Congenital_Diaphragmatic_Hernia

Congenital_Diaphragmatic_Hernia - Congenital Congenital...

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Unformatted text preview: Congenital Congenital Diaphragmatic Hernia Manuel A. Molina, M.D. University Hospital at Brooklyn SUNY Downstate Congenital Congenital Diaphragmatic Hernias Congenital Diaphragmatic Hernia Congenital Incidence 1 in 2000 to 5000 live births. Incidence 80% in the left side, 20% in the right. Rarely 80% bilateral. Survival 60-70% Survival Cause is unknown. Failure of normal closure of Cause the pleuroperitoneal canal in the developing embryo. Abdominal contents herniate and compress the ipsilateral developing lung, causing pulmonary hypoplasia and hypertension. Congenital Diaphragmatic Hernia Congenital Associated with genetic anomalies: Associated Smith-Lemli-Opitz syndrome Smith DiGeorge syndrome DiGeorge Chromosome 15,18,13 and 21 anomalies Chromosome Fryns syndrome Fryns Pallister-Killian syndrome Pallister Congenital Diaphragmatic Hernia Congenital Anatomy: Anatomy: Posterolateral: Bochdalek’s henia, most common type. Posterolateral Anteromedial: Morgagni’s hernia. Anteromedial Congenital Diaphragmatic Hernia Congenital Clinical presentation: Clinical Dyspnea Dyspnea Tachypnea Tachypnea Cyanosis Cyanosis Severe retractions of respiratory muscles Severe Scaphoid abdomen Scaphoid Large antero-posterior diameter of the chest. Large Three general presentations: Three Severe respiratory distress at the time of birth.( Severe Severe hypoplasia) Respiratory deterioration hours after delivery (honeymoon Respiratory period). Benefit from correction of hypoxemia and pulmonary hypertension. Feeding difficulties, chronic respiratory disease, pneumonia, Feeding intestinal obstruction 24h after birth. (10-20% of patients). Best Prognosis. Congenital Diaphragmatic Hernia Congenital Diagnosis: Diagnosis: 59% antenatal detection with average age 24.2 weeks.(Garne et al, Ult 59% Obst Gyn, 2002) Gyn, Polyhydramnios Polyhydramnios Intrathoracic stomach or liver Intrathoracic Lung-to-head ratio and lung/transverse thorax ratio. Lung Usually at prenatal ultrasound (15 weeks). Usually Recently fetal MRI and fetal echocardiography, helpful to Recently determine degree of pulmonary hypoplasia. (MRI lung volumetry, left ventricular mass and pulmonary artery diameter) Postnatal diagnosis, on CXR with gastric air bubble or Postnatal intestine in the chest. Amniocentesis is recommended to provide information Amniocentesis regarding possible chromosomal abnormalities. Congenital Diaphragmatic Hernia Congenital Congenital Diaphragmatic Hernia Congenital Prenatal management: Prenatal Open fetal surgery: remove the compression of the Open abdominal viscera. High risk for fetus and the mother. No survival advantage. (Harrison et al, J Ped Surg, 1997) (Harrison Fetal tracheal occlusion: stimulation of lung growth Fetal with accumulation of fluid. Result in larger but persistent abnormal lung. (Flake et al, Am J Obst Gyn, 183, 2000). (Flake Steroids therapy weekly to improve lung function is Steroids controversial (risk of brain and body development problems). (Ford et al., Ped Surg Int, 2002) (Ford Congenital Diaphragmatic Hernia Congenital Congenital Diaphragmatic Hernia Congenital Postnatal management: Postnatal Stabilization of cardiorespiratory system. Stabilization Endotracheal intubation is critical Nasogastric or Orogastric tube placement. Nasogastric Permissive hypercapnia and stable hypoxemia Permissive (>80%), associated with survival of 76%. (Boloker et al, J Ped Surg, 2002). Delayed repair (24 to 72 hours) improves survival Delayed when compared with early emergent repair. Allows stabilization of the infant before surgical repair. (Andrew (Andrew et al., J Ped Surg, june 2004). 1/3 of patients will require ECMO. Head US to r/o 1/3 hemorrhage prior to ECMO. Congenital Diaphragmatic Hernia Congenital Reports of 90%survival by Bohn with early use of Reports HFOV and virtually no ECMO. (Bohn. Am J Resp Crit Care Med, (Bohn. 2002) Nitric oxide or ECMO for patients unable to be Nitric stabilize on reasonable ventilatory setting (pH.7.25, Peak Pressures <30 cm H2O, pre-ductal SO2 >90% with FiO2<60%). iNO beneficial in isolated PPHN. Careful attention to right sided heart failure increases survival by 10%. Preliminary results from CDH Study Group: the use Preliminary of surfactant may worsen outcome. Congenital Diaphragmatic Hernia Congenital Factors with influence in the survival rate: Factors Each increase 1mmHg of pCO2 above 50 mmHg Each increases mortality by 1% Each decrease of 1mmHg in the pO2 below 40% Each the mortality rate increases by 1% Cardiac disease 3 times most likely to die. Cardiac Renal disease 6 times more likely to die. Renal Congenital Diaphragmatic Hernia Congenital Approaches for surgical repair: Approaches Abdominal subcostal Abdominal Thoracotomy Thoracotomy Laparoscopic vs Thoracoscopic Laparoscopic MIS ideal for Morgagni hernias but can be challenging because the MIS peumoperitoneum widens the defect. Laparoscopy for Bochdalek’s has a high failure rate and is associated with ↑pCO2 and acidemia Contraindicated if very high pCO2. Contraindicated Thoracoscopy is better approach for Bochdalek hernias with Thoracoscopy recurrence of 14%. Open approach 3-22%.(Marjorie et al, J Ped Surg, Nov 2003.) Small defect can be repaired primarily. Large defect Small will require abdominal or thoracic muscle flaps, or prosthetic patch (tension free). Congenital Diaphragmatic Hernia Congenital Congenital Diaphragmatic Hernia Congenital Long term follow up: Long GERD GERD Foregut dysmotility Foregut Chronic lung disease Chronic Scoliosis Scoliosis Pectum excavatum Pectum Cognitive skills, developmental delay, seizures, Cognitive hearing loss if severe respiratory distress. Conclusions Conclusions CDH is a congenital anomaly with a high mortality. Usually CDH associated with pulmonary hypoplasia and hypertension. Surgical repair is only treatment. Delayed surgery until the Surgical patient is stable is associated with better outcomes. Congenital cardiac and renal diseases, hypoxemia and Congenital hypercapnia increases mortality. HFOV, ECMO, iNO has improved the survival of CDH. HFOV, Permissive hypercapnia with acceptable pO2 has shown to Permissive improve survival. Long term follow up is necessary to detect complications. Long Tracheal occlusion in utero, keeps lung expanded but is a Tracheal abnormal lung. Primary repair is small defect, patch if large defect, to prevent Primary tension. ...
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