Congenital Diaphragmatic Hernia

Grand Rounds 5/20/11

Christopher Broussard, MD

Lecture Outline

• Case Report• Pathophysiology• Management• Perioperative Planning• Board Review

Example Case

• Should we fix it immediately?

• Not so fast

Case Presentation

• Pt born via C/S at 39 3/7 weeks• Apgars 5&7• Pregnancy complicated by placenta previa • Third trimester ultrasound demonstrated

polyhydramnios - not CDH• Pt born in outlying community hospital, stabilized

and transferred to Tulane.• Large diaphragmatic hernia with R Lung collapse

on CXR.

Case Presentation

• DOL #1• PSV• ABG 7.18/62/78.6/24/-6.5 on 90% O2• A-a gradient 500mmHg• Echo: PDA with R L shunt, PFO, PASP

68mmHg (suprasystemic), enlarged RA and RV.

Case Presentation

• DOL # 2• ECMO started in the NICU• Treatment with NO• Pulmonary vasodilators

– Sildenafil – Bosentan

• Dobutamine

Overview of CDH

• Incidence - 1/2500 – 1/5000• Male/Female ratio – 2:1• Left/Right – 5:1• Types

– Posterolateral (Bochdalek): 80%– Anterior (Morgagni): 2%– Paraesophageal: 15-20%

Embryologic Development

• Diaphragm, lungs and GI tract begin development at 4 weeks.

• Pleuroperitoneal canal separate by 9th week• Rotation and settling of midgut before

fusion of diaphragm• Limited thoracic space for lung growth• Genetic vs. environmental

Review of Fetal Circulation

• High resistance pulmonary circuit

• Low resistance systemic circuit

• R > L shunt via foramen ovale and ductus arteriosus

Pathophysiology

Pathophysiology

• Pulmonary Hypertension– Pulmonary hypoplasia– Thickened tunica media and adventita– Hypoxemia, hypercarbia and acidosis– Inflammatory mediators (TxA2, LT C4 & D4)

• Suprasystemic pulmonary pressures R to L shunt via PDA and PFO

• Poor cardiac output due to small LV

CHD SIDS

Diagnosis

• Typically prenatal (40%-60% by Ultrasound)

• Classic Triad – cyanosis, dyspnea, apparent dextrocardia.

• Scaphoid abdomen• Bulging chest• Variable Presentation

Immediate Management

• Determine adequacy of ventilation• Gastric decompression• Limit mask ventilation• Intubation• Gentle ventilation

Associated Abnormalities

• 10-40% overall, often coexist • Cardiovascular 13-23%• CNS 28%• GI 20%• GU 15%

Migliazza L, et al. J Pediatr Surg 2007

Prognosis

• Mortality 20-60%• Stege G, et al. Pediatrics 2003

– Case selection bias– Additional defects – 79% v 30%

• Prenatal findings– LHR, PA diameter, thoracic liver

Timing of Surgery

• Why would repair worsen pulmonary compliance?– Increased intra abdominal pressure– Decreased chest wall compliance

Timing of Surgery

• Historical Management– Hyperventilate with 100% O2– Immediate surgical repair– Use of ECMO post operatively

Timing of Surgery

• Nakayama DK, et al. J Pediatr 1991– Medical stabilization (2-11 days) vs.

emergency surgery– Decreased mortality– Improvement noted in pulmonary mechanics

Protective Ventilation

• Wung JT, et al. J Pediatr Surg 1995– Spontaneous, pressure support ventilation– Permissive Hypercapnia– Preductal saturation >90%– Improved survival and decreased need for

ECMO with delayed surgery

HFOV

• Typically used as rescue therapy in CDH

• 3.5-15Hz

HFOV

• No prospective trials of HFOV vs. traditional ventilation in CDH

• Migliazza L, et al. J Pediatr Surg 2007– Retrospective review of 111 cases intended to

look at prognostic factors.– HFOV used as primary mode of ventilation

• Bhuta T, et al. Cochrane Database Syst Rev 2001– Infants with respiratory failure, all types– No survival improvement

Nitric Oxide

• Crosses cell membrane, increases cGMP concentration

• Selective pulmonary vasodilator• Free radical with short half life• Potential for methemoglobinemia and

accumulation of NO2 with prolonged use.

Nitric Oxide

• Controversial in CDH• Finer NN, Barrington KJ. Cochrane Database Syst

Rev 2006– Supports use of Nitric Oxide in PPH hypoxic

respiratory failure• NINOS. Pediatrics 1997

– No improvement in mortality– Increased risk of ECMO

ECMO

• Historically central in treatment of CDH

• Utilization has fallen in current practice

• Is ECMO a good idea for this patient?

ECMO• CDH Study Group. J Pediatr Surg 1999

– mortality data for ECMO vs. no ECMO based upon predicted mortality.

– Birth weight and Apgar score– ECMO improved survival in infants with >80%

predicted mortality only.– Worse mortality in the remainder of the patients.

ECMO

• Wilson JM, et al. J Pediatr Surg 1997– Retrospective review of cases at two tertiary

centers.– Toronto – HFOV as rescue– Boston – ECMO as rescue– Similar mortality data between the two centers.

ECMO

• A-a gradient 600mgHg• PIP > 25cmH2O• Oxygenation Index

– MAP×FiO2/PaO2– >40

Case Presentation

• Serial echocardiograms decreased PAP, reversal of PFO and PAD shunts L R

• ECMO weaned on DOL#12• Sepsis/DIC – delay• Closure on DOL#16

Preoperative Evaluation

• 4kg• NO(40ppm), Sildenafil, Bosentan• Dopamine 12 mcg/Kg/min• Fentanyl/versed• TPN• IJ central line, UAC• PRVC RR 40, PEEP 5, TV 17

Preoperative Preparation

• Warm room, warm fluids• CVP, Art line• Maintain ventilator settings/NO• Paralysis• Maintain ICU drips (including TPN)• Adequate volume access• Two pulse oximeters

Operative Details

• Subcostal incision• Hepatic Pulmonary fusion• Thoracotomy• Liver resection• Gore-Tex patch• 80% defect in R diaphragm

Anesthetic Management

• EBL 400 mL• PRBC 200 mL• FFP 160 mL• Plts 40 mL• UOP 20 mL

Postoperative Course

• Pneumothorax • BPD• Tracheostomy• Ventilator dependence• Nissen Fundoplication• G-Tube• Discharged at 3 months

Hepatic Pulmonary Fusion

• Schmuel K, et al. J Pediatr Surg 1998• Jeffrey W, et al. J Pediatr Surg 2010

– Case presentation plus literature review– Majority of cases identified intraoperatively– 50% success rate in separation– MRI identified as modality most likely to

Complications in CDH survivors

• Respiratory – obstructive disease, poor exercise tolerance

• GI – oral aversion, GERD, failure to thrive• Neuro – developmental delay, sensorineural

hearing loss• Musculoskeletal – pectus excavatum, scoliosis• Reherniation (10%)

Review

• US is only 50% sensitive for prenatal Diagnosis

• Early recognition and prompt treatment is essential

• The use of lung protective ventilation is the most effective therapy.

Appropriate management of a neonate born with congenital diaphragmatic hernia (CDH) should include 

 A.   Ventilation of the lungs with a bag and mask to keep saturation greater than 95%

  B.   Insertion of an orogastric tube  C.   Expansion of the hypoplastic lung with positive-

pressure ventilation  D.   Hyperventilation to keep the PaCO2 below 40 and pH

greater than 7.40  E.   Rapid transport to the operating room for surgical

correction

Appropriate management of a neonate born with congenital diaphragmatic hernia (CDH) should include 

 

  B.   Insertion of an orogastric tube 

Congenital Diaphragmatic Hernia may likely be associated with all of the following EXCEPT   A.   VSD  B.   Meningomyelocele  C.   Tetralogy of Fallot  D.   Gastroschisis  E.   Duodenal Atresia

Congenital Diaphragmatic Hernia may likely be associated with all of the following EXCEPT  

  D.   Gastroschisis  

References• Yao SF, Stein D, Savarese J: Congenital Diaphragmatic Hernia. In: Yao and Artusio's Problem

Oriented Anesthesia. Yao, F, editor. 6th ed.; 2008. p. 115-129• Motoyama & Davis: Smith's Anesthesia for Infants and Children, 8th ed.• Yamataka P, Puri P. Pulmonary Artery Structural Changes in Pulmonary Hypertension Complicating

Congenital Diaphragmatic Hernia. Journal of Pediatric Surgery, Vol32, No 3 (March), 1997: pp 387-390

• Migliazza L, et al. Retrospective study of 111 cases of congenital diaphragmatic hernia treated with early high-frequency oscillatory ventilation and presurgical stabilization, Journal of Pediatric Surgery, Volume 42, Issue 9, September 2007

• Nakayama D.K., Motoyama E.K., Tagge E.M.: Effect of preoperative stabilization on respiratory system compliance and outcome in newborn infants with congenital diaphragmatic hernia.  J Pediatr  1991; 118:793-799.

• Wung JT, Sahni R, Moffitt ST et al. Congenital diaphragmatic hernia: survival treated with very delayed surgery, spontaneous respiration and no chest tube. J Pediatr Surg 1995; 30: 406–409.

• Bhuta T, Clark RH, Henderson- Smart DJ. Rescue high frequency oscillatory ventilation vs conventional ventilation for infants with severe pulmonary dysfunction born at or near term. Cochrane Database Syst Rev 2001; 1: CD0002974

References• Finer NN, Barrington KJ. Nitric oxide for respiratory failure in infants born at or near term.

Cochrane Database Syst Rev 2006; 4: CD000399• de Buys Roessingh AS, Dinh-Xuan AT (2009) Congenital diaphragmatic hernia: current status and

review of the literature. Eur J Pediatr 168(4):393–406• Neonatal Inhaled Nitric Oxide Study Group, Inhaled nitric oxide and hypoxic respiratory failure in

infants with congenital diaphragmatic hernia, Pediatrics 99 (1997), pp. 838–845.• The Congenital Diaphragmatic Hernia Study Group. Does extracorporeal membrane oxygenation

improve survival in neonates with congenital diaphragmatic hernia? J Pediatr Surg 1999; 34: 720–725

• Azarow K., Messineo A., Pearl R., et al: Congenital diaphragmatic hernia—a tale of two cities: the Toronto experience.  J Pediatr Surg  1997; 32:395-400.

• Wilson J.M., Lund D.P., Lillehei C.W., et al: Congenital diaphragmatic hernia—a tale of two cities: the Boston experience.  J Pediatr Surg  1997; 32:401-405.

• Katz S, Kidron D, Litmanovitz I, et al. Fibrous fusion between the liver and the lung: an unusual complication of right congenital diaphragmatic hernia. J Pediatr Surg 1998;33:766-7

References• Keller RL, Aaroz PA, Hawgood S, et al. MR imaging of hepatic pulmonary fusion in neonates. AJR

Am J Roentgenol 2003;180: 438-40.• Gander JW, Kadenhe-Chiweshe A, Fisher C, et al. Hepatic pulmonary fusion in an infant with a

right-sided congenital diaphragmatic hernia and contralateral mediastinal shift . Journal of Pediatric Surgery (2010) 45, 265–268