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Cricotracheal resection as treatment for laryngeal stenosis cotton grade IV

Short summary

The patient is a former premature baby who underwent tracheostomy after 30 days of intubation and laryngotracheoplasty with anterior and posterior costal grafts and implantation of a T-tube. She is now 10 years-old and was diagnosed with Cotton’s grade 4 subglottic stenosis. The restricted airways (laryngeal stenosis) cause severe breathing and communication difficulties.

Patient's questions

1) Can you indicate centers of excellence and names of specialist surgeons for treatment of laryngeal and tracheal stenosis in the USA and Europe?
2) Do you suggest any other additional therapy?
3) What is the prognosis?

Medical opinion
Patient's History
Case history:
A 10 year old girl born prematurely at week 28 by gestosis, weighed 750 g at birth. Due to the small size of her body, there were problems performing required intubation in the intensive care unit. At age of 30 days a tracheotomy was performed.
The patient also presented ocular malformations. Medical diagnosis was bilateral chorioretinal coloboma with significant visual deficit in future.
During subsequent months, the patient’s clinical situation stabilized and she was able to breathe on her own by means of a fitted prosthesis (cannula) , and to feed using a bottle. She was reactive to external stimuli, very lively and constantly moving, and displayed a strong vitality. After spending her first 5 months of life in hospital and having attained a weight of 2 Kg, the patient was discharged and sent home with her parents, who dealt with considerable difficulties at first, including managing the prosthesis (cannula) and feeding. Patient’s growth rate was very slow, despite attention by her parents and care of specialists who followed her up. At 10 months she started attending a Centre for visual rehabilitation. She continued attending this center for 4 years, during which she was followed up for her psychomotor and speech skills. She managed to achieve results that in certain cases were excellent.
The patient started walking at age 2 and a half years, and to express the desire to communicate, and showed behavioral features characterized by aggression that was initially directed towards herself and then towards adults (parents, educators, staff), but rarely towards other children. The main cause of this behavior was identified as inability to communicate verbally or produce sounds. In an attempt to solve this problem, she was taught sign language in cooperation with speech therapy, for 2 years and she attended kindergarten and primary school. The results have been good. The patient is now 10 years of age, weighs 17 Kg and her height is 116 cm. She is in her 2nd year of primary school and shows an increasing interest in learning more sign language to communicate. However, the amount of sign language that the patient learned allows only very basic communication, not enough for her needs, and her aggression is still present and even becoming more intense. She adequately completed a personalized school curriculum. She is able to recognize and write all the letters of the alphabet in dictation, she can name images of objects and acquired the concept of quantity. Despite her visual deficit, she is able to move independently both in familiar and new environments.
Her parents hope that the patient would be able to have her prosthesis (cannula) removed, allowing her to breathe through her nose and mouth and communicate verbally. Surgical intervention had not been possible initially due to the high risk associated with the girl being under-weight and very small in size. The patient’s parents consulted a professor at a clinical facility who diagnosed the patient as non-cooperative and this would not provide a good outcome to surgery.
The patient had a laryngotracheoscopy where the following diagnosis was given: Laryngeal Stenosis in a tracheotomy patient with nasotracheal intubation-> LARYNGEAL STENOSIS COTTON GRADE IV. Surgery was performed and the results were: Laryngoplasty with autologous graft of costal cartilage (X right), and implantation of a T prosthesis (Kehr No. 8). Monitoring the laryngotracheoplasty dilation for laryngotracheal stenosis in the tracheotomy patient was satisfactory, with therapy consisting of progressive dilation to 24 Ch. Subsequently, complications arose and endoscopy showed decubitus and granulations on laryngeal surface of epiglottis from contact with the superior edge of the T prosthesis (Kehr No.8), undamaged trachea beyond the inferior edge of the prosthesis. Severe laryngeal stenosis was found. This was a return to the same situation that existed before surgery.
In my opinion, the patient will need an endoscopy under general anesthesia to evaluate the exact location (glottis, subglottis, and trachea) and degree of the airway stenosis. If she has further severe subglottic stenosis (grade 3 or grade 4), the best surgical option would be a cricotracheal resection (CTR) with primary anastomosis. However, stenosis involving or close to the vocal cords is preferably treated with laryngotracheal reconstruction with cartilage grafts. The success rate of the procedure in centers that specialized in airway surgery is about 85%. Since the patient has already undergone previous surgery to the larynx, the success rate may be somewhat lower.
The best center in the USA for treatment of laryngeal stenosis is the Department of Pediatric Otolaryngology at the Children's Hospital Medical Centre, under direction of Prof. Robin Cotton. A second option with surgical experience of CTR is the Department of Otolaryngology in Lausanne, Switzerland, under direction of Philippe Monnier.