LAPSUSMEDICINA 2018, Volume 49, Number 2: 251-256

P-ISSN.2540-8313, E-ISSN.2540-8321

251

CrossMark

ABSTRACT

Congenital diaphragmatic hernia (CDH) is an anomaly characterized by a discontinuity of the diaphragm, which allows the abdominal viscera to herniate into the chest during embryonic and fetal development. Almost certainly fatal if untreated. Hernia repair is now widely accepted and has become established as the primary treatment for CDH. There were three cases identified during six months that were diagnosed with CDH. These patients with respiratory distress, and

radiology and laboratory evidence of CDH, need urgent surgical exploration of the hernia. These patients underwent hernia repair. All of the operations were succeeded although all experienced early post-operative complications, pneumothorax and pleural effusion. These patients were received different mode of ventilation. One patient died, while the others survived. The prognosis of these patients were poor without hernia repair.

Keywords: congenital diaphragmatic hernia, hernia repair, childrenCite This Article: Swandewi, L.P., Artana, I.W.D., Sukmawati, M., Darmajaya, I.M., Arianta, K.D. 2018. Congenital diaphragmatic hernia: three cases after hernia repair. Medicina 49(2): 251-256. DOI:10.15562medicina.v49i2.11

ABSTRAK

Hernia diafragma kongenital merupakan kelainan yang ditandai oleh diskontinuitas diafragma sehingga organ-organ abdomen masuk ke rongga dada yang terjadi pada masa perkembangan embrio dan fetus. Kelainan ini berakibat fatal bila tidak segera ditangani. Operasi perbaikan hernia saat ini sudah umum dikerjakan dan menjadi terapi utama bagi hernia diafragma kongenital. Terdapat tiga kasus hernia diafragma kongenital. Pasien-pasien tersebut mengalami gangguan pernapasan,

serta bukti-bukti pencitraan dan laboratorium tentang hernia diafragma kongenital yang memerlukan pembedahan segera. Ketiga pasien menjalani operasi hernia. Seluruh operasi berjalan sukses walaupun mengalami komplikasi awal pascaoperasi yaitu pneumotoraks dan efusi pleura. Ketiga pasien tersebut mendapat jenis ventilasi yang berbeda. Satu pasien meninggal sedangkan yang lainnya selamat. Prognosis ketiga pasien tersebut buruk apabila tidak menjalani operasi.

Kata kunci: hernia diafragma kongenital, operasi hernia, anak-anakCite Pasal Ini: Swandewi, L.P., Artana, I.W.D., Sukmawati, M., Darmajaya, I.M., Arianta, K.D. 2018. Congenital diaphragmatic hernia: three cases after hernia repair. Medicina 49(2): 251-256. DOI:10.15562medicina.v49i2.11

Congenital diaphragmatic hernia: three cases after hernia repair

Luh Putu Swandewi,1* I Wayan Darma Artana,1 Made Sukmawati,1 I Made Darmajaya,2 Kadek Deddy Arianta2

INTRODUCTION

Congenital diaphragmatic hernia (CDH) is an anomaly characterized by a discontinuity of the diaphragm, which allows the abdominal viscera to herniate into the chest during embryonic and fetal development. Congenital diaphragmatic hernia is a significant cause of neonatal morbidity and mortal-ity that is often seen as a purely pediatric and surgi-cal problem.1 Globally, CDH is estimated to occur in 1 of every 3,000 live births. The true incidence remains unknown because of early death among severely affected fetuses and infants.2

Morgagni described the hiatal hernia through the foramina of Morgagni in 1761. In 1848, Bochdalek referenced the development of the pleu-roperitoneal canals while describing the hernia that

formed by passing through these canals.3 The most influential surgical approach for CDH came from William Ladd and Robert Gross which advocated rapid diagnosis by chest X-ray and/or barium meal with early operative intervention.4

Congenital diaphragmatic hernia is almost certainly fatal. Pulmonary hypoplasia, a hypoplastic left heart ventricle, and persistent pulmonary hypertension of the newborn (PPHN), with retention of the fetal circulation, lead to marked respiratory distress. Despite treatment in intensive care, survival is only 40-60% worldwide, or up to 80% in specialist centers. Early intrauterine diagno-sis is important.5 Unfortunately, in Indonesia, CDH often forgotten and accidentally diagnosed after

1Department of Child Health and 2Pediatric SurgeryUdayana University Medical School, Sanglah Hospital

*Correspondence to: Luh Putu Swandewi, Department of Child Health, Udayana University Medical School, Sanglah Hospital lpswandewi@gmail.com

Diterima: 2017-06-21 Disetujui: 2018-03-23 Diterditkan: 01-08-2018

Volume No.: 49

Issue: 2

First page No.: 251

P-ISSN.2540-8313

E-ISSN.2540-8321

Doi: http://dx.doi.org/10.15562/medicina.v49i2.11

LAPSUS

252 Medicina 2018; 49(2): 251-256 | doi: 10.15562medicina.v49i2.11

LAPSUS

birth. The purpose of this article is to remind and compare the evaluation, management, and deter-minant of outcome three patients with CDH who underwent hernia repair.

CASE ILLUSTRATION

Over a period of six months from April to September 2016, a total of three patients with CDH were considered retrospectively. Of these, two patients were survive and one patient was died. All of them presented as respiratory distress soon after birth.

First caseEKB, a three-hour-old baby, was referred to Sanglah Hospital because of respiratory distress after birth. He had already intubated when admitted to emer-gency room. He was born through cesarean section on the 38th week of gestation. Apgar scores were 6 and 7 at 1 and 5 minutes respectively. He began breathless, cyanotic and the chest wall became retracted predominantly on the right side.

There were tachycardia, tachypnea, nasal flare, decreased of respiratory sound on the left side accompanied by displacement of the heart sounds to the right side of the chest. The chest wall was asymmetrical on movement. Downes score was 8. The abdomen was excavated with sternal protru-sion, no bowel sound, the liver and spleen were not palpable. Complete blood count, C-reactive protein (CRP) and immature to total (IT) ratio were normal. The chest X-ray showed heart pushed toward right side of chest, multiple cystic lesion in left lung (Figure 1). Echocardiography was normal.

He was diagnosed with respiratory distress and CDH. He was fasting and decompressive enteric tube was inserted by mouth. He admit-ted to Neonatal Intensive Care Unit (NICU) and started on pressure-controlled ventilation. Blood gas analysis 2 hours after ventilation was normal. He underwent urgent hernia repair at 4 days old. Intraoperative findings revealed a left posterolat-eral diaphragmatic defect with ileum, colon, stom-ach and spleen found in the hernial sac (Figure 2) and definitely diagnosed with left Bochdalek type diaphragmatic hernia. Chest X-ray a day after hernia repair assumed minimal pleural effusion and pneumonia.

Two days after surgery, he got fever and jaun-dice and the antibiotics were changed. Ventilator support was stopped 3 days after surgery. The blood culture was positive and antibiotic continued until 14 days before he discharged from hospital.

Second caseKSB, a newborn baby, suffered respiratory distress soon after birth in Sanglah Hospital. There was no spontaneous breath and he was cyanotic. He was born through vaginal delivery on the 37th week of gestation. Apgar scores 2, 4, 6, and 8 at 1, 5, 10 and 20 minutes respectively. Soon after birth, he was intubated along with chest compression. After 10 minutes, he began to breathe spontaneously. The Downes score was 8.

There were tachycardia with decreased respi-ratory sounds on the left side. The chest wall was asymmetrical on movement. The abdomen was excavated and no bowel sound was heard. The liver and spleen were not palpable.

Complete blood count, CRP, IT ratio, and blood glucose were normal. Prothrombin time, activated partial thromboplastin time, and international normalized ratio (INR) were slight prolonged. Chest X-ray revealed heart pushed toward the right side of chest and bowel gas appeared in the whole field of left thorax. The chest X-ray assumed left diaphragmatic hernia (Figure 3).

He was diagnosed with severe asphyxia, respira-tory distress, and CDH. He got antibiotics, fasting

Figure 1 Preoperative chest X-ray

Figure 2 Hernia repair

253Medicina 2018; 49(2): 251-256 | doi: 10.15562medicina.v49i2.11

LAPSUS

and decompressive enteric tube was inserted by mouth. Reddish gastric juices was came out from the enteric tube. He admitted to NICU and started on pressure-controlled ventilation. Blood gas anal-ysis 2 hour after intubated showed severe acidosis. He underwent urgent hernia repair at 2 days old that revealed a left posterolateral diaphragmatic

Figure 3 Preoperative chest X-ray

Figure 4 Hernia repair

Figure 5 Preoperative chest X-ray

Figure 6 Postoperative chest X-ray

Figure 7 Development of diaphragm6

Figure 8 Location of diaphragmatic hernia6

254 Medicina 2018; 49(2): 251-256 | doi: 10.15562medicina.v49i2.11

LAPSUS

defect with small intestines, stomach and spleen found in the hernial sac (Figure 4). He definitely diagnosed with left Bochdalek type diaphragmatic hernia.

Postoperative chest X-ray showed left pneumo-thorax, minimal left pleural effusion differential diagnosis with hematothorax. A day after surgery, he got jaundice and planned for phototherapy with worsening of septic marker results. Four days after surgery, he experienced temperature instability and shock with oxygen desaturation many times a day. Fluid resuscitation was given and dobutamine was added as an inotropic support. The ventilation mode was changed to high-frequency oscillation ventilation (HFOV). He died 5 days after surgery because of severe sepsis.

Third caseMWB, a seven-hour-old baby, was referred to Sanglah Hospital because of respiratory distress after birth. He was born through cesarean section on the 41st week of gestation because of breech posi-tion. He was grunting, cyanotic around his mouth, and tachypnea. Apgar scores 4, 5, and 7 at 1, 5, and 10 minutes respectively.

Tachycardia accompanied with decreased of respiratory sounds on the left side of thorax. The chest wall was asymmetrical on movement. Downes score was 4. The fontanels were tense. The abdomen was excavated. The bowel sound was not heard. The liver and spleen were not palpable.

Complete blood count and CRP were normal with slight increase of IT ratio. Chest X-ray showed multiple radiolucent bubbles in the left hemithorax with suspicion of left CDH differential diagnosis with cystic lung disease (Figure 5). He also under-went head computed tomography (CT) scan that revealed: minimal subdural hemorrhage in right occipital and minimal subarachnoid hemorrhage in posterior subfalcine.

He was fasting and decompressive enteric tube was inserted by mouth. He admitted to NICU and started on HFOV. Blood gas analysis showed respi-ratory acidosis. According to neurologic surgeon, no specific treatment was needed for the intracra-nial hemorrhage. He got hernia repair at 9 days old that revealed a left posterolateral diaphragmatic defect with organs included within the hernial sac were duodenum, jejunum and ileum and definitely diagnosed with left bochdalek type diaphragmatic hernia. After surgery, he did chest X-ray that assumed left pneumothorax (Figure 6).

Two days after surgery he got jaundice and the septic marker worsen. The antibiotic was changed. Five days after surgery, ventilation support weaned into continuous positive airway pressure setting for

two days and continued to wean. He discharged from hospital after the antibiotic was stopped.

DISCUSSION

The diaphragm develops during weeks 4-12 of embryogenesis. It is composed of four components: the transverse septum, pleuroperitoneal folds, esophageal mesentery, and muscular body wall. A defect in fusion of the transverse septum to the lateral body wall leads to an anterior (Morgagni) hernia. Morgagni hernia constitute fewer than 10% of CDH. A posterior (Bochdalek) hernia represents a developmental defect of the pleuroperitoneal folds or failure of fusion of the folds and transverse septum with the intercostal muscles. Bochdalek hernia constitute 90% of CDH and more common on the left side (Figure 7 and Figure 8).6,7

Neonates with symptomatic CDH, usually pres-ent with respiratory distress and cyanosis in the first few minutes to hours after birth with scaphoid abdomen, barrel-shaped chest, increased work of breathing, decreased aeration over the ipsilateral chest, with heart tones shifted to the contralateral side. Bowel sounds may be heard in the chest. Following delivery, swallowed air leads to intestinal distention that worsens lung compression. In severe cases, APGAR score at 1 and 5 minutes are low.2,3

Using ultrasonography, most cases are identified prenatally. The characteristic finding of left-sided CDH is detection of the fluid-filled stomach within the lower thorax. Right-sided CDH is more diffi-cult to diagnose antenatally because the herniated viscera consists predominantly of the right lobe of the liver, which has a similar echogenicity to the fetal lung.2 The lung-to-head ratio (LHR) is the most used prenatal predictor of survival in fetuses who have CDH. Furthermore, LHR may also dependent on the gestational age at measurement and may be less reliable in mid-gestation.8 In these cases, all were diagnosed postnatally because of respiratory distress with later suspicion to CDH.

A chest radiograph should be performed for the investigation of CDH. Chest X-ray had 100% sensitivity and 100% specificity to diagnose CDH.9 This will demonstrate an opacified hemithorax with a contralateral shift of the mediastinum. Bowel gas may be seen in the chest.2,10

Echocardiography should be undertaken to exclude congenital cardiac lesions in infants with CDH and in any infant in whom pulmo-nary hypertension is suspected. It is important to determine right ventricular function, as the function of the right ventricle under increased afterload is an important determinant of illness severity.2,10 Early resolution of persistence of

255Medicina 2018; 49(2): 251-256 | doi: 10.15562medicina.v49i2.11

LAPSUS

pulmonary hypertension in 2 weeks, is an indica-tor of mild disease, with almost universally good outcomes.11 In these cases, one patient underwent echocardiography.

Immediate postnatal endotracheal intubation and mechanical ventilation can avoid the risk of intestinal distention. Bag-valve-mask ventilation should be avoided. An adequate-sized nasogastric tube should be placed and connected to contin-uous suction to allow intestinal compression. In the NICU, continuous pre- and post-ductal pulse oximetry is used to assess the degree of PPHN via right-to-left shunting at the level of the ductus arteriosus.2,12,13 In these cases, all of the patients had already intubated soon after delivery and also had decompressive nasogastric tube placement.

Surgical repair of the diaphragmatic defect should be performed after clinical stabilization, defined as mean arterial blood pressure normal for gestation, preductal saturation levels of 85-95% on FiO2 below 50%, lactate below 3 mmol/l, urine output more than 1 ml/kg/hour. Surgical repair can be performed while the patient is on ECMO.7 Repair via laparotomy or thoracotomy is the tradi-tional treatment for patients with CDH. Since the first report by Silen et al (cited from 14)., minimally invasive surgery (MIS) techniques (laparoscopic and thoracoscopic) have been considered as alter-native approaches for CDH repair. Although the evident was insufficient, endoscopic surgery was clearly associated with more recurrence than was open surgery. Therefore, endoscopic surgery should not be the routine treatment for every neonate.15 In these cases, all patients underwent open surgery. None of them got ECMO because of limitation in our facility.

High-frequency oscillatory ventilation has also been utilized in the perinatal management of CDH both as a “rescue therapy” prior to ECMO and as a primary ventilatory modality in an attempt to reduce pulmonary barotrauma.13 Snoek et  al16 found that there was no statistically significant difference in the combined outcome of mortality or bronchopulmonary dysplasia between HFOV and conventional ventilation groups in prenatally diagnosed CDH infants. In these cases, the second and the third cases treated with HFOV, and the first case was treated with conventional mechanical ventilation.

The leading cause of CDH related morbidity and mortality is respiratory failure resulting from pulmonary hypoplasia and pulmonary hyper-tension.17 Pneumothorax or chylothorax is the most common early post-operative complication. Diagnosis of a pneumothorax on a chest X-ray should be made with caution. The lung, on the side of a repaired CDH, usually become hypoplasia

and will not fill the hemithorax thereby giving the false impression of a pneumothorax. Post-operative effusions are common, e.g. chylothorax (28% in some series), but no routine chest tube placement postoperatively. The majority of effusions are small. Overall recurrence rates are approximately 15% in the first two years of life. Risk factors for recurrence include large defect size and the need for a patch.12,13 In these cases, the first and second cases had pleural effusion and pneumothorax, and the third case had pneumothorax only. None of the cases had postop-erative chest tube placement. The second case was died five days and the other two cases were alive. A careful monitoring of recurrence was commended for the survivors.

SUMMARY

Three cases of CDH were reported. The diagnosis was established based on clinical manifestation, laboratory investigation, and intraoperative. One case died. All of the cases suffered from complica-tions such as pleural effusion and pneumothorax but no one need additional chest tube placement. The survivor prognosis were good because of better preoperative conditions.

REFERENCES1. Balayla J, Abenhaim HA. Incidence, predictors and out-

comes of congenital diaphragmatic hernia: a popula-tion-based study of 32 million births in the United States. J Matern Fetal Neonatal Med. 2013;27:1-7.

2. Benjamin JR, Bizzarro MJ, Cotton CM. Congenital dia-phragmatic hernia: updates and outcomes. NeoReviews. 2011;12(8):e439-51.

3. Shehata BM, Lin J. Congenital diaphragmatic hernia with emphasis on embryology, subtypes, and molecular genet-ics. Intechopen. 2012;1:3-18.

4. Davenport M, Rothenberg SS, Crabbe DCG, Wulkan ML. The great debate: open or thoracoscopic repair for oesoph-ageal atresia or diaphragmatic hernia. Journal of pediatric Surgery. 2015;50:240-6.

5. Wessel LM, Fuchs J, Rolle U. The surgical correction of congenital deformities. Deutsches Arzteblatt International. 2015;112:357-64.

6. Nason LK, Walker CM, McNeely MF, Burivong W, Fligner CL, Godwin JD. Imaging of the diaphragm: anatomy and function. RadioGraphics. 2012;32:E51-70.

7. Merrell AJ, Kardon G. Development of the diaphragm-a skeletal muscle essential for mammalian respiration. FEBS Journal. 2013;280:4026-35.

8. Zhu Y, Wu Y, Pu Q, Ma L, Liao H, Liu L. Minimally invasive surgery for congenital diaphragmatic hernia: a meta-analysis. Hernia. 2016;20:297-302.

9. Shahri HMM, Naghibi S, Mahdavi E, Khademi G. Diagnostic utility of chest X-rays in neonatal respira-tory distress: determining the sensitivity and specificity. International Journal of Pediatrics. 2014;2(6):65-72.

10. Kotecha S, Barbato A, Bush A, Claus F, Davenport M, Delacourt C, et al. Congenital diaphragmatic hernia. Eur Respir J. 2012;39:820-9.

11. Wynn J, Krishnan U, Aspelund G, Zhang Y, Duong J, Stolar CJH, et  al. Outcomes of congenital diaphragmatic hernia in the modern era of management. J Pediatr. 2013;163(1):114-9.

256 Medicina 2018; 49(2): 251-256 | doi: 10.15562medicina.v49i2.11

LAPSUS

12. Mohamed MA, Aly H. Birth region, race and sex may affect the prevalence of congenital diphragmatic hernia, abdominal wall and neural tube defects among US new-borns. Journal of Perinatology. 2012;31:861-8.

13. Peyvastesh M, Dehdashtian M, Javaherizadeh H, Askarpour S, Ranjbar L, Khodadadi M. A study of the epidemiology and outcomes of patients with congenital diaphragmatic hernia. The Internet Journal of Surgery. 2009;26(1): 1-5.

14. Van den Hout L, Shuiter I, Gischler S, De Klein A, Rottier  R. Can we improve outcome of congenital dia-phragmatic hernia? Pediatr Surg Int. 2009:25:733-43.

15. Terui K, Nagata K, Ito M, Yamoto M, Shiraishi M, Taguchi T, et  al. Surgical approaches for neonatal congenital dia-phragmatic hernia: a systematic review and meta-analysis. Pediatric Surgery International. 2015;31:891-7.

16. Snoek KG, Reiss IKM, Greenough A, Capolupo I, Urlesberger B, Wessel L, et al. Standardized postnatal man-agement of infants with congenital diaphragmatic hernia in europe: the CDH EURO consortium consensus-2015 update. Neonatology. 2016;110:66-74.

17. Lusk LA, Wai KC, Moon-Grady AJ, Steurer MA, Keller RL. Persistence of pulmonary hypertension by echocardiogra-phy predicts short-term outcomes in congenital diaphrag-matic hernia. J Pediatr. 2015;166(2):251-6.

This work is licensed under a Creative Commons Attribution