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Congenital Diaphragmatic Hernia
Jeff WuPediatric Surgery Clerkship, David Geffen SOM at UCLAMarch 8, 2006
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ID/CC: 38 4/7 week gestation newborn male NSVD to 31 y/o mother.
PMHx: patient antenatally diagnosed with CDH at 28 weeks.
Infant intubated in delivery room
Placed on conventional mechanical ventilation
PE: Right-deviated trachea; diminished breath sounds on L; abdomen scaphoid
pH of 6.74, pCO2 of 111, pO2 of 98, bicarbonate 14.8, oxygenSaturation 82%, and base deficit of -22.
High-frequency oscillator: Mean airway pressure of 18, FiO2 100%, Nitric oxide 20 ppm. Amplitude was 38 to 42 and freq 10 Hz.
O2 Sat 48%.
Head ultrasound was obtained no evidence of intraventricular hemorrhage
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What is it?1) Failure of diaphragmatic fusion
Foramen of Bochdalek (85-90%)Foramen of Morgagni
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What is it?2) Herniation of abdominal contents
into thoracic cavity3) Pulmonary hypoplasia
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Incidence~1:2500 live births~1100 cases in the U.S. annually$230M spent on hospitalization
Despite advances in care, survival remains around ~65%
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DiagnosisAntenatal:
U/S at ~20 weeks gestation~60% of CDH patients are diagnosed antenatallyProposed prenatal determinants of outcome: polyhydramnios; intrathoracic stomach or liver; abdominal circumference; lung-to-head ratioAlso search for associated malformations
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Postnatal:clinical signs of respiratory distressXR: absent diaphragmatic outline, loops of bowel in chest, tip of NG tube in thorax
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Initial ManagementGoal: oxygenate, avoid
barotraumaIntubate: conventional mechanical
ventilation+/- SedateNGT for decompression
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Pathophysiology1) Pulmonary hypoplasia
“compression theory”: - modeled in fetal lambs- rationale for early surgery to remove
“compressive” bowels from thorax
“global embryopathy”: - modeled in newborn rats- rationale for new therapeutic ideas
2) Pulmonary hypertensioncauses persistent fetal circulation
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Medical ManagementGoal: stabilize patient until
definitive surgical repairPulmonary vasodilators: inhaled nitric oxideInotropes, systemic vasoconstrictors: dobutamine, dopamine, epinephrinehigh frequency oscillatory ventilationECMOSurfactantAntenatal steroids?Liquid ventilation?
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ECMO
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Surgical Management
typically a subcostal incision (thoracotomy rarely considered)
gentle reduction of abdominal visceraidentification and excision of hernia
sac (found in 10%)approximate diaphragmatic tissue with
sutures, Goretex, or muscle flaps
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Developing Therapy- Fetal surgery idea to remove herniated bowels early
in development stemmed from “Compression hypothesis”; initial findings demonstrated no survival benefit.
- PLUG fetal surgery is an idea which makes use of the observation that laryngeal atresia is associated with enlarged hyperplastic lungs; Plug the Lung Until it Grows
- Growth factors injected during embryologic development
- Vitamin A important in lung development, maybe can prevent CDH; prenatal treatment of Nitrofen rats with Vit A showed decreased incidence of CDH at term.
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“An ounce of prevention is worth more than a pound of cure.” - Benjamin Franklin
Take home pointsDefect, herniated bowel, and pulmonary hypoplasiaTreat pulmonary hypoplasia/hypertension medically until
stabilizedTiming of surgical therapy based on optimization of
patientPossible new therapies including prevention?
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References[1] Downard CD, Wilson JM. Current therapy of infants with
congenital diaphragmatic hernia. Semin Neonatol. 2003 Jun;8(3):215-21.
[2] Smith NP, Jesudason EC, Losty PD. Congenital diaphragmatic hernia. Paediatr Respir Rev. 2002 Dec;3(4):339-48.
[3] Jesudason EC. Challenging embryological theories on congenital diaphragmatic hernia: future therapeutic implications for paediatric surgery. Ann R Coll Surg Engl. 2002 Jul;84(4):252-9.
[4] O’Neill J, Grosfeld J, Fonkalsrud E. Chap 44. Congenital Diaphragmatic Hernia. Principles of pediatric surgery, 2nd Ed. Mosby 2003.