Russian Journal of Woman and Child Health
ISSN 2618-8430 (Print), 2686-7184 (Online)

Congenital tracheal stenosis: case report

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DOI: 10.32364/2618-8430-2021-4-3-298-301

E.V. Gol’tsman1, O.A. Kuchma1, E.N. Bondarenko1, D.A. Maiseenko2

1I.S. Berzon Krasnoyarsk Interregional Clinical Hospital, Krasnoyarsk, Russian Federation

2Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University, Krasnoyarsk,
Russian Federation

The rate of congenital malformations of the larynx is challenging to assess. This is probably accounted for by the fact that these diseases occur more commonly than they are diagnosed. This paper describes a case report of congenital laryngeal stenosis, which was a diagnosis of exclusion since this congenital malformation is very rare in neonatal and pediatric practice. After a preliminary diagnosis in the maternity hospital, a newborn was transferred to Krasnoyarsk’s large pediatric hospital for examinations and treatment. Clinical diagnosis was verified by laryngotracheoscopy and CT scan of the larynx and trachea. The newborn’s condition was severe for 28 days. He stayed in the intensive care unit and was on a ventilator and oxygen. Tracheal intubation followed by extubation (performed three times) has no effect due to the increase in respiratory failure. Newborn’s condition improved only after palliative tracheostomy. The child was discharged in satisfactory condition with recommendations on tracheostoma care. Management decision on the surgical technique to address this malformation will be taken later.

Keywords: primary congenital tracheal stenosis, children, clinical presentations, diagnosis.

 For citation: Gol’tsman E.V., Kuchma O.A., Bondarenko E.N., Maiseenko D.A. Congenital tracheal stenosis: case report. Russian Journal of Woman and Child Health. 2021;4(3):298–301 (in Russ.). DOI: 10.32364/2618-8430-2021-4-3-298-301.


Congenital tracheal stenosis (CTS) is a rare congenital malformation that occurs in 1 per 64,500 newborns, being more common in boys than in girls. Complex congenital malformations (50% are cardiovascular malformations) are diagnosed in 80% [1–3]. The most severe one is congenital laryngeal stenosis (CLS) due to the rapid development of asphyxia as a result of tracheal anatomy in newborns, i.e., narrower tracheal lumen compared to adults. Without treatment, the rate of unfavorable outcomes is 80% [3, 4].

The etiology of malformation is unknown. However, CTS being organic stenosis is characterized by the malformation of cartilage framework, i.e., closed cartilage rings without membranous parts are generated. As a result, tracheal diameter is reduced, being not in the norm for the age group. Deformation, tracheomalacia, coalescence of several cartilages, increase of cartilage rings up to 30, and tracheal elongation are typical [3, 5, 6]. 

Onset occurs at birth and manifests as dyspnea and noisy breathing (stridor). In controlled disease, stridor is evident in intercurrent viral or bacterial infection.

Computed tomography (CT) is the gold standard for the diagnosis of CTS. CT is useful to assess the level, length, and extent of narrowing, tracheal wall thickness, and the presence of peri-process [7]. Chest X-ray and bronchography are additional diagnostic tools. The importance of laryngotracheobronchoscopy (LTB) to specify disease patterns cannot be overemphasized. In addition, LTB allows surgical interventions (in the lack of pyogenic inflammation) [8].

Respiratory relevant variants of CTS require surgical procedures to reestablish airway lumen since conservative treatment is ineffective (maintenance therapy is only possible). The endoscopic technique can be applied in non-extensive narrowing and membrane, stenosis stage 1 or 2. Among minimally invasive procedures, bougienage with or without prolonged intubation, stent, balloon dilation, laser vaporization, etc., are used. According to some authors, the efficacy of endoscopic surgery is as high as 100% [3, 9]. This technique is also helpful to expand anastomotic area after tracheoplasty.

Circular resection is considered optimal management for CTS. However, absolute contraindications to circular resection are the length of stenosis more than 7 or 8 rings (broad mobilization), a short distance from the vocal cords, multi-level stenosis, bilateral palsy of the recurrent laryngeal nerve, large tracheoesophageal fistula, an impaired dividing function of the larynx [3, 8].

This malformation is rare but associated with a high risk of death. Therefore, neonatologists should be highly suspicious to select excellent and timely management strategies to prevent unfavorable outcomes. We report a case of CTS in a newborn that clearly illustrates that abundant routine work masks relatively rare but hazardous diseases.

Case report

A 30-year-old woman (40 weeks of pregnancy) was admitted to the maternal hospital.

The woman attended an antenatal clinic from 9 weeks of pregnancy. The current pregnancy is the 5th one (first pregnancy in 2014, delivery at 39-40 weeks, a 3,250-g weighed girl was delivered; three medical abortions, no complications). At 16 weeks, threatened miscarriage was diagnosed. The woman received therapy to continue the pregnancy in the day hospital. In the 2nd trimester of pregnancy, the woman received a bivalent iron supplement for low Hb levels. At 20 and 33 weeks of pregnancy, hemodynamic abnormalities stage 1A were identified by sonography. In the 3rd trimester of pregnancy, the woman received anti-inflammatory therapy for vaginitis with clotrimazole (vaginal suppositories).

The duration of the 1st period (second delivery, anterior position) was 4 hours 10 minutes and the duration of the 2nd period was 10 minutes, and the duration of the anhydrous period was 10 min. The anterior amniotic fluid was bright, and the posterior amniotic fluid was greenish. Amniotic band syndrome was diagnosed. Apgar score was 8/8, a newborn cried immediately after birth. At birth, the height was 56 cm, weight 3,500 g, head circumference 36 cm, and chest circumference 34 cm. The newborn was laid out on the mother's abdomen. Stridor, generalized wheezing (by auscultation), and the indrawing of the epigastrium were identified. Saturation was 76%. To prevent amniotic fluid aspiration, the doctors decided to intubate the newborn. On the 3rd minute, technically challenging intubation was performed, i.e., a 3.5-mm endotracheal tube was passed through the vocal cords but no further. A 3.0-mm endotracheal tube was passed through the vocal cords into the trachea at a depth of 9 cm with technical challenges. The tracheal tube was then moved to the angle of the mouth, tracheal sanitation was performed (no discharge). Bag-valve-mask ventilation was started. Generalized wheezing and indrawing of the epigastrium persisted. Saturation was 92%. The doctors suggested that the severity of a newborn's condition is accounted for by congenital airway malformation, tracheal stenosis.

Over 3 hours after birth, the management strategy was staying in the incubator, monitoring, ventilation, and parenteral feeding. Finally, considering the poor condition of the newborn, he was referred to the ICU of a large children's clinical hospital in Krasnoyarsk.

During a stay in the ICU, a complex examination was performed.

Tracheobronchoscopy: congenital concentric stenosis of the lower third of the trachea, tracheitis.

CT scan: the endotracheal tube is at the level of the middle third of the trachea. It is not possible to assess the severity of stenosis at this level. The lower third of the trachea and large bronchi are patent.

Neurosonography: echo signs of bilateral intraventricular hemorrhages, mild dilation of the posterior part of the left horn, mild periventricular edema.

Echocardiography: open oval window 3.0 mm, dump in the right atrium. Borderline enlargement of the right heart. No thickening of the left ventricular wall, no abnormalities of cardiac valves, no hemodynamic abnormalities. The normal systolic function of the left ventricle. Pulmonary artery systolic pressure is within normal ranges.

Abdominal ultrasound: no abnormalities.

Over a 10-day stay in the ICU, the newborn's condition was very severe due to respiratory failure resulting from CTS and hypoxic hemorrhagic cerebral impairment. Weight gain (+240 g). On day 11, extubation was attempted. However, the condition worsened, i.e., indrawing of the intercostal spaces, tachypnea up to 60/min, tachycardia up to 180 bpm, and desaturation (85%) were identified. Re-intubation using a 2.5-mm endotracheal tube at a depth of 10 cm was performed. Breath was conducted symmetrically and uniformly over all lung fields, single wheezing (by auscultation), mucous sputum. No neurological, musculoskeletal, gastrointestinal, or urinary dysfunctions were revealed. The newborn stayed in the incubator and received partially parenteral feeding (with switching to enteral feeding). Antibacterial and antifungal treatments were prescribed. Parameters of ventilation (SiMV (PC) + PS) were as follows: PIP 17-16 mm wg, PS+ 14 cm wg, PEEP 4 cm wg, FiO2 0.21, f 40–30–20 min, tin 0.4 sec. On day 13, re-extubation was attempted. Spontaneous breathing was sustained for three days. A 2.5-mm endotracheal tube was exchanged for a 4.0-mm endotracheal tube. However, on day 15, respiratory failure increased, i.e., indrawing of the intercostal spaces and sternum, tachypnea, and desaturation (85%) were identified. Re-intubation using a 4.5-mm endotracheal tube at a depth of 10 cm was performed (ventilation SiMV (PC) + PS). On day 20, re-extubation was attempted. However, the newborn did not sustain spontaneous breathing for more than 3 hours, respiratory failure increased (tachypnea, indrawing of the xiphoid process and intercostal spaces were seen). Orotracheal intubation using a 4.0-mm endotracheal tube at a depth of 10 cm was performed.

Over 28 days, the newborn was on a ventilator and oxygen until referral to the Department of Newborn and Premature Infant Disorders. On day 29, a palliative tracheostomy was performed by life-saving indications.

On day 32, the baby was transferred to the Department of Newborn and Premature Infant Disorders for further care and monitoring. Over a 10-day follow-up in the intensive care ward, the condition was stable and satisfactory. The infant maintained proper suction and gained weight. The skin was pinky and clear, with normal skin turgor. Puerile respiration without wheezing (respiratory rate 140 per min) was identified by auscultation. The abdomen was soft (neither abdominal distension nor rigidity) and could be entirely deeply palpated. The umbilical wound healed and was clear, with no signs of umbilical cord infection. The liver was 10 cm below the costal margin. The spleen was not palpated. Normal urination and stool. No pathological neurological signs were revealed.

On day 41, the infant was discharged in satisfactory condition with recommendations on daily tracheostomy care using special aspiration probes, regular examinations by a pediatrician, ENT, neurologist, and (after one month) neurosonography. Management decision on the surgical technique to address this malformation will be taken after 3 to 6 months.


Two potential conditions that resulted in the development and progression of poorly controlled respiratory failure immediately after birth were considered, i.e., amniotic fluid aspiration and a congenital malformation of the airways. This situation required a prompt and correct differential diagnosis. The preliminary diagnosis was congenital stenosis since intubation was technically challenging (an obstacle that prevented endotracheal tube insertion). The lack of timely diagnosis and treatment is potentially fatal. N.N. Maleshko et al. [3] describe a child with undiagnosed at birth congenital tracheal malformation (stenosis). The child was admitted to the hospital and referenced to instrumental tests (CT, LTB) only after eight months when this malformation manifested itself with clinical signs (decompensation). Two-step urgent surgical intervention was performed. Step 1 was a median sternotomy and putting on the heart-lung bypass machine, and step 2 was a tracheal resection and anastomosis creation without ventilation. Diagnostically challenging diseases and conditions at birth are now common in neonatal and pediatric practices. In our case report, correct differential diagnosis accounted for a favorable outcome.


Even though CTS is a rare condition, early and correct differential diagnosis in the maternity hospital and subsequent referral to the multidisciplinary children's hospital with broad diagnostic potentialities (CT, LTB) allowed to determine surgical strategy and prevent the unfavorable perinatal outcome.

About the authors:

Elena V. Gol’tsman — neonatologist, I.S. Berzon Krasnoyarsk Interregional Clinical Hospital; 12, Instrumentalnaya str., Krasnoyarsk, 660123, Russian Federation.

Olga V. Kuchma — I.S. Berzon Krasnoyarsk Interregional Clinical Hospital; 12, Instrumentalnaya str., Krasnoyarsk, 660123, Russian Federation.

Ekaterina N. Bondarenko — neonatologist, I.S. Berzon Krasnoyarsk Interregional Clinical Hospital; 12, Instrumentalnaya str., Krasnoyarsk, 660123, Russian Federation.

Dmitriy A. Maiseenko — C. Sc. (Med.), associate professor of the Department of Obstetrics & Gynecology of the Institute of Professional Education, Prof. V.F. Voino-Yasenetsky Krasnoyarsk State Medical University; 1, Partizan Zheleznyak str., Krasnoyarsk, 660022, Russian Federation; ORCID iD 0000-0003-1569-5098.

Contact information: Dmitriy A. Maiseenko, e-mail:

Financial Disclosure: no authors have a financial or property interest in any material or method mentioned.

There is no conflict of interests.

Received 28.03.2021.

Revised 20.04.2021.

Accepted 17.05.2021.

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