Subglottic stenosis can be congenital something you are born with or acquired usually due to scar tissue. Pediatric subglottic stenosis occurs when a childs airway becomes narrow in the larynx voice box. Management of congenital tracheal stenosis american academy. We report our surgical techniques, comprising anterior. Frequent or longterm intubation having a breathing tube is the most common cause of acquired subglottic stenosis in children and adults. Severe congenital subglottic stenosis in association with.
The severity of symptoms correlates with the length of affected trachea, the presence of concomitant respiratory conditions, degree of luminal narrowing, and any bronchial involvement. The patient had weak crying after birth and stridor was noticed from one month old. The most common cause of ac quired stenosis is postintubation. Three patients with congenital subglottic stenosis are presented and whole organ serial. Stenotic lesions involving the glottis and subglottis can be ac quired or congenital in nature. Several surgical techniques have been introduced, but outcomes of these procedures remain unclear, because css is rare and few reports have described surgical results. He had tracheostomy at two month old and the incision of the web was attempted under laryngomicrosurgery with co2 laser, although the attempt failed due to combined subglottic stenosis.
Congenital stenosis may be caused by either a small cricoid cartilage or a thick sub mucosa secondary to. With congenital subglottic stenosis, varying degrees of stridor are usually present after birth. Management of congenital tracheal stenosis sophie c. Introduction sgs congenital or acquired narrowing of the subglottic airway 3. Sgs in infants represents one of the most difficult problems facing the otolaryngologist. Subglottic stenosis is often therefore used to describe central airway narrowing in children, and laryngotracheal stenosis is more often used in adults. Anjali sharma, in nelson pediatric symptombased diagnosis, 2018. Congenital subglottic stenosis acquired subglottic stenosis. Congenital subglottic stenosis jama otolaryngologyhead. As the name suggests, congenital subglottic stenosis is a birth defect. Children who develop subglottic stenosis are subject to prolonged periods in hospital and are often referred to specialist units. Subglottic stenosis may be caused by trauma or infection acquired or may be there at birth congenital. Subglottic stenosis may be considered congenital or acquired.
With the introduction of prolonged intubation in neonates mid 1960s, acquired sgs became the most frequent cause of laryngeal stenosis. The subglottis is the area just beneath the vocal folds. Sep 30, 2010 congenital subglottic stenosis c sgs is defined as a restriction of the subglottic diameter to less than 4. Congenital subglottic stenosis is a rare birth defect, often occurring as part of a genetic syndrome with other symptoms. The main clinical sign isthepersistence ofstridor from birth. In the fullterm neonate, subglottic stenosis sgs is defined as a lumen. Strategy for surgical treatment of congenital subglottic stenosis in. It is often less severe if it is congenital, improving as the larynx grows. Laser surgeries and dilations are temporary short term fixes. The narrowest and most susceptible area of the subglottic trachea is the portion circumscribed by the cricoid cartilage as opposed to other portions where the cartilage rings are incomplete and the dimensions are wider.
The most common causes of acquired subglottic stenosis are prolonged tracheal intubation and tracheostomy, especially a high tracheostomy. The classification of congenital subglottic stenosis is based on study of the englishlanguage literature and the collection in the laryngeal development laboratory at the childrens memorial hospital in chicago. Thompson diagnosed is a condition called subglottic stenosis, a lifethreatening narrowing of the upper portion of the windpipe subglottis. Rare congenital subglottic stenosis treated with airway. Congenital familial subglottic stenosis article pdf available in otolaryngology head and neck surgery 1472 suppl. Although it is relatively rare, it is the third most common congenital airway problem after laryngomalacia and vocal cord paralysis. Congenital subglottic stenosis lies on a continuum of embryologic failure that includes laryngeal atresia, stenosis, and webs.
Congenital subglottic stenosis css is a rare anomaly caused by thickened cricoid cartilage. Subglottic stenosis can present as a lifethreatening airway emergency. Previously, the term subglottic stenosis was often. In the preterm infant, it is defined as a lumen pdf available in otolaryngology head and neck surgery 1472 suppl. Endoscopic view of the true vocal cords in the foreground and the elliptical congenital subglottic stenosis sgs in the center of the picture.
Subglottic stenosis complicating cardiac surgery in children. Idiopathic subglottic stenosis is a narrowing of the airway due to an unknown cause. Strategy for surgical treatment of congenital subglottic. Objectivehypothesis neonatal subglottic stenosis is a known entity arising from endotracheal tube intubation. Subglottic stenosis childrens hospital of philadelphia. Current followup has been obtained in 146 92% which constitutes the determinate group. Cricoid cartilage, being a complete, nonpliable and nonexpandable ring, is also the narrowest area of the entire human airway. It is the third most common congenital anomaly of the larynx after laryngomalacia and vocal cord paralysis, and the most common laryngeal anomaly necessitating. Subglottic stenosis as defined in this paper is a developmental defect.
P229p229 august 2012 with 26 reads how we measure reads. Symptoms include highpitched noisy breathing stridor. Subglottic stenosis can be of three forms, namely congenital subglottic stenosis, idiopathic subglottic stenosis iss and acquired subglottic stenosis. Prolonged endotracheal intubation subglottic balloon dilation. Tracheostomy is often required and management is not without. Hofferberth, mbbs a, karen watters, mb, bch, bao, mph b, reza rahbar, dmd, md, francis fynnthompson, md abstract congenital tracheal stenosis cts is a serious and rare condition. A severe case of congenital laryngeal web with subglottic stenosis was reported. Of the remaining 74 cases, the largest group 34 was congenital subglottic stenosis. Congenital subglottic stenosis epidemiology congenital sgs the third most common cause of neonatal stridor, and is thought to represent between 5 and 15 % of airway malformations 25, 26. Although there is a female preponderance, no familial cases have been reported in the literature. The aim of this study was to clarify outcomes of our surgical management and to. It is imperative that the otolaryngologist be an expert at dealing with the diagnosis and management of. Pdf subglottic stenosis in infants and children researchgate.
Ppt subglottic stenosis powerpoint presentation free. Congenital laryngeal atresia was first described by rossi in. Congenital tracheal stenosis cts is a rare but potentially lifethreatening disorder that often leads to severe respiratory insufficiency. Incidence of subglottic stenosis in intubated neonates.
Histopathologically, congenital sgs can be subdivided into cartilaginous and membranous. Management of congenital tracheal stenosis american. University of texas medical branch galveston, tx department of otolaryngology head and neck surgery 2. Management of subglottic stenosis and subglottic stenosis. Intubation occurs when a tube is inserted into the trachea to help maintain breathing during. Congenital tracheal stenosis cts is a serious and rare condition. Other reports 36 support the fact that subglottic stenosis occurs more frequently than is realized. It is a narrowing of the windpipe below my vocal cords. Fifteen of these children appeared to have acquired subglottic stenosis secondary to endotracheal intubation. Congenital subglottic stenosiscongenital subglottic stenosis thought to be secondary to failure ofthought to be secondary to failure of laryngeal lumen to recanalizelaryngeal lumen to recanalize defined as subglottic diameter less thandefined as subglottic diameter less than 4. In the 1970s and 1980s, estimates of the incidence of subglottic stenosis were in the range of 0. Acute management of unsuspected subglottic stenosis by. The cartilaginous type results from a thickened or distorted cricoid cartilage, creating an anterior subglottic shelf that extends to the posterior region. The aim of this study was to clarify outcomes of our surgical management and to outline our surgical strategy for css.
Congenital estenosiw and deformations of respiratory system q30q34 bronchiectasis pulmonary hypoplasia pulmonary sequestration congenital cystic adenomatoid malformation. Clinical characteristics and treatment of subglottic stenosis in patients with wegeners granulomatosis subglottic stenosis is a complication of wegeners granulomatosis caused by tracheal tissue damage and scarring. Subglottic stenosis sgs is a narrowing of the subglottic airway see image below, which is housed in the cricoid cartilage. Acquired subglottic stenosis is often linked to having a breathing tube in for a long time. This trapped first ring is demonstrated in horizontal, sagittal and coronal planes. Subglottic stenosis tests and treatments rush university. Subglottic stenosis cincinnati childrens hospital medical. Paediatric postintubation subglottic stenosis archives of.
Critically, cts is a disorder that can lead to life. Subglottic stenosis may be managed medically, as well as by endoscopic resection using electrocoagulation, criotherapy or laser ray, or by several surgical procedures. Severe congenital subglottic stenosis in association with congenital duodenal obstruction you will receive an email whenever this article is corrected, updated, or cited in the literature. Subglottic stenosis sgs is a congenital or acquired condition characterized by a narrowing of the upper airway extending from just below the vocal folds to the lower border of the cricoid cartilage. Histopathology of congenital subglottic stenosis tucker. Subglottic stenosis in infants and children article pdf available in singapore medical journal 5111.
Idiopathic subglottic stenosis has a female preponderance, usually affecting women between 30 and 50 years of age 3. The overall mortality rate for the 293 children was 29%. Subglottic stenosis healthcare baylor college of medicine. Subglottic stenosis can be caused by trauma, prolonged intubation with a breathing tube, infection, or the irritating reflux of. Subglottic stenosis is airway narrowing below the vocal folds. Intubation occurs when a tube is inserted into the trachea to help maintain breathing during a medical or surgical procedure. The subglottic airway is the narrowest area of the airway because it is a complete, nonexpandable, and nonpliable ring, unlike the trachea, which has a posterior membranous section, and the larynx, which has a posteri. Congenital subglottic stenosis c sgs is defined as a restriction of the subglottic diameter to less than 4. Symptoms of acquired and congenital subglottic stenosis are similar and basically involve mild biphasic.
Subglottic stenosis of congenital origin is less commonby comparison and can only be diagnosed with certainty in a child before its first intubation. Acquired subglottic stenosis often occurs after long periods of intubation and ventilation for respiratory problems congenital subglottic stenosis occurs as a rare birth defect and may be associated with other genetic syndromes and conditions. Subglottic stenosis is a narrowing of the airway in the part of the voice box below the vocal cords glottis. A superiorly displaced first tracheal ring is observed to form a cartilaginous subglottic stenosis in one. You can manage this and all other alerts in my account. The larynx is located above the trachea windpipe and below the vocal cords subglottis.
Histopathology of congenital subglottic stenosis lauren. Of 158 cases of subglottic stenosis 115 were congenital and 43 acquired. It is a more severe form of disease than the congenital variety and almost always requires operative intervention. The cause of congenital subglottic stenosis is unknown. This type is the most common and mild form of congenital subglottic stenosis. Subglottic stenosis will involve narrowing of the cricoid, the only complete cartilage ring in the airway. In this situation, the cartilage in the trachea, or windpipe, never formed completely during fetal development. Acquired subglottic stenosis can develop when scar tissue forms in the trachea due to prolonged intubation, previous airway surgery or both. Tracheostomy was necessary in the management of 15. When severe, subglottic stenosis, causes noisy breathing during inspiration and expiration. This narrowing is often caused by scarring in the larynx just below the vocal cords but may also involve the vocal cords and. Key words laryngotracheal stenosis intubation tracheotomy airway obstruction management introduction laryngotracheal stenosis is a congenital or acquired narrowing of the airway, representing a continuum of disease that may affect the glottis, subglottis, andor trachea. Paediatric postintubation subglottic stenosis archives.
Subglottic stenosis is a narrowing of the subglottis the area of the windpipe just below the vocal folds. The stridor becomes worse as the child grows, and is precipitated when there is an upper respiratory infection. Petahtiqva, israel congenital subglottic stenosis, which islifethreatening tothe infant, can bediagnosed bythe correlation ofthe clinical and the roentgenologic findings. Aside from cases of congenital subglottic stenosis in siblings at birth 4, there are no cases of adult familial subglottic stenosis described in the literature. Congenital subglottic stenosis is the third most common congenital anomaly. Subglottic stenosis causes shortness of breath, especially during times of exertion. Clinical characteristics and treatment of subglottic. Aug 29, 2012 congenital subglottic stenosis css is a rare anomaly caused by thickened cricoid cartilage. Subglottic stenosis an overview sciencedirect topics. Thought to be secondary to failure of laryngeal lumen to recanalize. We describe two pairs of sisters, as well as a mother and a. Congenital subglottic stenosis occurs when the airway cartilage does not develop as it should before birth and leads to a narrowing of the airway. Acquired subglottic stenosis may develop secondary to endotracheal intubation, particularly if.
Idiopathic subglottic stenosis is a narrowing of the trachea at the level of the cricoid cartilage of unknown etiology. Histopathology of congenital subglottic stenosis lauren d. Subglottic stenosis can be divided into congenital or acquired. Subglottic stenosis can be caused by trauma, prolonged intubation with a breathing tube, infection, or the irritating reflux of stomach acid, explains dr. Neonatal subglottic stenosisincidence and trends walner. Eighteen of the 603 children were diagnosed as having subglottic stenosis. Different degrees of atresia, stenosis, or webbing can be found in these patients fig.
Living with idiopathic subglottic stenosis rare disease day. It is situated in the region of the cricoid cartilage. Subglottic stenosis sgs is a narrowing of the airway below the vocal cords subglottis and above the trachea. In its mildest form, congenital subglottic stenosis merely represents a normalappearing cricoid with a smallerthanaverage diameter, usually with an elliptical shape. Congenital subglottic stenosis classification css can be classified into 2 types. Two patients were diagnosed with definite congenital stenosis and 16 patients with acquired stenosis.
Subglottic stenosis sgs can be congenital rare or acquired. I had 3 tracheal dilation surgeries before undergoing a tracheal resection in 20. Subglottic stenosis complicating cardiac surgery in. Illustration of thyroid cartilage, vocal fold, cricoid cartilage, trachea and thyroid gland. Acquired and congenital subglottic stenosis in the infant. Subglottic stenosis is a congenital or acquired narrowing of the subglottic airway. Epidemiology frequency the frequency of congenital subglottic stenosis sgs is unknown. Management of severe pediatric subglottic stenosis with. Chronic subglottic stenosis is defined as a partial narrowing to complete obliteration of the airway bounded by the inferior margin of the cricoid at the caudal side and cranially by the insertion of the fibres of the conus elasticus into the true vocal cords. Congenital subglottic stenosis is a rare anomaly caused by thickened cricoid cartilage. It may occur as a presenting feature of the disease leading to diagnosis, or instead as a latestage manifestation.
Subglottic stenosis secondary to lymphoplasmacytic lymphoma. The most common cause of acquired sgs is prolonged intubation. Congenital subglottic stenosis occurs when the subglottic space is narrowed, and typically presents in children younger than 3 months with respiratory distress, biphasic or inspiratory stridor, and recurrent croup. Subglottic stenosis is the narrowing of the subglottic airway or lumen. Twentynine specimens with congenital cricoid abnormalities have been identified. Beyond the normal vocal folds is a circular narrowing of the airway. The incidences related to congenital sgs had a significant increment in the late 1960s. It is a rare condition for which the real incidence has never been established owing to the difficulty of making the diagnosis.
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