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Esophageal atresia

Esophageal atresia is a congenital medical condition (birth defect) which affects the alimentary tract. It causes the esophagus to end in a blind-ended pouch rather than connecting normally to the stomach. It occurs in approximately 1 in 4425 live births. more...

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This condition takes several different forms, often involving one or more fistulas connecting the trachea to the esophagus (tracheoesophageal fistula). Approximately 85% of affected babies will have a 'lower fistula'.

This birth defect arises in the fourth fetal week, when the trachea and esophagus should begin to separate from each other. Other birth defects may co-exist, particularly in the heart, but sometimes also in the anus, spinal column, or kidneys.

This condition is visible some time shortly before birth on an ultrasound, or it may be detected soon after birth as the affected infant will be unable to swallow its own saliva. Any attempt at feeding could cause aspiration pneumonia as the milk collects in the blind pouch and overflows into the trachea and lungs. Furthermore, a fistula between the lower esophagus and trachea may allow stomach acid to flow into the lungs and cause damage. Because of these dangers, the condition must be treated as soon as possible after birth.

Treatments for the condition vary depending on its severity. The most immediate and effective treatment in the majority of cases is a surgical repair to close the fistula/s and reconnect the two ends of the esophagus to each other. This is not possible in all cases, since the gap between upper and lower esophageal segments may be too long to bridge. In this situation, a gastrostomy is performed, allowing tube feedings into the stomach through the abdominal wall, and often a cervical esophagostomy will also be done, to allow the saliva which is swallowed to drain out a hole in the neck. Months or years later, the esophagus may be repaired, sometimes by using a segment of bowel brought up into the chest, interposing between the upper and lower segments of esophagus.

Post operative complications sometimes arise, including a leak at the site of closure of the esophagus. Sometimes a stricture, or tight spot, will develop in the esophagus, making it difficult to swallow. This can usually be dilated using medical instruments. In later life, most children with this disorder will have some trouble with either swallowing or heartburn or both.


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Obstructive sleep apnea in children
From American Family Physician, 3/1/04 by James Chan

Obstructive sleep-disordered breathing is common in children. Snoring, mouth breathing, and obstructive sleep apnea (OSA) often prompt parents to seek medical attention for their children. The estimated prevalence of snoring in children is 3 to 12 percent, while OSA affects 1 to 10 percent. (1-3) The majority of these children have mild symptoms, and many outgrow the condition. OSA often results from adenotonsillar hypertrophy, neuromuscular disease, and craniofacial abnormalities.

Sleep-disordered breathing refers to a pathophysiologic continuum that includes snoring, upper airway resistance syndrome, obstructive hypopnea syndrome, and OSA. (4) The mildest form of OSA in children is upper airway resistance syndrome. Affected children have symptoms of OSA but lack the accompanying polysomnographic findings. While many children demonstrate intermittent snoring and mouth breathing, true OSA results in detrimental clinical sequelae such as failure to thrive, behavior problems, enuresis, and cor pulmonale.

Sleep-disordered breathing in children is a timely public health concern, given the increasing rates of obesity and hyperactivity in this population. As demonstrated in one study, (5) a large percentage of children with hyperactivity or inattentive behaviors had underlying sleep-disordered breathing. These children would be cared for more effectively with appropriate recognition and treatment of sleep-disordered breathing than with the use of stimulant medications.


Physical examination reveals adenotonsillar hypertrophy in most children with OSA. There is some evidence that adenotonsillectomy improves clinical symptoms. (6-8) [Strength-of-recommendation (SOR) Evidence level B, clinical cohort studies] However, many children with documented adenotonsillar hypertrophy never have symptoms of OSA. This finding suggests that the etiology of OSA in children may result from a complex interplay between adenotonsillar hypertrophy and loss of neuromuscular tone. Children with craniofacial syndromes have fixed anatomic variations that predispose them to airway obstruction, while in children with neuromuscular disease, obstruction is caused by hypotonia.

Clinical Manifestations


The presenting problem in children with sleep-disordered breathing depends on the child's age. In children younger than five years, snoring is the most common complaint (Table 1). Other nighttime symptoms frequently reported by parents include mouth breathing, diaphoresis, paradoxic rib-cage movement, restlessness, frequent awakenings, and witnessed apneic episodes.

Children five years and older commonly exhibit enuresis, behavior problems, deficient attention span, and failure to thrive, in addition to snoring. Compared with adults, fewer children with OSA report excessive daytime somnolence, with the notable exception of obese children. (9) In extreme cases of OSA in children, cor pulmonale and pulmonary hypertension may be the presenting problems.

Poor growth and failure to thrive are more common in children with sleep-disordered breathing. (10) Growth velocity increases after adenotonsillectomy. (11) The hypothesized etiology for failure to thrive is increased work of breathing, with subsequent increase in baseline caloric expenditure. Decreased production of growth hormone during fragmented sleep may contribute further to poor growth.

Enuresis associated with OSA often resolves after successful treatment of sleep-disordered breathing. (12) Increased urine production results from hormonal disregulation. These alterations are accompanied by increased levels of catecholamines and frequent arousal that further contribute to enuresis. (4)

Behavior and cognitive deficits can recur in children with OSA. (13) Poor academic performance in the teenaged years is associated with snoring. (14) Reports that these performance deficits resolve after successful treatment of OSA suggest causation. (14) Intermittent nocturnal hypoxia accompanied by frequent arousals from sleep (documented by electroencephalography) results in sleep fragmentation. (15) The neurobehavioral consequence of this sequence is altered behavior in children.


A thorough physical examination of a child suspected of having OSA must include evaluation of the child's general appearance, with careful attention to craniofacial characteristics such as midface hypoplasia, micrognathia, and occlusal relationships. Evaluation for nasal obstruction depends on the child's age. Septal deviation, choanal atresia, nasolacrimal cysts, and nasal aperture stenosis must be considered in infants. In older children, nasal polyps and turbinate hypertrophy must be ruled out.

When examining the oral cavity, physicians should evaluate the geometry of the soft palate for size, redundancy, and clefting; document the size of the tongue and tonsils (Figure 1); and perform lateral neck radiography (Figure 2) or a nasopharyngoscopic examination to evaluate the size of the adenoidal tissue and the site of airway collapse. Detection of tonsillar hypertrophy on routine examination should prompt physicians to question parents about snoring and other symptoms of OSA in their children.

The physical examination must include a neurologic survey for hypotonia and an assessment for obesity. If examination findings do not correlate with the reported severity of snoring and apnea, children should be evaluated for less common causes of sleep-disordered breathing (Table 2).


The role of polysomnography in the diagnosis of childhood sleep-disordered breathing remains controversial. Although polysomnography is the current gold standard, authorities cite the lack of reliable sleep laboratories for children, excess cost, and lack of consensus on interpretation of polysomnograms as reasons it is not required for diagnosis. (16,17)

The parameters originally used to evaluate childhood polysomnograms were based on adult values. OSA in adults is defined as a respiratory pause lasting more than 10 seconds. Because of children's different physiology and higher baseline respiratory rate, clinically relevant apneas may not last this long. Apneas of three to four seconds' duration can be accompanied by desaturations. These findings have led to the development of separate guidelines for the interpretation of polysomnograms in children. (18) [SOR evidence level B, study of polysomnograms of normal children]

In children, an apnea-hypopnea index greater than 1 (average: 0.1 to 0.5 events per hour) or a minimum oxygen saturation of less than 92 percent (average: 96 percent [+ or -] 2 percent) is considered abnormal (Table 3). The apnea-hypopnea index is calculated as the average number of apneas and hypopneas per hour of sleep.

Children may have sleep disruption because of an increased effort to breathe but show no evidence of apnea on polysomnography. (19) This condition is called upper airway resistance syndrome. The clinical significance of upper airway resistance syndrome remains controversial and is under investigation. To document the increased respiratory effort in children with upper airway resistance syndrome, esophageal pressure monitoring is necessary. A pressure probe placed in the esophagus measures frequent or extreme negative pressures that lead to sleep disruption. Currently, esophageal pressure monitoring is not routinely available in most sleep laboratories.

The reliability of clinical assessment in the diagnosis of sleep-disordered breathing has not been determined. Several studies indicate that parents' observation of their child's breathing is an inaccurate basis for the diagnosis of OSA. (20,21) [SOR evidence level B, clinical cohort studies] Clinical evaluation that included witnessed apneas, mouth breathing, tonsil size, and snoring was found to have poor predictive accuracy. (21)

Adenotonsillectomy should be considered first-line treatment for sleep-disordered breathing in children when there is physical evidence of adenotonsillar hypertrophy (Figure 3). (22) [SOR evidence level C, expert opinion] Adenotonsillectomy, a routine procedure, has been shown to improve snoring, OSA, weight problems, enuresis, and behavior problems in children who have the entire clinical spectrum of sleep-disordered breathing. (4,11,13) Polysomnography is necessary for diagnosis and treatment of patients with multiple medical comorbidities, children with craniofacial syndromes, and patients with an unclear etiology (i.e., modest physical findings or examination findings inconsistent with severity of apnea), and to determine the degree of apnea. (22)



The use of video photography to record apneic events and nighttime arousals in children has demonstrated mixed results and poor predictive accuracy. (23) Airway fluoroscopy provides information on the degree of obstruction and the dynamics of the child's airway. However, this procedure rarely is used because nasopharyngoscopy is more reliable and can be performed during the initial office visit.

Lateral neck radiography provides useful information about the size of the adenoids and their relationship to the upper airway. (24) Concurrent cervical computed tomography and magnetic resonance imaging also demonstrate pharyngeal lymphoid hyperplasia. When nasopharyngoscopy is unavailable, lateral neck radiography should be considered as a reliable alternative for detecting adenoidal hypertrophy.

Pulse oximetry, often recommended as a screening tool, is not an accurate predictor of OSA because large numbers of children have sleep disturbance without desaturations. (25) In addition, a subgroup of children who have nighttime desaturations do not have OSA.



Several options are available for the medical management of OSA in children. Continuous positive airway pressure (CPAP) is effective in children with OSA. CPAP is the treatment of choice when adenotonsillectomy is contraindicated or has failed. CPAP is difficult for approximately 20 percent of children to tolerate. (26) Because children grow rapidly, frequent follow-up visits are necessary, and the mask must be adjusted at least every six months.

If snoring and OSA occur intermittently and are associated with recurrent tonsillitis or adenoiditis, antibiotic therapy may help. Reduction in the bacterial antigen load, secondary reduction in the population of B lymphoctyes in the germinal centers of tonsils and adenoids, and the physics of airflow (Poiseuille's law) are such that small changes in airway diameter dramatically affect airway resistance. Reduction of post-infectious lymphoid hyperplasia relieves the obstruction. Even small reductions in adenotonsillar size can eliminate snoring and OSA. An established pattern of recurrent infections accompanied by sleep disturbance may warrant consideration of adenotonsillectomy.

In obese children, weight loss is an excellent therapeutic measure, but it can be a difficult process. Allergy testing and treatment of rhinitis are important in children with OSA secondary to nasal obstruction.

Nasal steroid use may have a role but is unlikely to provide definitive therapy. (27) [SOR evidence level A, randomized controlled trial] Nasal steroids should be prescribed temporarily until a referral can be made for treatment. While systemic steroids are used to decrease upper airway obstruction in patients with infectious mononucleosis (because of antiinflammatory and lympholytic effects), one study of systemic steroids demonstrated no effect on the size of tonsils or adenoids, severity on polysomnography, or symptomatology in patients with OSA. (28)


Adenotonsillectomy remains the treatment of choice for most children with a strong clinical history of OSA or with OSA documented by polysomnography. Anatomically, the tonsils and adenoids represent the most common area of hypertrophy that contributes to airway obstruction. Numerous studies have documented improvement in snoring, OSA, enuresis, behavior, and growth following adenotonsillectomy. (6,7)

Uvulopalatopharyngoplasty is indicated when a thick soft palate and a long uvula are present. In addition, uvulopalatopharyngoplasty should be considered for children with modest adenotonsillar hypertrophy but severe symptoms of OSA, those with polysomnographically documented severe OSA, and children with trisomy 21. (29)

Tracheotomy, the definitive surgery for upper airway obstruction, is reserved for use in children with severe OSA who have failed to improve with other medical and surgical treatments and in special cases in which these modalities are contraindicated or not tolerated. Tracheotomy must be considered in children for whom traditional surgery is unlikely to be of benefit, such as those with Pierre Robin syndrome.

Children with Craniofacial Syndromes

OSA is more common in children with craniofacial syndromes. Children who have syndromes with craniosynostosis, such as Apert's syndrome, Crouzon's disease, Pfeiffer's syndrome, and Saethre-Chotzen syndrome; abnormalities of the skull base; and accompanying maxillary hypoplasia may have nasopharyngeal obstruction. Children with syndromes that involve micrognathia, such as Treacher Collins syndrome, Pierre Robin syndrome, and Goldenhar's syndrome, become obstructed at the hypopharyngeal level. In children with trisomy 21, a narrow upper airway combined with macroglossia and hypotonic musculature predisposes them to OSA.

The surgical management of craniofacial syndromes and OSA in children frequently requires more than standard adenotonsillectomy. Tracheotomy often is necessary. In children with midfacial hypoplasia, craniofacial advancement may be indicated. Glossopexy, mandibular distraction or advancement, or tongue suspension should be considered in patients with micrognathia. (30)

Postoperative Care


Common complications of adenotonsillectomy in patients with OSA include dehydration (1 to 3 percent of patients), hemorrhage (1 to 2 percent), and velopharyngeal insufficiency (less than 1 percent). (31) Velopharyngeal insufficiency refers to the "cleft-palate" type of hypernasal voice and nasopharyngeal reflux of food that result from incomplete approximation of the soft palate with the posterior pharyngeal wall. Complications such as nasopharyngeal stenosis and post-obstructive pulmonary edema are rare.

While most children with sleep-disordered breathing can be treated safely on an outpatient basis, children with severe OSA demonstrated on polysomnography, those younger than three years, and those with medical comorbidities, neuromuscular disease, bleeding diatheses, or syndromes should be admitted and monitored overnight after surgery. (22) Postoperative respiratory complications occur in children with a high preoperative apnea-hypopnea index, with rates of complications ranging from zero to 27 percent.

In children with craniofacial syndromes, preoperative polysomnography suggestive of severe OSA, or OSA that is refractory to standard management, repeating polysomnography six weeks after surgery is recommended. However, in most patients, postoperative polysomnography remains unnecessary.

The authors indicate that they do not have any conflicts of interest. Sources of funding: none reported.


(1.) Owen GO, Canter RJ, Robinson A. Overnight pulse oximetry in snoring and non-snoring children. Clin Otolaryngol 1995;20:402-6.

(2.) Hultcrantz E, Lofstrand-Tidestrom B, Ahlquist-Rastad J. The epidemiology of sleep related breathing disorder in children. Int J Pediatr Otorhinolaryngol 1995;32(suppl):S63-6.

(3.) Ferreira AM, Clemente V, Gozal D, Gomes A, Pissarra C, Cesar H, et al. Snoring in Portuguese primary school children. Pediatrics 2000;106:E64.

(4.) Messner AH, Pelayo R. Pediatric sleep-related breathing disorders. Am J Otolaryngol 2000;21: 98-107.

(5.) Chervin RD, Archbold KH, Dillon JE, Panahi P, Pituch K J, Dahl RE, et al. Inattention, hyperactivity, and symptoms of sleep-disordered breathing. Pediatrics 2002;109:449-56.

(6.) Suen JS, Arnold JE, Brooks LJ. Adenotonsillectomy for treatment of obstructive sleep apnea in children. Arch Otolaryngol Head Neck Surg 1995;121: 525-30.

(7.) Nieminen P, Tolonen U, Lopponen H. Snoring and obstructive sleep apnea in children: a 6-month follow-up study. Arch Otolaryngol Head Neck Surg 2000;126:481-6.

(8.) Lim J, McKean M. Adenotonsillectomy for obstructive sleep apnea in children. Cochrane Database Syst Rev 2003;(4):CD003136.

(9.) Gozal D, Wang M, Pope DW Jr. Objective sleepiness measures in pediatric obstructive sleep apnea. Pediatrics 2001;108:693-7.

(10.) Lind MG, Lundell BP. Tonsillar hyperplasia in children. A cause of obstructive sleep apneas, CO2 retention, and retarded growth. Arch Otolaryngol 1982;108:650-4.

(11.) Freezer NJ, Bucens IK, Robertson CF. Obstructive sleep apnoea presenting as failure to thrive in infancy. J Paediatr Child Health 1995;31:172-5.

(12.) Weider DJ, Sateia MJ, West RP. Nocturnal enuresis in children with upper airway obstruction. Otolaryngol Head Neck Surg 1991;105:427-32.

(13.) Blunden S, Lushington K, Kennedy D, Martin J, Dawson D. Behavior and neurocognitive performance in children aged 5-10 years who snore compared to controls. J Clin Exp Neuropsychol 2000; 22:554-68.

(14.) Gozal D, Pope DW Jr. Snoring during early childhood and academic performance at ages thirteen to fourteen years. Pediatrics 2001;107:1394-9.

(15.) Owens J, Opipari L, Nobile C, Spirito A. Sleep and daytime behavior in children with obstructive sleep apnea and behavioral sleep disorders. Pediatrics 1998;102:1178-84.

(16.) Goldstein NA, Sculerati N, Walsleben JA, Bhatia N, Friedman DM, Rapoport DM. Clinical diagnosis of pediatric obstructive sleep apnea validated by polysomnography. Otolaryngol Head Neck Surg 1994;111:611-7.

(17.) Messner AH. Evaluation of obstructive sleep apnea by polysomnography prior to pediatric adenotonsillectomy. Arch Otolaryngol Head Neck Surg 1999;125:353-6.

(18.) Marcus CL, Omlin KJ, Basinki DJ, Bailey SL, Rachal AB, Von Pechmann WS, et al. Normal polysomnographic values for children and adolescents. Am Rev Respir Dis 1992;146(5 pt 1):1235-9.

(19.) Guilleminault C, Pelayo R, Leger D, Clerk A, Bocian RC. Recognition of sleep-disordered breathing in children. Pediatrics 1996;98:871-82.

(20.) Carroll JL, McColley SA, Marcus CL, Curtis S, Loughlin GM. Inability of clinical history to distinguish primary snoring from obstructive sleep apnea syndrome in children. Chest 1995;108:610-8.

(21.) Wang RC, Elkins TP, Keech D, Wauquier A, Hubbard D. Accuracy of clinical evaluation in pediatric obstructive sleep apnea. Otolaryngol Head Neck Surg 1998;118:69-73.

(22.) Schechter MS, for the Section on Pediatric Pulmonology, Subcommittee on Obstructive Sleep Apnea Syndrome. Technical report: diagnosis and management of childhood obstructive sleep apnea syndrome. Pediatrics 2002;109:E69.

(23.) Lamm C, Mandeli J, Kattan M. Evaluation of home audiotapes as an abbreviated test for obstructive sleep apnea syndrome (OSAS) in children. Pediatr Pulmonol 1999;27:267-72.

(24.) Cohen LM, Koltai PJ, Scott JR. Lateral cervical radiographs and adenoid size: do they correlate? Ear Nose Throat J 1992;71:638-42.

(25.) Brouillette RT, Morielli A, Leimanis A, Waters KA, Luciano R, Ducharme FM. Nocturnal pulse oximetry as an abbreviated testing modality for pediatric obstructive sleep apnea. Pediatrics 2000;105:405-12.

(26.) Marcus CL, Ward SL, Mallory GB, Rosen CL, Beckerman RC, Weese-Mayer DE, et al. Use of nasal continuous positive airway pressure as treatment of childhood obstructive sleep apnea. J Pediatr 1995;127:88-94.

(27.) Brouillette RT, Manoukian JJ, Ducharme FM, Oudjhane K, Earle LG, Ladan S, et al. Efficacy of fluticasone nasal spray for pediatric obstructive sleep apnea. J Pediatr 2001;138:838-44.

(28.) Al-Ghamdi SA, Manoukian JJ, Morielli A, Oudjhane K, Ducharme FM, Brouillette RT. Do systemic corticosteroids effectively treat obstructive sleep apnea secondary to adenotonsillar hypertrophy? Laryngoscope 1997;107:1382-7.

(29.) Strome M. Obstructive sleep apnea in Down syndrome children: a surgical approach. Laryngoscope 1986;96:1340-2.

(30.) Hoeve HL, Joosten KF, van den Berg S. Management of obstructive sleep apnea syndrome in children with craniofacial malformation. Int J Pediatr Otorhinolaryngol 1999;49(Suppl 1):S59-61.

(31.) Randall DA, Hoffer ME. Complications of tonsillectomy and adenoidectomy. Otolaryngol Head Neck Surg 1998;118:61-8.

JAMES CHAN, M.D., is a resident in the Department of Otolaryngology at the Cleveland Clinic Foundation. He received his medical degree from the University of Rochester School of Medicine and Dentistry, N.Y.

JENNIFER C. EDMAN, M.D., is a resident in the Department of Family Practice at Fairview Hospital, Cleveland. She received her medical degree from the University of Rochester School of Medicine and Dentistry and completed an internship in pediatrics at Rainbow Babies & Children's Hospital, Cleveland.

PETER J. KOLTAI, M.D., is head of the Section of Pediatric Otolaryngology at the Cleveland Clinic Foundation. Dr. Koltai received his medical degree from Albany Medical College, N.Y. He completed residency training in surgery and otolaryngology at the University of Texas Medical Branch, Galveston. Dr. Koltai received fellowship training in pediatric otolaryngology at the Hospital for Sick Children, London.

Address correspondence to Peter J. Koltai, M.D., Cleveland Clinic Foundation, A71, 9500 Euclid Ave., Cleveland, OH 44195 (e-mail: Reprints are not available from the authors.

COPYRIGHT 2004 American Academy of Family Physicians
COPYRIGHT 2004 Gale Group

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