Congenital Subglottic Stenosis Research Articles

Cricotracheal Resection in Children Weighing Less Than 10 kg

To review cricotracheal resection (CTR) in children weighing less than 10 kg. Retrospective study of 17 patients (mean follow-up, 23 months) from 3 ear, nose, and throat pediatric centers. Seventeen children (10 boys and 7 girls; mean age, 14.6 months; and mean weight, 7.6 kg) undergoing CTR from June 1995 to March 2003. Decannulation rates and endoscopies. The cause was congenital subglottic stenosis in 2 children (12%) and acquired subglottic stenosis in 15 (88%). All but 1 had grade 3 or 4 stenosis. The mean hospitalization duration was 34 days. Single-stage CTR was performed in 11 children (65%), with peroperative decannulation in 7. Extubation of these patients occurred between days 3 and 9. Decannulation of the other 6 patients was performed after a median of 15 days. Sixteen (94%) of the 17 children were decannulated. Four patients required additional carbon dioxide laser treatment for subsequent glottic or subglottic edema or granulomas, but no reintubation was necessary. One child could not be decannulated because of bronchopulmonary disease, and subglottic stenosis recurred. Long-term tracheotomy was avoided in all other patients. Another child died of cardiac disease. All other patients remained free of significant subglottic stenosis at follow-up. Cricotracheal resection in small children weighing less than 10 kg was a safe and effective procedure for severe subglottic stenosis. To our knowledge, this is the first reported attempt of CTR in this weight category, providing results comparable to those published in older children.

Congenital stridor due to bilateral vocal cord palsy.

Congenital stridor is one of the rare presentations of respiratory distress at birth. The commonest cause of congenital stridor is laryngomalacia, which accounts for 60% of the causes. The other common causes are congenital subglottic stenosis and vocal cord palsy (VCP). VCP is usually unilateral and most often linked with birth trauma, and is temporary. Bilateral palsy can be associated with other congenital anomalies. The current report describes a case of congenital bilateral VCP, not related to birth trauma and severe enough to require tracheostomy.

Changes in speech and language development of a young child after decannulation

This report reviews the speech and language development of a child who, as result of complete subglottic stenosis, was aphonic from birth until 2 years and 11 months of age at which time laryngotracheal reconstruction provided normal respiration. The boy had congenital subglottic stenosis requiring neonatal tracheostomy. The congenital subglottic stenosis progressed to complete subglottic stenosis during the neonatal period. The child's speech and language development was monitored for a 24-week period following airway reconstruction. Learning outcomes: The reader will learn about and be able to describe: (1) the speech and language development of a child who was aphonic from birth until 35 months of age, (2) the effects of surgical repair of a tracheotomy on the child's speech and language development, (3) the likely importance of babbling in speech and language development.

Laryngotracheal reconstruction in congenital laryngeal webs and atresias

To review the role of open laryngotracheal reconstruction (LTR) in congenital laryngeal webs and atresias. A retrospective chart review was undertaken in a specialist otorhinolaryngology unit in an academic tertiary referral paediatric hospital. Twenty-one patients with congenital laryngeal subglottic stenosis (SGS) underwent LTR between 1993 and 2003. Two groups were identified; one group had SGS alone (n = 6) and the other group had SGS associated with a laryngeal web (n = 15). Information recorded included presenting features, classification of lesion, surgery performed, and whether decannulation was achieved. All grades of stenosis (Myer-Cotton classification) and webs of type 2, 3, and 4 (Cohen's classification) were recorded. Fifteen patients had a staged reconstruction and 6 patients had a single-stage LTR. Complications included 2 revision procedures, 3 tracheocutaneous fistulas, and 1 death (cause unrelated). All appropriate patients were successfully decannulated at between 3 and 18 months post-LTR (median, 5 months). This series illustrates the effective role of open LTR in both congenital SGS alone and that in association with glottic webbing.

Laryngeal myxedema: An unusual etiology of infant stridor

Objectives: We report the clinical findings of a child with laryngeal myxedema and stridor due to untreated congenital hypothyroidism. A review of the literature regarding the incidence, radiologic findings, and clinical characteristics of this rare, but life-threatening disease are presented. Methods: Case presentation of a 7-month-old infant from Micronesia who presented to our service with stridor and severe hypoxemia. The clinical features of untreated congenital hypothyroidism will be presented along with a review of the literature. Results: A 7-month-old male was admitted to the pediatric service for stridor and hypoxemia. Examination of the infant revealed biphasic stridor in all positions, reduced oxygen saturation on room air, retractions, and grunting. On nasopharyngoscopic exam, the patient had diffuse edema of the larynx and supraglottis that obscured the normal anatomy. He also had diffuse hypotonia and generalized weakness. Thyroid function tests were obtained, and the patient was found to have severe hypothyroidism. He was started on levothyroxine therapy, and subsequently had a decrease in his laryngeal edema and improvement in his oxygenation. Conclusion: Although most stridor in infants is due to laryngomalacia, vocal cord paralysis, or congenital subglottic stenosis, less common etiologies are still encountered and must be kept within a broad differential diagnosis in order to achieve appropriate patient care.

Unanticipated Difficult Intubation in a Patient with Asymptomatic Congenital Subglottic Stenosis Caused by a Laryngeal Web and Thyroid Cartilage Anomaly: A case report

Both a laryngeal web and thyroid cartilage anomaly are rare airway abnormalities and can cause the obliteration of the tracheal diameter, which may be asymptomatic or symptomatic. In asymptomatic patients, the presence of unsuspected obliteration of the tracheal diameter may be problematic if endotracheal intubation is indicated. We report a case of unsuspected difficult intubation caused by a combined laryngeal web and congenital anomaly of the thyroid cartilage. A 14-year-old female was scheduled for corrective surgery for idiopathic scoliosis of the spine. After injection of IV induction agent and muscle relaxant, most of the vocal cords were exposed clearly by laryngoscopy, but a 6.0 mm internal diameter (ID) endotracheal tube could not be advanced below the level of the vocal cords because of resistance. Intubation was re-attempted with smaller tube (5.0 mm ID) only to fail. We awakened the patient and consulted an otolaryngologist. An Otolaryngologic examination and radiologic study revealed the narrowing of the trachea was caused by a laryngeal web and congenital anomaly of the thyroid cartilage. Later, a tracheotomy was performed under local anesthesia and then the laryngeal web and anomalous portion of the thyroid cartilage were removed under general anesthesia.

Fire during the use of Nd–Yag laser

Fire during the use of Nd–Yag laser is a very rare complication. A case report of laser fire during the use of Nd–Yag laser for congenital subglottic stenosis is reported. The probable causes for the fire, management and short review of literature are dealt with. This case report highlights the importance of following the safety protocol during laser surgery.

Thyroid alar cartilage laryngotracheal reconstruction for severe pediatric subglottic stenosis

Background/Purpose: Laryngotracheoplasty has become an accepted treatment alternative for subglottic stenosis. However, the best autogenous material for laryngotracheoplasty remains controversial. Autogenous superior thyroid alar cartilage (TAC) has been used successfully in single stage laryngotracheal reconstruction in children with subglottic stenosis. Methods: This is a retrospective study of 6 children (mean age, 16.6 months) undergoing TAC graft laryngotracheoplasty between September 1995, and June 1999. Two children had immediate tracheal intubation for congenital subglottic stenosis. Four others had previous tracheostomy: 3 for severe postintubation subglottic stenosis and 1 for congenital subglottic stenosis. After an anterior cricoid split, a piece of TAC was sutured between the cut ends of the cricoid, with the graft perichondrium facing intraluminally. Endotracheal intubation was maintained postoperatively. Results: Four children underwent successfully extubation 9 to 21 days (mean, 15.5 days) postoperatively. Two required tracheostomy, which was maintained because of severe laryngomalacia and laryngotracheobronchomalacia. One child was treated with CO2 laser because of symptomatic recurrence of the subglottic stenosis 3 weeks after the surgery; another required fundoplication for gastroesophageal reflux 12 months after laryngotracheoplasty. There were no donor site complications in any of the 6 cases. Repeat laryngoscopy and bronchoscopy showed a patent subglottic airway. All of them are without symptoms after a mean follow-up of 26 months. Conclusions: (1) This preliminary experience indicates that the TAC graft technique is a viable option for laryngotracheal reconstruction; (2) the TAC graft has significant advantages, including a single operative incision and absence of donor-site morbidity. J Pediatr Surg 36:1258-1261. Copyright © 2001 by W.B. Saunders Company.

Apoptosis in the developing human cricoid cartilage: A pilot study

Apoptosis is widely recognized as a major phenomenon in normal development. Deficiencies in this process may lead to developmental abnormalities such as congenital subglottic stenosis. We studied apoptosis using in situ end labeling of the 3′-OH ends of fragmented DNA in 5 progressively older, normal, human cricoid cartilage specimens. Results show that apoptosis is a very active process in fetal and neonatal tissue. The process gradually slows with advancing age. In the 4- and 13-year-old specimens, minimal to no apoptosis was seen. We conclude that apoptosis plays a critical role in the intraluminal and extraluminal expansion of the cricoid cartilage. (Otolaryngol Head Neck Surg 2000;123:677-81.)

Surgery of subglottic stenosis in neonates and children.

The excellent management of patients in the different Intensive Care Units has decreased mortality but, as a side effect, we have to treat an increasing number of patients with airway problems secondary to prolonged intubation. The clinical records of patients diagnosed of acquired or congenital subglottic stenosis (SE) between 1990 and 1995 were retrospectively reviewed. Types of treatment included conservative, endoscopic, and open surgery: anterior cricoid split (ACS), anterior laryngotracheoplasty (ALTP) and anteroposterior laryngotracheoplasty (APLTP). 46 patients had SE: 7 congenital and 39 acquired. According to Cotton's classification 13 had grade I, 16 grade II, 12 grade III and none grade IV. Eleven of twelve cases treated conservatively did well (92%); one out of six patients managed endoscopically required further surgery (7%); good results were obtained in 5 of 7 cases treated by ACS (71 %); 8 out of 9 patients treated by ALTP did well (89%) and 7 out of 8 managed by APLTP had good results (87.5%). One iatrogenic suture dehiscence required further surgery. There is no statistical difference in the complication rate between patients treated conservatively and those treated by open surgery, while the mean hospital stay was higher in the latter (p < 0.05). An appropriate surgical technique should be offered to those patients with SE who do not do well with conservative management, since these techniques have yielded good results with a low rate of complications. Long-term follow-up shows the absence of recurrence.

Histopathology of congenital subglottic stenosis.

A precise delineation of each laryngeal abnormality is critical to planning rational therapy. Since congenital cricoid cartilage anomalies are easily overlooked, a systematic approach to their identification is as important as a clear concept of their classification. The purpose of this communication is to present the pathologic findings and classification of congenital cricoid cartilage deformities and to summarize updated information derived by the whole organ macrosection technique. The classification of congenital subglottic stenosis is based on study of the English-language literature and the collection in the Laryngeal Development Laboratory at The Children's Memorial Hospital in Chicago. Twenty-nine specimens with congenital cricoid abnormalities have been identified. Fourteen have an elliptical cricoid cartilage; 12, a laryngeal cleft; 5, a flattened shape: 1, a large anterior lamina; and 3, generalized thickening. Some specimens have more than 1 anomaly. Eight cases of fragmented or distorted cricoid cartilages are thought to be acquired lesions. An anterior submucous cricoid cleft has been identified and is presented for the first time.

Posterior cricoidotomy lumen augmentation for treatment of subglottic stenosis in children.

To determine the results of posterior cricoidotomy lumen augmentation in the treatment of moderate and severe subglottic stenosis in children, and to assess the effect of this surgery on the growth of the larynx in young children. A 17-year retrospective study. Thirteen children with moderate and severe subglottic stenosis who underwent posterior cricoidotomy lumen augmentation from 1980 to 1996. Eight children (62%) were male and 5 (38%) were female. The average age was 4.7 years (age range, 8 months to 15 years). Six (46%) of the 13 children were younger than 3 years. Nine children (69%) had acquired subglottic stenosis, 3 of whom had a combined subglottic-posterior glottic stenosis, and 4 (31%) had congenital subglottic stenosis. All the children already had a tracheotomy tube placed and had undergone treatment at our clinic or at other institutions. The surgical procedure we used included a cricoid lamina split with grafting (2 children with costal cartilage graft and 11 with buccal mucosal graft) and stenting. Twelve (92%) of the 13 children underwent decannulation, and 1 (8%) is still undergoing treatment: decannulation is expected to be carried out in the near future. Of the 12 children who underwent decannulation, 9 demonstrated good postoperative voice quality and 3, who had combined subglottic-posterior glottic stenosis, developed impaired voice function. Following stent removal, all children experienced some degree of difficulty in swallowing saliva and liquids, which was soon overcome. No evidence of surgery-induced laryngeal growth impairment in younger children was observed. The posterior cricoidotomy lumen augmentation is a safe and effective technique for the treatment of moderate and severe subglottic stenosis in children of any age. This study lends further support to the assertion that external surgery does not affect the growth of the larynx in younger children.

Anterior cricoid split. Use of hyoid as autologous grafting material.

To examine the use of hyoid as a readily available autologous grafting material for the anterior cricoid split (ACS) procedure. Prospective analysis of 20 patients undergoing ACS with hyoid interposition grafting for subglottic stenosis over a 3-year period. The patients received at least 1 year of follow-up after surgery. Tertiary care children's hospital. Twenty infants (age range, 2-9 months) with endoscopically confirmed acquired and congenital subglottic stenosis. Presenting symptoms included stridor, failure to extubate, and recurrent atypical croup. All 20 children underwent ACS with hyoid interposition grafting. All 20 patients exhibited improvement in their symptoms of airway obstruction. All 12 patients in whom extubation had previously failed subsequently underwent successful extubation. The 8 patients with symptoms of stridor and atypical croup showed marked improvement in their symptoms. Serial bronchoscopy revealed mucosal healing and incorporation of the hyoid grafts. Hyoid provides a readily available and reliable grafting material for interposition grafting in the ACS procedure for neonates and infants.

Malformation and Stenosis of the Cricoid Cartilage in Association With Larsen's Syndrome

Three patients with the typical features of Larsen's syndrome are described. All three developed severe respiratory symptoms caused by a congenital subglottic stenosis. Tracheotomy and treatment of the stenosis by means of laryngotracheoplasty resulted in complete collapse of the cricoid cartilage and the proximal tracheal skeleton. Lack of rigidity of the laryngeal and tracheal cartilages in patients with Larsen's syndrome could well be responsible for this failure. Surgical treatment consisted of resection of the stenotic and collapsed areas and end-to-end anastomosis. This therapy was eventually successful in all three patients.

Upper airway obstruction in children with Down syndrome.

To analyze the complex nature of upper airway obstruction (UAO) and the response to surgery in children with Down syndrome. Retrospective medical chart review of all patients with Down syndrome who had UAO during a 5-year period. Academic tertiary care children's hospital. Seventy-one pediatric patients with Down syndrome who had significant UAO. Thirty-four patients had pulmonary artery hypertension; 44 patients had multiple sites of airway obstruction. The obstructive problems included lymphoid hyperplasia, macroglossia, narrow nasopharynx, laryngomalacia, congenital subglottic stenosis, tracheobronchomalacia, and tracheal stenosis. Children with Down syndrome and UAO underwent surgical procedures including tonsillectomy, adenoidectomy, tonsillar pillar plication, uvulopalatopharyngoplasty, anterior tongue reduction, tonguehyoid suspension, laryngotracheoplasty, and tracheotomy. Postoperative symptoms found on medical chart review, or parental telephone survey, or both, and results of postoperative diagnostic tests. Patients were grouped as "improved" or having "significant residual symptoms." Twenty-seven of the 55 surgical patients had mild obstructive symptoms, and most improved after tonsil or adenoid surgery, or both. The remaining patients were younger and had more severe symptoms, multiple sites of obstruction, and a high incidence of cardiac disease. Eleven (39%) of the 28 patients in this group had significant residual symptoms after surgery. Four children are tracheotomy-dependent. Five deaths occurred; 3 were attributable to the upper airway. Upper airway obstruction in children with Down syndrome often is a complex process with multifocal causes. Residual symptoms of airway obstruction are common after surgery. A comprehensive and individualized approach is important in the management of UAO in Down syndrome.

Acute Management of Unsuspected Subglottic Stenosis by Tracheal Dilation

Acute Management of Unsuspected Subglottic Stenosis by Tracheal Dilation

Difficult tracheal extubation in a patient with an unsuspected congenital subglottic stenosis

A case is described of difficulty in advancing a tracheal tube and subsequent difficult extubation in an asymptomatic patient. The origin of this complication was an unsuspected subglottic stenosis, and a subsequent experiment showed how the relatively large folds of the cuff of an armoured tracheal tube could become caught on a narrow section of airway.

Experience with one-stage laryngotracheal reconstruction

Eight patients (ages 10 months to 5 years) underwent laryngotracheal reconstruction (LTR) without intraluminal stenting or tracheostomy. All patients had moderate to severe acquired or congenital subglottic stenosis. Six patients were tracheostomy-dependent prior to reconstruction. The other two children were intubated and would have required tracheostomy for airway control. LTR and decannulation/extubation were accomplished as a one-stage procedure. Autologous rib cartilage was used (anterior graft only — 6 patients, anterior and posterior graft — 3 patients) and patients were intubated for 7–10 days. Pulmonary complications from the week-long intubation and intensive care unit stay were common, but easily managed. Seven of eight patients were successfully decannulated/extubated.

Does early expansion surgery have a role in the management of congenital subglottic stenosis?

To better define the clinical course of congenital subglottic stenosis, the authors reviewed a cohort of 27 children managed with long-term tracheotomy and interval bronchoscopy. Rates of spontaneous resolution were 19% at 6 months, 39% at 12 months, and 69% at 18 months (Kaplan-Meier survival analysis). Children older than 4 months of age at tracheotomy tended to have more rapid resolution of their stenoses (P = .08). Overall, the median time to decannulation was 15.8 months (95% confidence interval, 13.9 to 17.6 months). One death was possibly tracheotomy-related, and 12 (44%) of the children experienced complications or sequelae resulting in new or extended hospitalization. Considering the prolonged cannulation necessary for spontaneous resolution of congenital subglottic stenosis, the role of long-term tracheotomy as the sole form of management may be limited. Once the need for intervention is apparent, the authors recommend early consideration of cricoid split or laryngotracheoplasty as alternatives to long-term tracheotomy.

Anterior cricoid split for subglottic stenosis: experience at the Children's Hospital of New Jersey.

Twenty-five children with acquired and congenital subglottic stenosis (SGS) were managed with the anterior cricoid split (ACS) operation at the authors' institution from September 1987 to January 1990. Ages ranged from 2.5 months to 5.5 years. Twenty-one (84%) of the children were extubated after 5 to 14 days of nasotracheal intubation and have remained stable after an average follow-up of 10 months. Atelectasis was a common postoperative problem, encountered in 12 (48%) of the patients. Other complications included a tracheocutaneous fistula, prolapse of soft tissue into the tracheal lumen via the cricoid incision, a subglottic granuloma, and 2 cases of prolonged lower extremity paresis following reversal of vecuronium. The results of this retrospective study indicate that the ACS is a valuable first-line procedure for the management of SGS in a variety of pediatric patients.