Symposium on Common Pediatric Surgical Problems - II

Respiratory Distress in Neonates

Arun Kumar and V. Bhatnagar
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.

Abstract. Respiratory distress due to either medical or surgical causes occurs commonly in neonates. It is the most common cause of admission to a neonatal surgical intensive care facility in a tertiary care hospital. The distress can be caused by a variety of clinical conditions; common conditions treated in medical intensive care units are transient tachypnea of the new born, respiratory distress syndrome, pulmonary air leak and pneumothorax. In surgical causes of respiratory distress in neonates the underlying mechanisms include airway obstruction, pulmonary collapse or displacement and parenchymal disease or insufficiency; the common causes are congenital diaphragmatic hernia, congenital cystic adenomatoid malformation, congenital lobar emphysema and esophageal atresia with or without tracheo-esophageal fistula. Obstructive lesions of the new born airway include choanal atresia, macroglossis, Pierre-Robin syndrome, lymphangioma, teratoma or other mediastinal masses, cysts, subglottic stenosis and laryngo tracheomalacia. Imaging plays a very major role in the pre-operative diagnosis of these conditions and proper pre-operative resuscitation helps in improving the results of surgery dramatically. [Indian J Pediatr 2005; 72 (5) : 425-428] E-mail : veereshwarb@hotmail.com.
Key words : Respiratory distress; Tachypnea; Airway obstruction; Congenital malformations

Respiratory distress in a neonate is defined as a respiratory rate of more than 60 per minute, dyspnea, with intercostal or subcostal indrawing, sternal retraction and a predominantly diaphragmatic breathing pattern. A characteristic expiratory or inspiratory grunt may or may not be present. The respiratory distress in neonates may be because of a predominantly medical or surgical pathology or may be a medical condition superimposed on a surgical pathology. This article reviews, in brief, the medical and surgical causes of respiratory distress in the light of cases from a single tertiary care institute in India. Records of all cases admitted to the surgical neonatal intensive care unit in the last five year (2000-2004) were reviewed and the patients categorized according to the pathology and final diagnosis (Table 1). A little less than half of all neonatal surgical ICU admissions are because of conditions leading to respiratory distress and of these the
TABLE 1. Yearly Average (2000-2004) of Cases Admitted to the Neonatal Surgical ICU of a Tertiary Care Hospital (AIIMS) (Unpublished Data). Diagnosis Number of patients 155 63 48 2 9 2 2

majority are because of esophageal atresia with or without tracheo-esophageal fistula. The other conditions causing neonatal respiratory distress are far fewer in number. Medical Causes of Respiratory Distress in Neonates The medical conditions which commonly lead to respiratory distress are transient tachypnea of the new born, pulmonary air leaks leading to accumulation of air in the thorax, mediastinum, pericardium or peritoneum and the respiratory distress syndrome. Transient Tachypnea of the New Born The word transient tachypnea of the new born was coined by Avery.1 Also known as wet lung2, benign unexplained respiratory distress in the newborn 3, neonatal tachypnea4 and type 2 respiratory distress syndrome.5 Classically, it presents with isolated tachypnea and respiratory rate of 100 120 per minute. Grunting and retractions are rare. This form of tachypnea usually settles within 24 hours but may persist for several days. X-ray of the chest shows hyperinflation, sometimes with intercostal bulging of the pleura. There are prominent perihilar vascular markings, edema of the interlobar septae and fluid in the fissures. Most infants do not require any form of respiratory support other than added oxygen and rarely require FiO 2 > 40% or support for more than three days. The proposed mechanism for transient tachypnea of the new born is a delay in the fetal lung fluid reabsorption after birth. Although diuretics have been tried in randomized prospective studies no significant difference was found in the duration of tachypnea or hospitalization. 6 Respiratory Distress Syndrome Respiratory distress syndrome (RDS) is an acute illness,
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Total number of admissions Cases with respiratory distress Esophageal atresia with or without tracheoesophageal fistula Tracheoesophageal fistula without esophageal atresia Congenital diaphragmatic hernia Eventration of diaphragm Congenital lobar emphysema, lung cyst, CCAM

Correspondence and Reprint requests : Prof. V.Bhatnagar, Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi-110029. India. Fax : +91-11-26588663.

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Anil Kumar and V. Bhatnagar usually of preterm infants,clinically characterized by respiratory distress presenting within 4-6 hours of birth. There is a reticulogranular chest X-ray appearance as a result of widespread atelectasis. Pathophysiologically, the condition is characterized by a non-compliant lung (stiff lung), which contains less surfactant than normal and becomes atelectatic at the end of expiration. The signs of respiratory distress must develop before the neonate is 4 hrs old and persist beyond 24 hrs of age.7 Pulmonary Air Leaks Pneumothorax and pulmonary interstitial emphysema (PIE) are the most common forms of air leak in the newborn, although pneumomediastinum, pneumopericardium and pneumoperitoneum may also occur. Rarely, multiple air leaks may be complicated by subcutaneous emphysema and systemic air embolism. Pulmonary air leaks occur when there is uneven alveolar ventilation, air trapping and high transpulmonary pressure swings the final common pathway being alveolar overdistention and rupture. The gas tracks along the roots of the lungs but not in the sheaths of the bronchi. Air may then rupture into the pleura, mediastinum, pericardium or extrathoracic areas. Pneumothorax Pneumothorax may occur immediately after birth owing to the high transpulmonary pressure swings generated by the newborn during his first breaths or during active resuscitation. Thereafter, pneumothorax is usually a complication of respiratory disease, for example RDS or meconeum aspiration syndrome, congenital malformations in which there may be alveolar overdistension and air trapping, made worsen by intermittent positive pressure ventilation (IPPV). Pneumothorax may occasionally result from direct injury to the lungs by causes other than mechanical ventilation e.g. perforation by suction catheters passed through the endotracheal tube.8 Surgical Causes of Neonatal Respiratory Distress Surgical causes of neonatal respiratory distress are due to airway obstruction, pulmonary collapse or displacement and parenchymal disease or insufficiency. Any two or all three mechanisms may occur simultaneously in the same baby. If a cyanosed baby makes strenuous and active respiratory effort, with labored breathing and retraction, agitation, wheezing, stridor and tachypnea, the cause is most probably airway obstruction. Examination of the neck, nose, mouth and throat alongwith bronchoscopy and laryngoscopy and X-rays of the head, neck and chest both PA and lateral, will usually establish the diagnosis and indicate treatment. Common Obstructive Lesions of the Newborn Airway Nose, oral cavity and pharynx Bilateral choanal atresia, macroglossia, Pierre-Robin Syndrome or neoplasms like
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lymphangioma, teratoma or cyst. Larynx Laryngomalacia, bilateral vocal cord palsy, subglottic stenosis, cysts and laryngoceles, webs, lymphangioma, teratoma, cystic hygroma. Tracheobronchial tree Tracheomalacia, bronchomalacia, vascular anomalies, tracheo-esophageal fistula, webs, mediastinal masses like bronchogenic cysts or teratomas. Bilateral choanal atresia causes acute symptoms and can be readily diagnosed by failure of passing a nasogastric tube through the nostrils into the pharynx and may require an oropharyngeal airway, orogastric tube feeds or tracheostomy as emergency measures if specific treatment of choanal atresia is not forthcoming. Laryngomalacia is the most common obstructive cause of the neonatal airway; it produces inspiratory stridor and there is relief of symptoms in the prone position with the neck extended. In most cases, spontaneous improvement by 2-3 years of age is usual. Lymphangiomas may be localized or extensive while hemangiomas are usually subglottic. Surgical excision, CO2 laser (if available) or emergency tracheostomy are the commonly used methods of treatments. Oral steroids (prednisolone 5 mg/ kg on alternate days with gradual tapering over 100 days) and intralesional injection sclerotherapy are alternative methods of treatment in hemangiomas. Subglottic stenosis may be congenital or acquired (post endotracheal intubation) and may require dilatation with intralesional steroids, intraluminal stents, laryngotracheoplasty or tracheostomy. Similarly, tracheomalacia can be primary or secondary to esophageal atresia with or without tracheoesophageal fistula and vascular rings. Spontaneous resolution with time is known; otherwise it may require aortopexy or repair of the vascular anomaly. Common Conditions Causing Pulmonary Compression in the New Born Congenital diaphragmatic hernia (CDH), pneumothorax, congenital lobar emphysema (CLE), congenital cystic adenomatoid malformation (CCAM), eventration of diaphragm, pleural effusions (especially chylothorax) and congenital lung cysts are the common lesions which produce respiratory distress in a neonate due to pulmonary compression. Congenital Diaphragmatic Hernia (CDH) It is one of the most difficult challenges in the realm of pediatric surgery. Herniation of abdominal visceral contents through the posterolateral foramen of Bochdalek (more common) or the anterior foramen of Morgagni (rare) leads to CDH. Maldevelopment of the post hepatic mesenchymal plate and closure of pleuroperitoneal canal, which are crucial for normal diaphragmatic development, leads to CDH. Presence of abdominal viscera in the chest during fetal life prevents normal development of the lungs. The onset of symptoms depends on the volume of
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Respiratory Distress in Neonates viscera in the chest and the degree of pulmonary hypoplasia. A history of polyhydramnios is noted in approximately 80 % and prematurity is frequent. There is a high incidence of still birth. Postnatal immediate presentation is seen in the most severely affected. Cyanosis, tachypnea, grunting and retraction are commonly seen. On physical examination, the abdomen is scaphoid, the antero-posterior diameter of the chest is increased, mediastinal shift may be noted and breath sounds are absent on the affected side and bowel sounds may be heard in chest. Less severely affected babies may present beyond the first few hours of life in 1020% cases. The symptoms in this group of patients are non-specific and may include recurrent chest infections, failure to thrive, vomiting and abdominal pain. The diagnosis is made on the chest radiograph which shows airfilled bowel loops in the chest cavity with non-vizualization of diaphragmatic margin, mediastinal shift and a relative paucity of abdominal gas. A small portion of the ipsilateral lung may be visible superiorly. An echocardiogram should be performed to exclude associated congenital heart defects. Routine neonatal management measures include nasogastric tube insertion and drainage. Bag and mask ventilation is contraindicated as it causes bowel distension and increases the mediastinal shift further which affects the contralateral lung and also compromises cardiac function. Ionotropic support may be needed to maintain blood pressure and peripheral circulation. Neuromuscular blockade and sedation with morphine or fentanyl is usually required for proper ventilatory support. Arterial blood gases are the primary determinants in management protocol for prognosis in neonates with CDH. A right radial artery cannula is inserted after checking adequacy of ulnar circulation to monitor preductal blood gases. The hemodynamic status is also monitored. CDH is a surgical emergency but adequate preoperative stabilization is a prerequisite for good results and has helped in selecting survivors from those considered to be nonsurvivors. For the surgical repair, a transabdominal route is used because it is easier to reduce viscera and accurately visualize the defect and repair is possible without risk of injury to the intestines and other abdominal organs; other associated intestinal anomalies can be corrected and the abdominal cavity can be enlarged by manual stretching if required or a silo. Presently only in a few selected centers extra corporeal membrane oxygenation (ECMO) support is provided either before or after repair of the defect. With the advent of delayed repair more infants are being placed on ECMO preoperatively in the hope of achieving stabilization. But the incidence of hemorrhagic complications is high on ECMO. Antenatal diagnosis has added a new dimension in the management of CDH, with particular reference to prevention of lung hypoplasia. Eventration of the diaphragm results from maldevelopment of the diaphragm muscle which is replaced by a thin membranous structure. It usually
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presents in older children but in new borns it may be difficult to differentiate from CDH. The treatment consists of diaphragmatic plication which can be carried out either by thoracotomy or laparotomy. Congenital Cystic Adenomatoid Malformation (CCAM) CCAM is characterized by a mass of cysts that may or may not intercommunicate. The primary defect is a segmental bronchial atresia. 9 Stocker described 3 histological types. Type 1 macrocystic : few large cysts with walls containing thick smooth muscle and elastic tissue with normal alveoli between the cysts (55%); Type 2 - small evenly placed cysts less than 1 cm in diameter with thin muscular coat, large alveolar like structures between cysts (40%); and type 3 - microcystic : bulky firm masses of folded epithelium and thick muscular layer. Symptoms of respiratory distress may develop soon after birth; 60% of patients become symptomatic within one month of life, another 10% between one and six months and 15 % by adoloscence. Recurrent respiratory tract infection is the other common mode of presentation. Fetal diagnosis on ultrasonography is possible by the 20 th week of gestation. Plain chest. X-ray may show multiple air filled spaces (macrocystic) or a solid area within the lung field (microcystic). There will also be compression of surrounding lung and shift of mediastinum to the opposite side. CT scan of the chest is diagnostic. Prompt thoracotomy and resection of the CCAM is the treatment of choice. In most instances, a limited resection of involved segment while preserving the normal lung tissue is possible. The prognosis depends upon the severity of the associated lung hypoplasia. Congenital lung cysts may present in the neonatal age group and are an important differential diagnosis for CCAM. The presentation and management is similar. Congenital Lobar Emphysema (CLE) CLE has been defined as the post natal overdistention of the air spaces of one or more segments or lobes of the lung. This overdistended portion of the lung compresses the adjacent lung and thus compromises ventilation. Pathologically the alveoli distend during inspiration but are unable to deflate during expiration. Involvement of the left upper lobe (42%) occurs most frequently followed by right middle (35%) the right upper lobe(21%). Males are affected three times as often as females. 10 One half of the patients become symptomatic in the neonatal period while others may present months later.11 The presentation is with respiratory distress, which can be of varying severity. The baby may become dyspneic immediately after birth. Physical examination reveals a hyper resonance in the affected area with diminished air entry on auscultation. Chest radiography is best diagnostic tool; a hyperlucent overexpanded area of the lung with compression or atelectasis of the adjacent lobes of the lung, depression of the diaphragm and mediastinal shift to the opposite side are the usual features noticed. The
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Anil Kumar and V. Bhatnagar emphysematous segment may also herniate anterior to the middle mediastinum. CT scan of the chest is a useful adjunct and can help in the differential diagnosis. 11 Echocardiography is done to rule out an associated cardiac anomaly while a ventilation-perfusion scan, if available, may show unventilated and hypoperfused lung segments. Prompt resection of the involved lobe is the ideal treatment in symptomatic children. A lobectomy or segmental resection is needed. A diagnostic bronchoscopy in suspected patients may be done only if facilities for open surgery are ready because it can lead to sudden deterioration due to air trapping. Esophageal Atresia with or without Tracheoesophageal Fistula Esophageal atresia (EA) with or without tracheoesophageal fistula (TEF) is the commonest pediatric surgical condition leading to neonatal respiratory distress. The cause of respiratory distress is flooding of the tracheobronchial tree with saliva or gastric juices either through the TEF, or due to aspiration from the saliva pooled in the upper pouch or through an upper pouch fistula in case of EA. These neonates present soon after birth with frothing at the mouth or choking with attempted feeds or with manifestations of aspiration pneumonitis. An obstruction to the passage of an orogastric tube at about 10 cm from the mouth is usually diagnostic and dye studies are not required for confirmation (in fact, spillage of dye into the lungs makes matters worse!). A lateral chest x- ray with a red rubber catheter in the upper pouch marks the level of the upper pouch and an AP film gives valuable information on the status of the lungs. A preoperative ECHO helps in the evaluation of associated cardiac defects or in detection of a right sided aortic arch. Other imaging studies to assess the associated malformations (VATER/VACTERL) are best carried out after the primary surgical treatment. The surgical treatment aims at disconnection of the TEF and restoration of esophageal continuity either as a primary or a staged procedure or the esophagus can be initially diverted or then replaced with stomach or colon at a later date. The results of surgery are largely dependent on the birth weight, associated congenital malformations and condition of the chest. There is now a wealth of information from India on local factors which influence the results of surgery.13, 14 REFERENCES
1. Avery ME, Gatewood OB, Brumley G. Transient tachypnea of the newborn. Am J Dis Child 1966; 111 : 380-385. 2. Wesenberg RL, Graven SN, McCabe EB. Radiological findings in wet lung disease in the newborn. Radiology 1971; 98 : 69-74. 3. Taylor PM, Allen AC, Stinson DA. Benign unexplained respiratory distress of the newborn. Pediatr Clin North Am 1971; 18 : 975-1004. 4. Malan AF. Neonatal tachypnea. Aus Pediatr J 1966; 3 : 159-163. 5. Prodhom LS, Levison H, Cherry RB, Smith EA. Adjustment of ventilation, intrapulmonary gas exchange and acid balance in the first day of life. Pediatrics 1965; 35 : 662-676. 6. Wiswell TE, Rawling JS, Smith FR, Goo ED. Effect of furosemide on clinical course of transient tachypnea of the newborn. Pediatrics 1985; 75 : 908-910. 7. Rudolph AJ, Smith CA. Idiopathic respiratory distress syndrome of the newborn. J Pediatr 1960; 57 : 905-921. 8. Anderson KD, Chandra R. Pneumothorax secondary to perforation of segmental bronchi by suction catheters. J Pediatr Surg 1976; 11 : 687-693. 9. Moerman P, Gryns, Vanderberyhe K et al. Pathogenesis of CCAM of the lung. Histopathology 1992; 21 : 135-139. 10. Man DW, Handy MH, Hendry GM et al. Congenital lobar emphysema: problems in diagnosis and management. Arch Dis Child 1983; 58 : 709-714. 11. Coran AG, Drongowski R. Congenital cystic diseases of tracheobronchial tree in infants and children: experience with 44 consecutive cases. Radiographics 1994; 129 : 521-527. 12. Markowitz RI, Merurio MR, Vahjen GA et al. Lobar emphysema: the role of CT and V/Q scan. Clin Pediatr 1989; 28 : 19-22. 13. Agarwala S, Bhatnagar V, Bajpai M et al. Factors for poor results of treatment of esophageal atresia in developing countries. Pediatr Surg Int 1996; 11 : 312-315. 14. Bhatnagar V, Agarwala S, Bajpai M et al. Results of treatment of esophageal atresia. J Indian Assoc Pediatr Surg 1997; 2 : 55-59.

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