Ghai Essential Pediatrics8th

Treatment. Although a significant number of acute sinusitis episodes will resolve spontaneously, treatment with antibiotics is preferred. Therapy with amoxicillin is recommendedfor 10-14 days. Longer courses and second­ line antibiotic agents are indicated for refractory infections. Parenteral antibiotics are necessary for sinusitis with orbital or intracranial complications. Other adjuvant mea­ sures include oral decongestants, mucolytic agents and topical nasal saline. Topical decongestants may be used in sinusitis with complications. Antihistamines are avoided due to their drying effect.

Antibiotics are also required for chronic sinusitis. As most of these patients have already failed a course of standard-dose amoxicillin, initial therapy consists of coamoxiclav, high-dose amoxicillin or cefuroxime. The duration of treatment is longer than for acute sinusitis, typically 3 to 6 weeks. Patients with penicillin allergy may be treated with a macrolide antibiotic, although there is increasing resistance of pathogens to these agents. Topical nasal steroids are occasionally useful for treatment.

Surgical intervention for acute sinusitis is limited to those with orbital or intracranial complications. Surgery may be considered for patients with chronic sinusitis who have not responded to aggressive medical management or who show anatomical obstruction after maximal medical management and extensive medical workup with allergy testing and immune evaluation. Adenoidectomy, to remove a potential bacterial reservoir for the sinuses, must be considered in younger children. The indications for endoscopic sinus surgery include patients with sinonasal polyposis, cystic fibrosis, failure to improve despite one-month course of medical therapy and any orbital or cranial complication.

Nasal Obstruction

Causes. Chronic mouth breathing in children is generally caused by blockage of nasal airflow. The site of nasal blockage is most often in the nasopharyngeal area due to adenoid hypertrophy. Intranasal causes of obstruction include allergic rhinitis, recurrent sinusitis, nasal septum deviation, turbinate hypertrophy, nasal polyps and less commonly, neoplasms. As a rule, bilateral nasal polyps do not occur in normal children and their presence should prompt testing for cystic fibrosis. Congenital causes of nasal airway obstruction include choanal stenosis or atresia, dermoid cysts, teratomas, encephaloceles, and pyriform aperture (bony opening to the nasal cavity in the skull) stenosis.

Diagnosis. Adenoid enlargement should be suspected in children, usually older than 2 yr, who present with nasal blockage, mouth breathing, sleep disturbance and chronic nasal discharge. Examination must rule out nasal pathology such as septal deviation or polyposis. Neonates with pyriform aperture stenosis may present with a single midline maxillary incisor. A CT scan or X-ray confirms the diagnosis (Fig. 13.5).

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Fig. 13.5: Lateral radiograph of the neck showing adenoid hypertrophy occluding the nasopharyngeal airway in a 6-yr-old boy (Courtesy, Textbook of ENT, Hazarika)

Treatment. Adenoidectomy is recommended for symptomatic younger children. Pubertal growth of the rnidface and regression of adenoid size tend to result in relief of adenoid-related nasal obstruction in children older than 9 yr. Pyriform aperture stenosis is treated with surgical drilling of the obstructing bony plates. Treatment for sinonasal polyposis includes topical and systemic steroids for limited disease and surgical intervention for larger, obstructing polyps.

Surgery on the nasal septum should be avoided in pre­ pubertal children, as it may lead to retardation in rnidface growth and saddling of the nasal dorsum. A conservative operation to correct a limited portion of the septum may be justified in a particularly symptomatic child. Turbinate hypertrophy usually responds to treatment of allergy, though electrocautery may be used in refractory cases.

Eplstaxls

Bleeding from the nose occurs frequently in children. Most pediatric epistaxis occurs in the anterior portion of the nasal septum at a confluence of arterial vessels known as Little's area (Kiesselbach plexus). Local trauma, especially nose picking, is by far the most common cause of pediatric epistaxis. Reduced ambient humidity also places the patient at risk. Examination reveals prominent vessels in Little's area that bleed promptly when touched with a cotton-tipped probe. Digital pressure by pinching the nose invariably stops the bleeding. Avoidance of nose picking, application of an antibiotic ointment for lubrication and,

forrefractorycases,cauterizationwithtopicalsilver nitrate or electrocautery are curative. Bleeding disorders must be suspected in children with suggestive family history, a history of frequent bleeding from other sites, or any nasal bleeding which does not respond in the usual fashion.

Less frequent causes of recurrent epistaxis include juvenile nasopharyngeal angiofibroma and hereditary hemorrhagic telangiectasia. The former is a benign, vascular tumor occurring exclusively in adolescent males that can cause profuse, brisk bleeding. Hereditary telan­ giectasia also known as Osler-Weber-Rendu syndrome, is a genetic defect in blood vessel structure resulting in arteriovenous malformations. Patients may suffer from severe, recurrent epistaxis, as well as gastrointestinal bleeds and pulmonary hemorrhage.

Choanal Atresia

Congenital failure of the nasal cavities to open into the nasopharynx is called choanal atresia. It results from failed resorption of the buccopharyngeal membrane either unilaterally or bilaterally or even partial with a severe stenosis. As neonates are obligate nasal breathers for 6 months, bilateral choanal atresia presents immediately afterbirth with respiratory distress. The affected baby cycles between silent cyanosis and crying. Suckling immediately precipitates cyanosis. Bilateral atresia can present as part of the CHARGE association, consisting of coloboma, heart abnormalities, choanal atresia, retardation of growth and development, genitourinary defects and ear anomalies.

Unilateral choanal atresia is a more indolent process and may present later in childhood with unilateral nasal discharge or blockage. Atresia typically manifests when theoppositenasalpassagebecomesblocked due to rhinitis or adenoid hypertrophy.

Diagnosis. Inability to pass an 8 French catheter can aid in diagnosis. Flexible nasal endoscopy confirms the diagnosis. CT scan demonstrates the atretic plate thickness and differentiates between bony and membranous atresia.

Treatment. Bilateral choanal atresia requires urgent management by inserting a finger in the baby's mouth and depressing the tongue down and forward away from the back of the throat. This should be replaced with a plastic oropharyngeal airway or a McGovern open-tip nipple. Failure of these measures may necessitate intubation or tracheostomy.

Treatment of choanal atresia is surgical. The two primary approaches are transpalatal and transnasal. Transnasal endoscopic repair isoften attempted first as it is less invasive. Transpalatal repair, which involves removal of the posterior hardpalate, is often reserved for failed endoscopic repair. Stents are placed in the nasal passages to prevent restenosis and are typically left in place for 3 to 6 weeks postoperatively.

DISEASES OF THE ORAL CAVITY AND PHARYNX

Inflammatory Disorders

Recurrent aphthous stomatitis is a common pediatric disorder that presents as painful white ulcers of variable size on the oral mucosa. The exact etiology is unknown. The ulcers resolve spontaneously over several days. If symptomatic management does not suffice, topical steroids and rarely, systemic steroids are employed, but may require intravenous fluids for dehydration.

Herpetic stomatitis presents in children with small, painful vesicles that evolveintograypseudomembranous mucosal ulcers. Antiviral medications may be used to hasten recovery, though the lesions usually heal spon­ taneously within 10-14 days. Once again intravenous fluids may be required.

Oral ca11didiasis (thrush) appears as small, white, curd-like lesions on the tongue and oral mucosa. In children under age 6 months or those on antibiotics, it is a benign finding. It can also be related to systemic diseases such as diabetes or immunodeficiency. Oral antifungals are effective.

Congenital Disorders

A11kyloglossia (tongue tie) is a limitation of anteriortongue mobility caused by a congenitally short lingual frenulum. This condition is not related to speech impairment.

Cleft palate may appear with or without cleft lip and can cause serious feeding difficulties. The etiology is multi­ factorial. Treatmentshould include staged reconstruction of the lip and palate defects and multidisciplinary management.

Micrognathia (small mandible), if severe, may displace the tongue posteriorly and cause respiratory distress in the neonate. Congenital micrognathia is most commonly seen with the Pierre Robin sequence, in which patients also have cleft palate and glossoptosis. If the micrognathia is severe, the neonate may require tracheostomy to secure the airway.

Macroglossia may be idiopathic or associated with syn­ dromes such as Down syndrome, Beckwith-Wiedemann syndrome and neurofibromatosis. If significant, the enlarged tongue may cause drooling, speech impairment and airway obstruction.

Lingual thyroid may present as a posterior midline tongue mass and is caused by an abnormal descent of the thyroid from the tongue base inutero. It maypresent with neonatal respiratory distress and may be associated with a thyroglossal dust cyst. As lingual thyroid often represents the only functioning thyroid tissue, its removal may necessitate chronic thyroid hormone supplementation.

Sore Throat

Viral pharyngitis is very common and is caused by a number of different pathogens including adenovirus,

enterovirus, coxsackievirus and parainfluenza virus. It typically presents with nonexudative pharyngeal erythema and tender cervical adenopathy. Upper respiratory complaints (rhinorrhea, nasal obstruction, cough, fever) are common. Treatment is supportive, as this is nearly always self-limited.

Infectious mononucleosis, caused by the Epstein Barr virus, presents with sore throat, gray pharyngeal exudate and soft palate edema. Patients show significant cervical lymphadenopathy and hepatosplenomegaly. Monospot or Paul-Bunnell tests are useful screening tests (only 40% accurate) and antibody titer confirms the diagnosis. Medical treatment is supportive and may include steroids for respiratory difficulty or severe dysphagia.

Acute bacterial pharyngotonsillitis is caused by group A

-hemolytic streptococci. Less common pathogens include nongroup A streptococcus, S. aureus (Fig.13.6), H. influ­ enzae, M. catarrhalis,diphtheria, gonococci, chlamydia and mycoplasma. Streptococcal pharyngitis presents as bil­ ateraltonsilhypertrophy and erythema with characteristic exudate. To distinguish between viral and bacterial pharyngotonsillitis, a rapid strep test should be obtained.

A negative result should be confirmed by throat culture. Treatment is with a ten-day course of penicillin VK or a first-generation cephalosporin. Both suppurative and nonsuppurative complications can result from incom­ pletely treated streptococcal pharyngitis. Non-suppura­ tive complications include scarlet fever, acute rheumatic fever and poststreptococcal glomerulonephritis. Suppura­ tive complications include peritonsillar, para-pharyngeal or retropharyngeal abscesses.

f"lg. 13.6: Acute staphylococcal pseudomembranous tonsillitis with unilateral hypertrophy of the right tonsil. This condition has to be differentiated from other causes of white patch on the tonsil (Courtesy:

Textbook of ENT, Hazarika)

Peritonsillar abscesstypically presents with a muffled voice, trismus and decreased oral intake. Physical examination reveals a unilateral displacement of the affected tonsil towards the midline with a bulge in the peritonsillar region and uvular deviation to the opposite side. CT scan may aid in diagnosis. Treatment consists of incision and drain­ age by experienced personnel. This should be followed by a 7 10day course of oral or parenteral penicillin or clindamycin. Steroids, to reduce pain and fever, may be considered as adjunctive therapy. Immediate tonsillec­ tomy (Quinsy tonsillectomy) may be performed, but has increased hemorrhage risk. A single peritonsillar abscess is a relative indication for tonsillectomy. Patients with recurrent abscesses should always be considered for tonsil removal.

Pharyngeal injury may occur in children after falling with a pen, stick or other sharp object in the mouth. Exami­ nation reveals a puncture or laceration of the soft palate, tonsil, or pharyngeal wall. The most significant risk is a carotid injury. The presence of significant bleeding, neurologic findings or a puncture lateral to the exposed tonsil should prompt immediate consultation and evaluation with angiography.

Adenotonsillectomy

Removal of the tonsils and adenoids is one of the most commonly performed pediatric operations. Recurrent tonsillitis is a common indication. More than 5-6 episodes of tonsillitis in a year or significant missed time from school or work should prompt consideration for tonsillectomy. Other indications include obstructive sleepapnea, suspicion of malignancy and previous peritonsillar abscess. Surgery is performed on an outpatient basis in older children. The most significant risk of tonsillectomy is postoperative hemorrhage.

Obstructive Sleep Apnea

Obstructive sleep apnea (OSA) is characterized by episodic obstruction of airflow through the upper airway during sleep. Adenotonsillar hypertrophy is the most common cause for pediatric OSA. Congenital nasal masses may be responsible for neonatal OSA. Physiologic sequelae may include hypoxemia, hypercapnia and acidosis. The most severely affected patients may develop failure to thrive, right ventricular hypertrophy, pulmonary hypertension and cor pulmonale.

Patients with OSA present with noisy breathing, specifically stertor (sonorous upper airway breathing).

Other symptoms include snoring, breath holding, or gasping during sleep, as well as enuresis. Daytime manifestations include morning headache, halitosis and behavioral disorders. Physical examination often reveals audible breathing with open mouth posture, hyponasal speech and tonsillar hyperplasia. Polysomnography (sleep study) remains the gold standard for diagnosis.

Adenotonsillectomy is considered first-line therapy in pediatric OSA. If the apnea hypopnea index is greater than

ten then the child should be monitored closely in the postoperative period with pulse oximetery. Surgical removal of nasal masses may be required. In the most severe cases of OSA, tracheostomy may be considered.

DISEASES OF THE LARYNX AND TRACHEA

Stridor

The term strider refers to excessively noisy, musical breathing and is generally due to upper airway obstruc­ tion. The relationship of strider to the respiratory cycle often provides a clue to its etiology: Inspiratory stridor suggests obstruction above the vocal cords (supraglottis), while expiratory stridor usually originates from the distal trachea (Table 13.4). Biphasic (inspiratory and expiratory) strider usually originates from a subglottic or proximal tracheal lesion. Most pediatric strider originates from supraglottic lesions.

Table 13.4: Supraglottic compared to tracheal obstruction

Evaluation of the stridorous child should include a thorough history. Physical findings include nasal flaring and suprasternal or intercostal retractions. Chest X-rays or lateral neck films may confirm diagnoses such as retropharyngeal abscess, epiglottitis, or croup. Barium esophagram or CT may rule out extrinsic vascular compression. Flexible and rigid endoscopy is generally needed to confirm the diagnosis. There are multiple causes of pediatric airway obstruction, some of which are listed below.

Infections

Croup (laryngotracheobronchitis) is a viral upper respiratory tract infection and often presents in children 1-5 yr of age with biphasic strider, barking cough and low-grade fever. Onset of symptoms is usually over several days. Chest X­ ray reveals a characteristic narrowing of the subglottic region known as the steeple sign (Fig. 13.7).

Most cases of croup are mild and resolve within 1 to 2 days. Conservative management should include reassurance, cool mist and oral hydration. Children with strider at rest should be hospitalized for close observation, cool mist and supplemental oxygen. Therapy with epinephrine (1:1000 in doses of 0.1-0.5 ml/kg to a maximum dose of 5 ml), gives through a nebulizer helps in relief of symptoms. A single dose of dexamethasone (0.3-0.6 mg/kg IM) reduces overall severity during first 24 hr. Recently, inhalation of budesonide in doses of 1 mg twice a day for 2 days has shown satisfactory results.

Fig. 13.7: Laryngotracheobronchitis (croup). 'Steeple sign'

Antibiotics are indicated only if the child fails to improve or if purulent secretions are present. Coverage should be directed towards Staphylococcus and H. influenzae.

Acute epiglottitis (often called supraglottitis), although less common than croup, typically presents with a greater degree of airway compromise. Patients typically present with acute onset (over several hours) of sore throat, marked dysphagia and high fever. Patients are often encountered leaning forward in a 'tripod' position, toxic­ appearing and drooling. Unlike croup, cough is frequently absent. Lateral neck X-ray reveals a characteristic thickening of the epiglottis ('thumbprint' sign) or other supraglottic structures. H. influenzae type B is the major etiologic organism.

If epiglottitis is suspected, rapid airway management is essential and includes intubation by skilled personnel. Instrumentation of the throat with tongue depressors is not advised as this can precipitate a fatal laryngospasm. Management includes securing the airway and broad­ spectrum IV antibiotics, e.g. coamoxiclav, ceftriaxone or cefuroxime. The incidence of epiglottitis has declined since the use of vaccines against H. influenzae.

Bacterial tracheitis is typically seen in younger children following viral upper respiratory tract infection. The child appears toxic with a brassy cough and strider. Patients have a classic irregular tracheal wall on X-ray. Bronchos­ copy is both diagnostic and therapeutic, as the purulent tracheal secretions can be visualised, cultured and mechanically debrided. Bacterial tracheitis is a relative medical emergency, as life-threatening obstruction may develop from these tracheal secretions. The responsible pathogen is usually S. aureus.

Retropharyngeal abscess is a potential suppurative complication of bacterial pharyngitis that may present with strider. Patients often have high fever, reduced mobility of the neck and appear toxic. Complications of

retropharyngeal abscess include spread of infection into the mediastinum. Mediastinitis is potentially fatal. Lateral neck radiograph reveals a bulge in the posterior pharyngeal wall. Treatment is by surgical drainage and broad-spectrum parenteral antibiotics.

Congenital Causes

Laryngomalacia is the most common congenital laryngeal anomaly, accounting for up to 60% and the most common cause of infant stridor. Inspiratory stridor is the hallmark of thecondition. Symptoms are typically aggravatedwhen the child is supine or crying. Flexible endoscopy reveals partial collapse of a flaccid supraglottic airway with inspiration. If present, gastroesophageal reflux, should be managed. Laryngomalacia is generally benign and self­ limited,as most casesresolveby18 monthsof age. Surgical intervention is advised for either respiratory distress or failure to thrive.

Vocal cord paralysis is the second most common congenital

laryngeal anomaly. Bilateral vocal cord paralysis usually

presents with a high-pitched inspiratory stridor and cyanosis. It is usually iatrogenic by excessive stretch of the neck during vaginal delivery producing an Erb's palsy of the recurrent nerve, but can also be idiopathic. Other causes may include Arnold-Chiari malformation,

hydrocephalus or hypoxia. Unilateral vocal cord paralysis,

in contrast, may present with a mild stridor or with signs of aspiration. Iatrogenic injury during ligation of patent ductus arteriosus is a frequent cause. Tracheostomy is requiredtosecuretheairwayin bilateral paralysis, though generally not in unilateral paralysis unless there is excessive aspiration.

Congenital subglottic stenosis is the third most common

congenital laryngeal anomaly. It results from incomplete recanalization of the laryngotracheal tube during embryonic development. Subglottic stenosis may present as recurrent episodes of stridor and may be mislabeled as

'croup'. Many cases resolve spontaneously as the child grows, while severe cases usually require tracheostomy. Surgical excision of the stenosis may be necessary to relieve the obstruction in these cases.

Vascular ringis a great vessel anomaly that causes extrinsic compression of both the trachea and the esophagus. The child with vascular ring anomaly usually presents with dysphagia as well as stridor.Contrastswallowing studies

(esophagram) or CT may reveal the diagnosis. Treatment for vascular anomalies is surgical.

Subglottic hemangioma is a benign vascular tumor that may present in the trachea. Infants usually become sympto­ matic between 3-6 months of life. Symptoms include biphasic stridor and a barkingcough.Up to 50% may have concurrent cutaneous head and neck hemangiomas. Imagingmayreveal asymmetricsubglottic narrowing. The diagnosis in confirmed with endoscopy. Treatment

options include primarily oral propranolol, intralesional steroids, CO2 laser excision, tracheostomy, and open surgical excision.

Congenital saccular cyst, laryngeal web and laryngeal atresia

are rare laryngeal anomalies. They present with airway obstruction and require surgical intervention.

Iatrogenic Causes

Acquired subglottic stenosis is the most common cause of

acquired stridor. It most often results from longterm endotracheal intubation and subsequent scar formation.

Minor stenosis may be observed, while more severe stenosis may be treated by a variety of surgical methods including tracheostomy, widening of the stenosis with cartilage grafts, and excision of the stenotic segment.

Laryngeal granuloma may also result from prolonged intubation. Endoscopy reveals a vocal cord granuloma.

These are often amenable to endoscopic removal.

Neoplasms

Recurrent respiratory papilloma is the most common benign

laryngeal tumor and presents with gradual airway obstruction. Endoscopy reveals single or multiple irregular, wart-like masses in the larynx or pharynx. The conditionis caused by human papillomavirus; HPV types 6 and 11 are the most common. Transmission is believed to be vertical, from the passage of the fetus through an infected birth canal. Treatment is with CO2 laser ablation or microdebrider excision of the papillomas. Adjunctive therapies include intralesional cidofovir and alpha-inter­ feron. Multiple surgical procedures are usually necessary as the disease typically recurs.

Foreign Body Aspiration

Foreign body aspiration should always be considered as a potential cause of stridor and airway obstruction in children. Foreign bodies most commonly aspirated are food and coins. Conforming objects such as balloons pose the greatest risk of choking death, followed by round objects such as balls or marbles. After establishing airway patency, urgent endoscopicvisualization and removal by an experienced surgeon are necessary.

Hoarseness

Vocal nodules are the most common cause of hoarseness in children and are generally caused by vocal abuse. They are seen more frequently in habitually shouting or screaming children, usually boys with siblings. The severity of hoarseness fluctuates, worsening with vocal abuse and improving with rest. Endoscopy reveals small, bilateral, opposing nodules at the junction of the anterior and middle-thirds of the vocal cord. Speech therapy is usually effective in older children. Surgery is rarely indicated.

Reflux laryngitis may result from gastric secretions spilling onto the larynx. Reflux has been implicated in numerous diseases of the head and neck, ranging from laryngitis and subglottic stenosis to chronic sinusitis and otitis media with effusion. Diagnosis is best established with 24 hr pH monitoring. Medical management is usually effective, though surgical fundoplication may be needed in severe cases.

Hypothyroid myxedema may occasionally cause an increase in vocal fold edema and present as hoarseness or stridor. Thyroid function tests should be conducted in the hoarse child with a clinical history suggestive of hypothyroidism.

Laryngotracheal cleft is a rare congenital defect in the posterior cricoid cartilage of the larynx. In its mildest form, children with this process may experience feeding diffi­ culty, recurrent respiratory tract infection or hoarseness. In its more severe forms, the cleft may extend inferiorly between the entire trachea and esophagus. Severe clefts usually cause significant aspiration pneumonias and are often not compatible with life. The condition may be associated with hereditary conditions such as Opitz-Frias or Pallister-Hall syndromes. Management of symptomatic clefts is surgical.

DISEASES OF THE SALIVARY GLANDS

Infections

Bacterial parotid sialoadenitis is frequent in small children

and presents with painful unilateral parotid swelling. Purulent material may be expressed from the parotid duct intraorally with parotid massage. Dehydration leads to staphylococcal overgrowth in the duct system. Treatment includes oral antibiotics as well as hydration, sialogogues, massage and warm compresses.

Viral parotitis is caused most often by the mumps virus. Patients generally present with painful parotid enlarge­ ment and fever. They may also present with an acute unilateral hearing loss or vestibular weakness. Systemic manifestations such as meningoencephalitis, pancreatitis and orchitis may also be present.

Tuberculosis is the most common granulomatous inflam­ mation of the parotid. It may be limited to the salivary glands without lung involvement. Sarcoidosis may also present with unilateral or bilateral parotid swelling. It is usually seen with systemic symptoms and peripheral adenopathy. A variety of laboratory tests and radiological studies including chest radiograph support the diagnosis. Steroids are of value in treating xerostomia.

Human immunodeficiency virus (HIV) involvement of the

parotid glands is common, presenting as bilateral intraglandular cysts. The cysts invariably recur after aspiration.

Drooling

Drooling (sialorrhea) is a common, self-limited finding in young children. However, in children with neuromuscular disorders, dysphagia and poor lip closure may result in chronic drooling. If the swallowing mechanism is also abnormal, pooled secretions may allowchronic aspiration, leading to pneumonia or other complications.

Medical therapy for drooling not controlled with speech therapy consists of drying agents such as glycopyrrolate and antihistamines. Refractory cases of sialorrhea may be treated surgically with salivary gland excision; ductal ligationor rerouting; destruction of parasympathetic fibers; or some combination of the above. At present, the preferred surgical treatment is bilateral submandibular gland excision with parotid duct ligation. Tracheostomy or separation of the trachea from the upper airway is reserved for profound and life-threatening chronic aspiration.

SuggestedReading

Diseases of the Enr

American Academy of Pediatrics and American Academy of Family Physicians. Clinical Practice Guideline: Subcommittee on management of acute otitis media. Pediatrics 2004;113:1451-65

Leach AJ, Morris PS. Antibiotics for the prevention of acute and chronic suppurative otitis media in children. Cocluane Database Syst Rev 2006;4:4401

Powers JH. Diagnosis and treatment of acute otitis media: Evaluating the evidence. Infect Dis Clin N Am 2007;21:409-26

Smith JA and Danner CJ. Complications of cluonic otitis media and cholesteatoma. Otolaryngol Clin N Am 2006;39:1237-55

Diseases of the nose

Daniel SJ. The upper airway: Congenital malformations. Paed Resp Reviews 2006;75:5260-3

Lusk R. Pediatric cluonic rhinosinusitis. Curr Opin Otolaryngol Head Neck Surg 2005;14:393-6

Diseases of the oral cavity and pharynx

Darrow DH. Surgery for pediatric sleep apnea. Otolaryngol Clin N Arn 2007;40:855-75

Mueller OT, Callanan VP. Congenital malformations of the oral cavity. Otolaryngol Clin N Am 2007;40:141-60

Diseases of the lary11x and trachea

Ahmad SM, Soliman A. Congenital anomalies of the larynx. Otolaryngol Clin N Am 2007;40:177-91

Bjornson C, Russell KF, Vandermeer B, et al. Nebulized epinephrine for croup in children. Cochrane Database Syst Rev 2011;2:CD006619

Brodsky L, Carr MM. Extraesophageal reflux in children. Curr Opin Otolaryngol Head Neck Surg 2006;14:387-92

Knutson D, Aring A. Viral croup. Am Fam Physician 2004;69: 535-40

Rafei K, Lichenstein R. Airway infectiousdisease emergencies. Pediatr Clin North Arn 2006; 53: 215-42

Russell KF, Liang Y, O'Gormon K, et al. Glucocorticoids for croup. Cochrane Database Syst Rev 2011;1:CD001955

Diseases of the salivary glands

Mehta D, Willging JP. Pediatric salivary gland lesions. Sernin Pediatr Surg 2006;15:76-4

COM!v10N RESPIRATORY SYMPTOMS

Cough

After maximal inspiration, the air is suddenly released through the partially closed glottis, because of forceful contractionof the expiratory muscles. Thisproducesabout of cough. The cough reflex is controlled by a center in the medulla. Irritation of the pharynx, larynx, trachea, bronchi andpleuratransmittheafferentimpulsesthroughthevagus or glossopharyngeal nerves. Efferent pathways are in the nerve supply to the larynx and respiratory muscles.

Cough is an important defense mechanism of the respiratory system and helps to bring out the infected secretions from the trachea and bronchi. Cough should not be suppressed in younger children as retention of secretions in their lungs may result in atelectasis and pulmonary complications. On the other hand, persistent cough interferes with the sleep and feeding. It fatigues the child and may result in vomiting.

Etiology

Acute cough Causes include:

i.Upper respiratory tract infection (rhinovirus, influ­ enza virus, parainfluenza, respiratorysyncytialvirus,

adenovirus), postnasal discharge due to sinusitis (streptococci, Haemophilus influenzae or Moraxella, usually in older children), rhinitis, hypertrophied tonsils and adenoids, pharyngitis, laryngitis and tracheobronchitis

ii.Nasobronchial allergy and asthma

iii.Bronchiolitis

iv.Pneumonia and pulmonary suppuration (5. pneu­ moniae, 5. aureus, H. influenzae, Klebsiella, Chlamydia, Mycoplasma, gram-negative bacilli, viral pneumonia)

v.Measles

vi.Whooping cough and related syndromes (Bordetella pertussis, parapertussis, respiratory syncytial virus, adenovirus)

vii.Foreign body in the air passage

viii.Empyema

Chronic and recurrent cough Causes are as follows:

i.Inflammatory disorders of airway, such as: (a) asthma; (b)infections, e.g.viral, bacterial, chlamydia, mycoplasma, tuberculosis, parasitic infections; (c) inhalation of environmental irritants such as tobacco

smoke, dust; and (d) Loeffler syndrome.

ii.Suppurative lung disease, including: (a) bron­ chiectasis, (b)cystic fibrosis; (c)foreign body retained in bronchi; (d) congenital malformations, seques­ trated lobe, or bronchomalacia; and (e) immuno­ deficiency or primary ciliary dyskinesia.

iii.Anatomiclesions, tumors, tracheal stenosisor H-type tracheoesophageal fistula.

iv.Miscellaneous causes, e.g. (a) psychogenic, habit cough; (b) postnasal discharge, sinusitis; (c) gastro­ esophageal reflux disease; and (d) interstitial lung disease.

Expectoration

Young children are not able to expectorate and usually swallow the respiratory secretions. Older children with chronic respiratory problems may be able to bring out expectoration. Common causes of expectoration are bronchiectasis due to various causes, lung abscess, bronchitis, asthma and tuberculosis. The amount and nature of expectoration may give clue about the cause of respiratorydisease. Investigationssuchascellcount, Gram stain and culture or stain for AFB and culture help in diagnosis and guide for treatment.

Hemoptysis

Causes of hemoptysis in children include necrotizing pneumonia, foreign body aspiration, bleeding diathesis, cavitatory tuberculosis, idiopathic pulmonary hemo­ siderosis, mitral stenosis, dilated cardiomyopathy and Goodpasture syndrome.

- Essential Pediatrics

Respiratory Noises

The intensity and pitch of respiratory sounds depend on their site of origin within the respiratory tract. The pitch of the sound keeps increasing and the intensity keeps decreasing with decreasing size of the respiratory tract (Table 14.1). For example, snoring is a highly intense but lowpitchedsoundbecauseit resultsfrom the oropharynx. Wheeze is a high pitched, less intense sound originating from lower airway obstruction. As a rule, extrathoracic airway obstruction produce inspiratory sounds. Intra­ thoracic major airwayobstruction produces inspiratory as well as expiratory sounds while distal airway obstruction predominantly produce expiratory sounds.

Rattling

Rattling is due to excessive secretions in the pharynx or tracheobronchial tree during breathing. It is present in asthma, bronchitisandtracheobronchialstenosis. Inhalation of gastrointestinal content into the tracheobronchial tree can also result in rattling. Some normal infants may have transient rattling but prolonged rattling is pathological.

Wheezing

should be distinguished from rniliary tuberculosis. The leukocyte count shows eosinophilia. The patients are treated with diethylcarbamazine (10 mg/kg) in 3 divided doses orally for 2 to 3 weeks. Two or three spaced courses may be given.

Loeffler syndrome The pulmonary phase of migration of ascaris larvae may cause wheezing, pulmonary pro­ blems and eosinophilia. These features are characteris­ tically transient.

Inhaledforeignbodies cause unilateral localizedwheeze which begin suddenly. Wheezing tends to be continuous and becomes worse with crying, during excitement and with cold.

Rare causes These include pressure from enlarged mediastinal nodes or from anomalous left pulmonary artery compressing the right main bronchus.

Cystic fibrosis Recurrent wheezing, productive cough and malabsorption are usual features. Some may have history of meconium ileus in the neonatal period.

Stridor

Wheezing refers to high pitched whistling sounds audible without auscultation. Partial obstruction of the bronchi and bronchioles produces wheezing. Sufficient air must flow through the narrowed airway to produce the wheezing sotmd. This may be due tocauseswithin thelumenor in the wallsof thebronchi. The causesofwheezing arelistedbelow.

Wheeze associated lower respiratory tract infec­ tion Wheezingis mostoften due toheightenedsensitivity of the respiratory tract. Infections of the lower respiratory passages may cause bronchospasm in these patients. Attacks of wheezing are always preceded by a cold or acute respiratory disease. These aremostfrequent between 3 and8 yr of age and become less frequent thereafter. These attacks are relieved by bronchodilators.

Bronchiolitis (see page 381) Bronchial asthma (see page 382)

Tropical eosinophilia This is morefrequentin adultsthan inchildren.It is anunusualformofinfectionwith filariasis, e.g. Diroftlaria imitis, W. bancrofti, B. malayi. Clinical features simulate chronic recurrent asthma. X-ray films show fine pulmonary infiltrates with snowflake appearance. This

Stridor indicates upper respiratory obstruction and is usually accompanied by hoarseness, brassy cough, dys­ pnea, chestretractionsandrestlessness.Stridor is frequent in infants and is often attributed to (i) small size of the larynx; (ii) loose submucous connective tissue around the glottic region; and (iii) rigid cricoid cartilage encircling the subglottic zone.

Acute stridor Acute upper airway obstruction occurring in the region of glottis which isproduced byinflammation and edema may be life-threatening. The obstruction may either be supraglottic as in epiglottitis or subglottic as in infectious croup (Table 14.2).

Table 14.2: Stridor due to supraglottic and tracheal obstruction

Chronic stridor Common causes are listed below:

Congenital laryngeal stridor. This is caused by flaccidity (laryngomalacia) or easy collapsibility of the aryepiglottic folds or epiglottis. This condition usually manifests by the end of the first week or during the second week afterbirth. The stridor is characteristically intermittent and is aggravated by crying or feeding. It is modified in sleep or by the change of posture. The loud inspiratory sound frightens the parents but the infant is relatively less symptomatic. Respiratory distress and chest retraction are absent or minimal. Feeding behavior and activity of the infant are generally normal. Breathing difficulty may be significant, if micrognathia and cleft palate are also associated. Congenital laryngeal stridor disappears spontaneously by theageofsixmonths toone year. These infants are more prone to develop aspiration of feeds and frequent lung infection.

Congenital laryngeal or tracheal stenosis or web. Cry of the infant is weak and hoarse, breathing is labored and the airentry inlungsisreduced. Insubglottic tracheal stenosis, the cry is unaffected and the stridor is both inspiratory and expiratory.

Laryngeal cysts or neoplasm. Angioma, papilloma, lymph­ angioma and retention cysts may be responsible for stridor.

Neurogenic stridor. Bilateral vocal cord paralysis results from brainstem injury. Unilateral paralysis is due to the involvement of the peripheral nerve. The left recurrent laryngeal nerveis more liable to injury since it has alonger course and hooks around the aorta from the front to back.

Extrinsic obstruction. Vascular rings cause intermittent stridor that becomes worse when the neck is flexed. The infant prefers to keep the head in the position of hyper­ extension. Other causes of external airway compression are mediastinal goiter, lymphangioma and thyroglossal duct cyst. Congenital goiters which cause respiratory obstruction and stridor are due to maternal intake of antithyroid drugs and iodides during pregnancy. Goiter of neonatal hypothyroidism and that due to defect in the synthesis of thyroid hormones are usually not so big as to cause stridor.

Miscellaneous causes. Stridor is common in infants with hydrocephalus and those with Down syndrome. Other causes include micrognathia and glossoptosis, macro­ glossia and diaphragmatic hernia.

Treatment The diagnosis of congenitallaryngeal stridor can be established only by direct laryngoscopy. Fluoroscopy after barium swallow should be done to rule out the extrinsic causes of obstruction. Tumors and cysts require surgical excision. Corticosteroids hasten the recovery in laryngeal edema. Congenital laryngeal stridor does not require treatment. Gavage feeding is done if the respiratory distress is marked. Congenital goiter caused by the administration of antithyroid drugs or iodides to

Disorders of Respiratory System -

the mother during pregnancy is treated with triiodo­ thyronine and Lugol's iodine.

Dyspnea

Tachypnea refers to abnormally rapid respiration, while dyspnea means labored or difficult breathing, usually accompanied by pain and air hunger. The causes of dyspnearangefromillnessesaffecting thelungs,heartand musculoskeletal system.

Epistaxis

Epistaxis or bleeding from the nose is rare in children below the age of 3 yr. It may occur due to local or systemic causes. Local causes include: (i) trauma to nose caused by nose picking, (ii) capillary malformations in the Little's area, (iii) foreign body, (iv) bleeding polyps of the septum, and (v) allergic rhinitis and nasal diphtheria. Systemic causes of epistaxis are: (i) hypertension, (ii) blood dyscrasia, (iii) emphysema, and (iv) pertussis.

Pressure on alae nasi for 10 min controls bleeding in most cases ofepistaxis. Inresistantcases,the nasalmucosa is plugged with gauze piece soaked in 1:10,000 solution of adrenaline hydrochloride as a temporary measure. Plugging of the nose for a prolonged period (>48 hr) should be avoided. The bleeding points should be identi­ fied and cauterized with silver nitrate solution. Nasal bleed due to systemic causes should be evaluated and treated appropriately. The child should receive treatment with iron supplements to raise hemoglobin level. Profuse bleeding ismore likely to befrom theposterioraspectfrom sphenopalatine vessels. Cauterization is ineffective in these vessels. Firm anterior and posterior packing is done. Blooddyscrasiaifpresentshouldbe appropriately treated.

Suggested Reading

Kabra SK. History taking and physical examination. In: Essential Pediatric Pulmonology, 2ndedn. KabraSK, Lodha R (eds). Nobel Vision, New Delhi, 2010;15-22

INVESTIGATIONS FOR RESPIRATORY ILLNESSES

Bronchoscopy

Bronchoscopy can be of two types, fiberoptic and rigid. Fiberoptic bronchoscopy is done under local anesthesia and sedation. This is used for diagnosis of structural abnormality of airways,diagnosisofforeignbodyandfor obtaining bronchoalveolar lavagesamplestoidentifycell typeand infective etiology oflowerrespiratorytract. Rigid bronchoscopy can be used in place of fiberoptic broncho­ scopy. This is commonly used for removal of foreign bodies from airways or obtaining biopsy from airway tumors.

Pulmonary Function Tests

Pulmonary function tests (PFT) are important tools for monitoring of a patient with chronic respiratory illness. Flow rates and lung volumes are measured. The proce-

- Essential Pediatrics

dure requires cooperation of the patient. PFT may be performed inchildren above the age of 5-7 yr. Commonly used parameters include: forced expiratory volume in first second (FEVl), forced vital capacity (FVC), mid­ expiratory flow rate and ratio of FEVl/FVC. Normal FEVl/FVC ratio is between 0.8 and 1.0. In obstructive diseases (asthma) the ratio is reduced. In restrictive lung diseases (interstitial lung disease), the ratio ofFEVl/FVC is normal but FVC is reduced below 80% of predicted.

Blood Gas Analysis

Estimationofpartialpressuresofoxygen (Pa02) and carbon dioxide (PaC02) in blood along with blood pH gives a fair estimateofpulmonaryfunctions.Arterialbloodgasanalysis is useful in making a diagnosis of respiratory failure as well as for monitoring children with acute and chronic respiratory failure. Pa02 below 60 mmHgandPaC02 over 50 mm Hg suggest acute respiratory failure.

Imaging

Noninvasive diagnostic methods include X-rays, most commonly used to diagnose pulmonary infections and computerized tomography scans, used for visualization of lymphnodes,tumors,bronchiectasisandpleuralpathologies.

Sweat Chloride Test

Chloride in sweat is increased in children suffering from cystic fibrosis. Sweat chloride is estimated by quantitative pilocarpine iontophoresis. Values of sweat chloride in normal children is less than 40 mEq/1. Patients with cystic fibrosis show levels more than 60 mEq/1.

Suggested Reading

Beydon N, Davis SD, Lombardi E, et al. An official American Thoracic Society/European Respiratory Society statement: pulmonary fw1etion testing in preschool children. Am J Respir Crit Care Med 2007;175: 1304--45

Kabra SK, Lodha R. Investigations in pulmonology. In: Essential Pediatric Pulrnonology. New Delhi, Nobel Vision 2010;23-9

Nicolai T. The role of rigid and flexible bronchoscopy in children. Pediatr Respir Rev 2011;12:190-5

RESPIRATORY TRACT INFECTIONS

Common Cold or Nasopharyngitis

Common cold is a frequent illness in childhood and is usuallycaused by infections of the upperrespiratorytract with adenoviruses, influenza, rhinovirus, parainfluenza or respiratory syncytial viruses. These are spread by droplet infection. Predisposing factors include chilling, sudden exposure to cold air and overcrowding. Rhinitis could also be due to allergy.

Clinical features include fever, thin nasal discharge and irritability. Cervical lymph nodes may enlarge. Naso­ pharyngeal congestion causes nasal obstruction and respiratory distress. The latter is more pronounced in young infants. Eustachian tube opening may be blocked

leading to serous otitis media and congestion of tympanic membrane. In allergic rhinitis there is a clear mucoid discharge with sneezing.

Narrowing of the airway and pharyngeal irritation causes dry hacking cough. Excessive lacrirnation is due to the blocked lacrirnal ducts in the nose.Nasal discharge may become purulent, if secondarily infected especially in younger children. Purulent discharge does not necessarily mean secondary infection as it canresult from shedding of epithelial and inflammatory cells resulting from viral infection itself. The illness usually lastsfor three days but cold may persist up to two weeks.

Complications include otitis media, laryngitis, sinusitis, bronchiolitis, exacerbation of asthma and broncho­ pneumonia.

Differentialdiagnosisincludethepresenceofforeignbody which presents withunilateral serosanguineous or purulent discharge from a nostril. The intermittent use of rifarnpicin may cause flu-like syndrome in some children. Drugs like reserpine andprochlorperazine cause nasal stuffiness. Clear mucoiddischargefrom the nose in the first few weeksoflife is called snuffles. Snuffles of congenital syphilis is severe rhinitiswithbilateralserosanguineousdischargecommonly excoriating the upper lip and leaving fine scars. Nasal strictures may ulcerate leaving a flat nasal bridge.

Treatment

Relieve nasalcongestion. Babiessneeze andblowoutthe nasal discharge, if their anterior nares are tickled by the tip of a handkerchief. Nose drops of saline may give symptomatic relief. Nasal decongestants (ephedrine, xylometozoline) may cause rebound congestion. These should not be used routinely and used only in refractory cases for limited duration. Antihistaminics are best avoided in the first six months of life, but give symptomatic relief by drying up thinsecretionsandrelievingsneezing.Nonsedatingagents, e.g. loratidine andcetirizinemay beusedin allergicrhinitis. Terfenadine should not be prescribed in children because of potential cardiotoxicity.

Fever is controlled by antipyretics such as paracetamol (acetaminophen). Cough syrups should not be given. If the cough is suppressed in infants and young children, mucoid secretions may be retained in the bronchi and this maypredisposetospasmodiccough, wheezing, atelectasis and suppuration.

Antibiotics are of little value in viral infections. These are used ifthe secretionsbecomepurulent, the fever continues torise and ifthe childdevelopsbronchopneumonia. There is no evidence that large doses of vitamin C are helpful. The children should be protected from sudden exposure to chills and kept warm during the winter months.

Acute Tonsillopharyngitis

Sorethroatisduetoacuteinflammationofthepharynxand tonsils.Most often, it isassociatedwith the viral infections