24. Bétrémieux P, Lionnais S, Beuchée A, et al. Perinatal management and outcome of prenatally diagnosed congenital diaphragmatic hernia: a 1995-2000 series in Rennes University Hospital. Prenat Diagn 2002; 22:988-94. http://dx.doi.org/10.1002/pd.454 | ||||
| 25. Bedoyan JK, Blackwell SC, Treadwell MC, Johnson A, Klein MD. Congenital diaphragmatic hernia: associated anomalies and antenatal diagnosis. Outcome-related variables at two Detroit hospitals. Pediatr Surg Int 2004; 20:170-6. http://dx.doi.org/10.1007/s00383-004-1138-2 | ||||
| 26. Zaiss I, Kehl S, Link K, et al. Associated malformations in congenital diaphragmatic hernia. Am J Perinatol 2011; 28:211-8. http://dx.doi.org/10.1055/s-0030-1268235 | ||||
| 27. Fauza DO, Wilson JM. Congenital diaphragmatic hernia and associated anomalies: their incidence, identification and impact on prognosis. J Pediatr Surg 1994; 29:1113-7. http://dx.doi.org/10.1016/0022-3468(94)90290-9 | ||||
| 28. Lipshutz GS, Albanese CT, Feldstein VA, et al. Prospective analysis of lung-to-head ratio predicts survival for patients with prenatally diagnosed congenital diaphragmatic hernia. J Pediatr Surg 1997; 32:1634-6. http://dx.doi.org/10.1016/S0022-3468(97)90471-1 | ||||
| 29. Flake AW, Crombleholme TM, Johnson MP, Howell LJ, Adzick NS. Treatment of severe congenital diaphragmatic hernia by fetal tracheal occlusion: clinical experience with fifteen cases. Am J Obstet Gynecol 2000; 183:1059-66. http://dx.doi.org/10.1067/mob.2000.108871 | ||||
| 30. Laudy JA, Van Gucht M, Van Dooren MF, Wladimiroff JW, Tibboel D. Congenital diaphragmatic hernia: an evaluation of the prognostic value of the lung-to-head ratio and other prenatal parameters. Prenat Diagn 2003; 23:634-9. http://dx.doi.org/10.1002/pd.654 | ||||
| 31. Ba'ath ME, Jesudason EC, Losty PD. How useful is the lung-to-head ratio in predicting outcome in the fetus with congenital diaphragmatic hernia? A systematic review and meta-analysis. Ultrasound Obstet Gynecol 2007; 30:897-906. http://dx.doi.org/10.1002/uog.5164 | ||||
| 32. Jani J, Nicolaides KH, Benachi A, et al. Timing of lung size assessment in the prediction of survival in fetuses with diaphragmatic hernia. Ultrasound Obstet Gynecol 2008; 31:37-40. http://dx.doi.org/10.1002/uog.5198 | ||||
| 33. Adzick NS, Harrison MR, Glick PL, et al. Diaphragmatic hernia in the fetus: prenatal diagnosis and outcome in 94 cases. J Pediatr Surg 1985; 20:357-61. http://dx.doi.org/10.1016/S0022-3468(85)80219-0 | ||||
| 34. Thorpe-Beeston JG, Gosden CM, Nicolaides KH. Prenatal diagnosis of congenital diaphragmatic hernia: associated malformations and chromosomal defects. Fetal Ther 1989; 4:21-8. http://dx.doi.org/10.1159/000263386 | ||||
| 35. Brownlee EM, Howatson AG, Davis CF, Sabharwal AJ. The hidden mortality of congenital diaphragmatic hernia: a 20-year review. J Pediatr Surg 2009; 44:317-20. http://dx.doi.org/10.1016/j.jpedsurg.2008.10.076 | ||||