1 Diaphragmatic Function, Diaphragmatic paralysis, and Eventration of the Diaphragm.
Congenital Diaphragmatic Hernia Jeff Wu Pediatric Surgery Clerkship, David Geffen SOM at UCLA March...
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Transcript of Congenital Diaphragmatic Hernia Jeff Wu Pediatric Surgery Clerkship, David Geffen SOM at UCLA March...
Congenital Diaphragmatic Hernia
Jeff WuPediatric Surgery Clerkship, David Geffen SOM at UCLAMarch 8, 2006
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
What is it?1) Failure of diaphragmatic fusion
Foramen of Bochdalek (85-90%)Foramen of Morgagni
What is it?2) Herniation of abdominal contents
into thoracic cavity3) Pulmonary hypoplasia
Incidence~1:2500 live births~1100 cases in the U.S. annually$230M spent on hospitalization
Despite advances in care, survival remains around ~65%
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
Postnatal:clinical signs of respiratory distressXR: absent diaphragmatic outline, loops of bowel in chest, tip of NG tube in thorax
Initial ManagementGoal: oxygenate, avoid
barotraumaIntubate: conventional mechanical
ventilation+/- SedateNGT for decompression
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
Medical ManagementGoal: stabilize patient until
definitive surgical repairPulmonary vasodilators: inhaled nitric oxideInotropes, systemic vasoconstrictors: dobutamine, dopamine, epinephrinehigh frequency oscillatory ventilationECMOSurfactantAntenatal steroids?Liquid ventilation?
ECMO
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
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.
“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?
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.