ELİF AĞAÇAYAK, Mehmet ÖZER, ABDULKADİR TURGUT, Ali ÖZLER, SENEM YAMAN TUNÇ

Bi̇lateral Konjeni̇tal Di̇yafragmati̇k Herni̇: Nadi̇r Bi̇r Olgu

Konjenital diyafragma hernisi nedeni bilinmeyen, oldukça nadir görülen bir doğumsal anomalidir. Diğer anomaliler ile ilişkisi ve farklı klinik desenleri ile çeşitli nedenleri olabileceğini düşündürmektedir. Konjenital diyafragma hernisi 2.500 canlı doğumda 1 görülür. Olguların% 85’inde defekt [1] sol taraflıdır. Konjenital diyafragma hernisi vakalarının çoğu sporadiktir. Aileselkonjenital diyafragma hernisi, tüm vakaların sadece % 2‘ sini [2] oluşturur, oldukça nadirdir. Bu konjenital anomali hemen hemen her zaman doğum öncesi ultrasonografik muayene ile tanınabilir. Tedavisi ile sonuçlar herninin derecesine göre değişkenlik gösterir. Bilateral konjenital diyafragma hernisi kötü prognozile, nadir bir doğumsal anomalidir. Biz sağ taraflı konjenital diyafragma hernisi operasyon sırasında keşfedilen bilateral konjenital diyafragma hernisi olgusu sunulmuştur. Diyafragmatik defekt onarıldı ve bir prolen örgü abdominal kompartman sendromu önlemek için karın yara yerleştirildi. Hasta yine de ameliyat sonrası ciddi pulmoner hipertansiyon nedeniyle öldü. Öncelikle olgu sınırlı sayıda olup bilateral konjenital diyafragma hernisi, son derece nadirdir. CDH hasta bakımı yenidoğan ve cerrahlar için çok zordur. Olgumuzu sunmamızın amacı özellikle bilateral konjenital diyafragma hernisi olan hastaların tedavi ve sonuçlarını değerlendirmektir.

Anahtar Kelimeler:

Bilateralkonjenitaldiyafragmahernisi, Pulmoner, hipertansiyon

Bilateral Congenital Diaphragmatic Hernia: A Rare Case Report

Congenital diaphragmatic hernia is a relatively rare birth defect with unknown etiology. Its association with other anomalies and distinct clinical patterns suggest that several causes may be involved. Congenital diaphragmatic hernia occurs in 1 in 2500 live births. In 85% of cases the defect is left-sided [ 1]. Most cases of congenital diaphragmatic herniaare sporadic and familial congenital diaphragmatic herniais rare, comprising only 2% of congenital diaphragmatic herniacases[2]. Thiscongenital anomaly can almost always be recognized with prenatal ultrasound screening. There is a high degree of variability in both treatment and outcomes. Bilateral congenital diaphragmatic hernia is a rare birth defect, with grim prognosis. We describe a case of bilateral congenital diaphragmatic hernia discovered while repartitioning right sided congenital diaphragmatic hernia. The diaphragmatic defect was repaired and a prolene mesh was placed on the abdominal wound to avoid abdominal compartment syndrome. The patient nonetheless died post operatively due to severe pulmonary hypertension. Bilateral congenital diaphragmatic hernia, priorly identified through a limited number of case reports, is extremely rare. The care of congenital diaphragmatic hernia patients is very difficult for neonatologists and surgeons. Our report particularly the management and outcome of patients with bilateral congenital diaphragmatic hernia.

Keywords:

Bilateral congenital diaphragmatic hernia, Pulmonary, hypertension,

PDF

___

Pober BR, Lin A, Russell M et al. 2005. Infants with Bochdalek diaphragmatic hernia sibling precurrence and monozygotic twin discordance in a hospital-based malformation surveillance program. Am J Med Genet A138A:81–88
Song MS, Yoo SJ, Smallhorn JF, Mullen JB, Ryan G, Hornberger LK. Bilateral congenital diaphragmatic hernia: diagnostic clues at fetal sonography. Ultrasound Obstet Gynecol. 2001; 17: 255– 8.
.Furuta Y, Nakamura Y, Miyamoto K. Bilateral congenital posterolateral diaphragmatic hernia. J Pediatr Surg. 1987; 22: 182– 3.
Glüer S, von Schweinitz D. Delayed presentation of a right-sided diaphragmatic hernia following necrotizing enterocolitis: case report. Pediatr Surg Int 1997; 12 (1) 59-60
Jandus P, Savioz D, Purek L, Frey JG, Schnyder JM, Tschopp JM. Bochdalek hernia: a rare cause of dyspnea and abdominal pain in adults. Rev Med Suisse. 2009; 5: 1061– 4.
Poenaru D, Laberge J, Jéquier S, Blanchard P, Doody D. Ultrasound diagnosis of delayed-onset congenital diaphragmatic hernia associated with group B streptococcal infection. Pediatr Surg Int 1988; 3: 66-69.
Kufeji DI, Crabbe DC. Familial bilateral congenital diaphragmatic hernia. Pediatr Surg Int. 1999; 15: 58– 60
Neville HL, Jaksic T, Wilson JM, Lally PA, Hardin WD Jr, Hirschl RB, et al. Bilateral congenital diaphragmatic hernia. J Pediatr Surg. 2003; 38: 522– 4
Zaupa P, Kleinlein B, Höllwarth ME. Bilateral congenital diaphragmatic hernia and gastroschisis in a newborn: can low intrathoracic pressure prevent the pulmonary hypoplasia? Pediatr Surg Int. 2007; 23: 711– 3.
Anjan kumar Dhua, Satish K Aggarwal, NB Mathur, and GR Sethi. Bilateral Congenital Diaphragmatic Hernia. APSP J Case Rep. 2012 Sep-Dec; 3(3): 20