Mehmet Fatih DEVECİ, Meral ALAGÖZ, İsmail Kürşad GÖKCE, Ramazan ÖZDEMİR

Konjenital Diyafragma Hernisi Tanılı Hastalar; 10 Yıllık Tek Merkez Deneyimi

Amaç: Konjenital diyafragma hernisi (KDH), diyafragmanın gelişimsel kusuru olup nadir görülmektedir. Batın içi organların göğüs boşluğuna fıtıklaşması sonucu gelişen akciğer hipoplazisinin şiddeti, hastaların prognozunda temel etkendir. Biz bu çalışmamızda, son 10 yıllık süreçte KDH vakalarımızla ilgili deneyimimizi ve mortalite ile ilgili faktörleri değerlendirmeyi amaçladık. Gereç ve Yöntemler: Ocak 2012-Aralık 2021 tarihleri arasında KDH tanısı ile yatırılan hastaları retrospektif olarak inceledik. Hastaların demografik özellikleri ile klinik izlem bilgilerini değerlendirip, mortalite üzerinde etkili olan faktörleri inceledik. Bulgular: Toplam 37 KDH’li hastamızdan, 22’sinin exitus olduğunu tespit ettik. 19 hastamız başka merkezlerde doğup, tarafımıza sevk edilmişti. Ayrıca 19 hastamızın prenatal tanısı yoktu. Hastalardan 12’si opere edilmeden ilk saatlerde exitus oldu. Exitus olan grupta ek anomali oranı [n=13 (%59.1), n=1 (%6.7) sırasıyla (p=0.002)] istatiksel olarak daha fazlaydı. Opere edilen 25 hastamızdan 15’i taburcu edildi. Opere edilenlerden exitus olan grupta, pulmoner hipertansiyon gelişen hasta sayısı [n=9 (%90), n=4 (%26.7) sırasıyla (p=0.005)] istatiksel olarak daha fazlaydı. Sonuçlar: Pulmoner hipertansiyon ve ek anomali KDH hastalarında mortalite ile ilişkilidir. Bu hastaların doğumlarının uygun merkezde olması ve deneyimli ekiplerce takip ve tedavilerinin yapılması da önemlidir.

Anahtar Kelimeler:

Konjenital diyafragma hernisi, pulmoner hipertansiyon, yenidoğan

Patients with Congenital Diaphragmatic Hernia: 10-Year Single Center Experience

Objective: Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm and is rarely seen. The severity of lung hypoplasia, which develops as a result of herniation of abdominal organs into the chest cavity, is the main factor in the prognosis of patients. In this study, we aimed to evaluate our last10-year experience of CDH cases and mortality-related factors. Materials and Methods: We retrospectively analyzed the patients hospitalized with the diagnosis of CDH between January 2012 and December 2021. We evaluated the demographic characteristics and clinical follow-up information of the patients and examined the factors affecting mortality. Results: We found that out of 37 patients with CDH, 22 of them were died. 19 of our patients were born in other centers and referred to our center. In addition, 19 of our patients did not have a prenatal diagnosis. The rate of additional anomaly in the exitus group was statistically higher [n=13 (59.1%), n=1 (6.7%) respectively (p=0.002)]. 12 of the patients died in the first hours without surgery. 15 of our operated 25 patients were discharged. The number of patients who developed pulmonary hypertension in the exitus group was statistically higher [n=9 (90%), n=4 (26.7%), respectively (p=0.005)]. Conclusion: Pulmonary hypertension and additional anomaly are associated with mortality in patients with CDH. It is also important that these patients are delivered at the appropriate center and followed up and treated by experienced teams.

Keywords:

Congenital diaphragmatic hernia, pulmonary hypertension, newborn,

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Wynn J, Yu L, Chung WK. Genetic causes of congenital diaphragmatic hernia. Semin Fetal Neonatal Med. 2014;19(6):324-330.
The Congenital Diaphragmatic Hernia Study Group. Congenital diaphragmatic hernia: Defect size correlates with developmental defect. J Pediatr Surg. 2013;48(6):1177–1182.
Testini M, Girardi A, Isernia RM, De Palma A, Catalano G, Pezzolla A et al. Emergency surgery due to diaphragmatic hernia: case series and review. World J Emerg Surg. 2017;12:23.
Shieh HF, Barnewolt CE, Wilson JM, Zurakowski D, Connolly SA, Estroff JA et al. Percent predicted lung volume changes on fetal magnetic resonance imaging throughout gestation in congenital diaphragmatic hernia. J Pediatr Surg. 2017;52(6):933-937.
Deprest JA, Nicolaides K, Gratacos E. Fetal surgery for congenital diaphragmatic hernia is back from never gone. Fetal Diagn Ther. 2011;29(1):6–17.
Partridge EA, Peranteau WH, Herkert L, Rendon N, Smith H, Rintoul NE et al. Right- versus left-sided congenital diaphragmatic hernia: A comparative outcomes analysis. J Pediatr Surg. 2016;51(6):900-902.
Botden SM, Heiwegen K, van Rooij IA, Scharbatke H, Lally PA, van Heijst A et al. Bilateral congenital diaphragmatic hernia: Prognostic evaluation of a large international cohort. J Pediatr Surg. Canadian Congenital Diaphragmatic Hernia Collaborative, Puligandla P, Skarsgard E, Offringa M, Adatia I, Baird R, Bailey M et al. Diagnosis and management of congenital diaphragmatic hernia: A clinical practice guideline. CMAJ. 2018;190(4):103-112.
Grizelj R, Bojanic K, Vukovic J, Weingarten TN, Schroeder DR, Sprung J. Congenital diaphragmatic hernia: The side of diaphragmatic defect and associated nondiaphragmatic malformations. Am J Perinatol. 2017;34(9):895-904.
Kailin J, Dhillon G, Maskatia S, Cass DL, Shamshirsaz AA, Mehollin-Ray AR et al. Fetal left-sided cardiac structural dimensions in left-sided congenital diaphragmatic hernia - association with severity and impact on postnatal outcomes. Prenat Diagn. 2017;37(5):502-509.
McGivern MR, Best KE, Rankin J, Wellesley D, Greenlees R, Ador MC et al. Epidemiology of congenital diaphragmatic hernia in Europe: a register-based study. Arch Dis Child Fetal Neonatal Ed. 2015;100(2):137-144.
Chandrasekharan PK, Ravat M, Madappa R, Rothstein DH, Lakshminrusimha S. Congenital Diaphragmatic hernia-A review. Matern Health Neonatol Perinatol. 2017;3:6.
Datin-Dorriere V, Walter-Nicolet E, Rousseau V, Taupin P, Benachi A, Parat S et al. Experience in the management of eighty-two newborns with congenital diaphragmatic hernia treated with high-frequency oscillatory ventilation and delayed surgery without the use of extracorporeal membrane oxygenation. J Intensive Care Med. 2008;23(2):128-135.
Snoek KG, Reiss IK, Greenough A, Capolupo I, Urlesberger B, Wessel L et al. Standardized Postnatal Management of Infants with Congenital Diaphragmatic Hernia in Europe: The CDH EURO Consortium Consensus-2015 Update. Neonatology. 2016;110(1):66-74.
Deeney S, Howley LW, Hodges M, Liechty KW, Mervan A, Gien J et al. Impact of Objective Echocardiographic Criteria for Timing of Congenital Diaphragmatic Hernia Repair. J Pediatr. 2018;192:99-104.
Wehrmann M, Patel SS, Haxel C, Cassidy C, Howley L, Cuneo B et al. Implications of Atrial-Level Shunting by Echocardiography in Newborns with Congenital Diaphragmatic Hernia. J Pediatr. 2020;219:43-47.
Putnam LR, Tsao K, Morini F, Lally PA, Miller CC, Lally KP et al. Evaluation of variability in ınhaled nitric oxide use and pulmonary hypertension in patients with congenital diaphragmatic hernia. JAMA Pediatr. 2016;170(12):1188-1194.
Gupta VS, Harting MT. Congenital diaphragmatic hernia-associated pulmonary hypertension. Semin Perinatol. 2020;44(1):151167.
Patel N, Masolo AC, Kipfmueller F. Congenital diaphragmatic hernia-associated cardiac dysfunction. Semin Perinatol.2020;44(1):151168.