Pediatric Surgery Lim,Kirstyleigh Limbo, Michael Lindo, John Liwag, Conalou Llamas, Jacqueline Locaylocay, Charlene
A 6–hour old newborn was noticed to be in respiratory distress Physical Exam: - RR = 65 breaths/min - (+) intercostal retractions - (+) alar flaring
Disease of the respiratory system which invariably alter the exchange of gases between the air and the blood Causes: (1) Abnormal mechanical function. (2) Neuromuscular abnormalities of the respiratory nerves and muscle.
- alterations in breathing pattern diaphragmatic breathing pattern - irregular rate of breathing ( >65/bpm) - chest wall retractions - (+) alar flaring - (+/-) expiratory or inspiratory grunt - cyanosis - most common cause of admission to a neonatal surgical intensive care
- Check for the patients ABC’s A irway B reathing C irculation - Assess the patients over-all condition PE: neck masses, chest and abdomen ABG: Respiratory alkalosis Imaging: CXR
Respiratory distress, (-) breath sounds
Atelectasis
Pneumothorax
Congenital Diaphragmatic hernia
Congenital Lobar Emphysema
Congenital Cystic Adenomatoid Malformation
Respiratory distress, (-) breath sounds
Atelectasis
Pneumothorax
Congenital Diaphragmatic hernia
Congenital Lobar Emphysema
Congenital Cystic Adenomatoid Malformation
incomplete expansion or complete collapse of lung tissue Causes: compression, obstruction, paralysis
Clinical manifestations: dyspnea rapid shallow respirations tachycardia often, cyanosis
Physical findings: Flat chest on affected side Limited or reduced chest excursion Dull on percussion Diminished or absent breath sounds
Respiratory distress, (-) breath sounds
Atelectasis
Pneumothorax
Congenital Diaphragmatic hernia
Congenital Lobar Emphysema
Congenital Cystic Adenomatoid Malformation
1-2% of neonates Accumulation of extrapulmonary air within the chest At birth, due to the high transpulmonary pressure swings generated by the newborn during first breaths or during active resuscitation
Clinical manifestations: Dyspnea, cyanosis
Physical examination: Retraction Tympanitic on percussion Decreased or absent breath sounds on affected lung
Respiratory distress, (-) breath sounds
Atelectasis
Pneumothorax
Congenital Diaphragmatic hernia
Congenital Lobar Emphysema
Congenital Cystic Adenomatoid Malformation
a.k.a. infantile lobar emphysema Over inflation of air spaces with or without alveolar rupture causing respiratory function to decrease and air to leak out into the space around the lungs Often reversible but life threatening cause of RDS in the neonate Air can enter but cannot escape Assoc w/ CHD in 15% (PDA,VSD) Autosomal dominant For some it may occur for no apparent reason
Epidemiology 50% - First four weeks of life 75% - in infants <6months old Male predominance, 3:1 Mortality rate: 10% almost always one lobe involved Left upper lobe – 41% Right middle lobe – 34% Right upper lobe – 21%
Clinical manifestations Shortness of breath Wheezing
Physical Examination Cyanotic lips and fingernail beds
result of bronchial structures failing to mature properly at approximately the fifth or sixth week of gestation, about the time the lungs begin to form. benign mass of abnormal lung tissue usually located on one lobe of the lung caused by overgrowth of abnormal lung tissue that may form fluid filled cysts abnormal tissue will never function as normal lung tissue.
Clinical Manifestations most CCAMs either shrink or are small enough not to cause a problem “hydrops" or heart failure (large CCAMs) "pulmonary hypoplasia" or small lungs ( mother suffering from "polyhydramnios") babies may have difficulty breathing after birth and will require oxygen and assistance with breathing
Physical Examination decreased breath sounds on the affected area with mediastinal shift away from the lesion.
Respiratory distress, (-) breath sounds
Atelectasis
Pneumothorax
Congenital Diaphragmatic hernia
Congenital Lobar Emphysema
Congenital Cystic Adenomatoid Malformation
Herniation of abdominal contents into the thoracic cavity, commonly through the posterolateral foramen of Bochdalek 1 in 5,000 live births Predominantly on the left (70-85%) Trisomy 21, 13 & 18 Associated anomalies (20-30%) CNS lesions Esophageal atresia Omphalocoele CV lesions
Etiology 8th wk of gestation: separation of the developing thoracic & abdominal cavity is accomplished by closure of the posterolateral foramen (Bochdalek) Failure of pleuroperitoneal canal to close results to CDH Primary problem is pulmonary hypoplasia with 2° diaphragm maldevelopment surfactants is absent Alveolar growth and pulmonary vasculature are linked, so there is an abnormal pulmonary vasculature
Clinical manifestations: Signs of RDS either at birth or first few hours of life Retractions, cyanosis, grunting respirations
Scaphoid abdomen, barrel shaped chest 20-25% has congenital heart disease
Physical Examination Absent or decreased breath sounds shift of cardiac sounds over the contralateral CDH Bowel sounds heard in the thorax
Congenital Diaphragmatic Hernia
Respiratory distress, (-) breath sounds CHEST X-RAY
Atelectasis
Pneumothorax
Congenital Diaphragmatic hernia
Congenital Lobar Emphysema
Congenital Cystic Adenomatoid Malformation
Radiographic findings: Opacity of the atelectatic lung Deviation of mediastinal structures and heart towards the involved lung
Radiologic findings: Hyperlucency of involved lung Shift of mediastinal structures away from the affected side
A frontal radiograph of the chest in a neonate shows marked overdistention of the left upper lobe with mediastinal shift to the right.
Lateral chest radiograph shows anterior herniation of the right middle lobe.
On the Left side opaque mass is seen
Radiographic findings: “bubble bowel” in the chest
Prenatal Mgt Medical Therapy Fetal Surgical Therapy
Postnatal Mgt Surgical Mgt
Ventilatory Strategy initial mgt; GOAL: adequate O2 delivery and to mainatain acceptable arterial pH Minimize barotrauma by use of pressure limited vent, tolerance of high PaCO2, min sedation and avoidance of paralysis
High Frequency Oscillation Avoids high inspiratory pressures while providing effective ventilation; GOAL: adequate gas exchange Recently used as a second line therapy
Extacorporeal Membrane Oxygenation (ECMO) Most patients who are treated had ventilator-induced lung injury >90% of CDH px who are treated w/ ECMO are now placed on ECMO prior to repair CRITERIA for use: Clinically detoriorating Req level of ventilation that has a high risk of lung injury (peak inspiratory pressure >25mmHg)
Timing of Operation Ideal time to repair CDH is unknown Some repair 24 hrs after stabilization, but 7-10 days delay is often tolerable Some if normal pulmonary artery pressure maintained for at least 24-48 hrs based on ECG
Operative Approach Long Term Follow-Up
Subcostal incision – most common Transthoracic approach chest incision; very useful in a large rightsided defects e.g. reduction of the liver is easier
Transabdominal approach mostly used on the left side
Patch repair Polytetrafluoroethylene is the most widely used
Recurrence rate is high in large hernias Repair of diaphragmatic defect often worsen pulmonary compliance by reducing elasticity of the chest wall and increasing abdominal pressure
COMPLICATIONS: Chylothorax known complication after repair Due to injury o the thoracic duct during repair but possible there is an abnormality in the chyle drainage
Late bowel obstruction Wound hernias