2022 Volume 68 Issue 3 Pages 179-181
The development of stridor and periodic desaturation in a 2-day-old neonate born at term lead to the suspicion of upper airway obstruction. The patient underwent flexible fiberoptic laryngo-tracheo-bronchoscopy and was diagnosed as having an elongated soft palate and secondary mild pharyngomalacia. Early intervention with high PEEP therapy using nasal CPAP led to improvement in the patient condition.
We have sometimes encountered a neonate with symptoms of upper airway obstruction such as stridor and desaturation. Although there is a wide variety of conditions that can cause upper airway obstruction, such as laryngomalacia and glossoptosis, there are no specific symptoms for each. We report a very rare case of a baby diagnosed with an elongated soft palate by flexible fiberoptic laryngo-tracheo-bronchoscopy. Early examination led to successful conservative treatment.
A male baby was born at 37 weeks and 4 days of gestation by vacuum extraction because of decreased fetal heart rate. He has no family history or perinatal complications. He was admitted to the neonatal intensive care unit due to tachypnea and grunting and was diagnosed as having transient tachypnea of the newborn. He was treated with nasal continuous positive airway pressure (n-CPAP) for 23 hours.
From 60 hours after birth, stridor and periodic desaturations occurred after crying. He was treated once by mask ventilation for a hypoxic episode where his SpO2 level dropped to 70%. Thereafter, he experienced periodic hypoxic episodes not only after crying but also while sleeping. On day 10, we found that his periodic desaturation seemed to be related to periodic breathing as indicated on electrocardiographic monitoring (Figure 1), but on auscultation, we found it to be obstructive apnea with absence of aeration for 5-10 seconds despite thoracic motion. This was followed by tachypnea. Based on these symptoms, the baby was considered to have an upper airway obstruction such as glossoptosis, and we expected that his airway could be kept patent by use of a shoulder roll. This treatment slightly reduced the range of decline in his SpO2 level but did not change the frequency of the desaturation events.
On day 15, he underwent flexible fiberoptic laryngo-tracheo-bronchoscopy (FFB) under sedation, which revealed that his middle pharynx was mildly stenotic, and the posterior soft palate and uvula touched against the base of tongue during inhalation (Figure 2a). As a result when he fell asleep, the pharynx was completely obstructed. The patient was subsequently diagnosed as having an elongated soft palate and secondary mild pharyngomalacia. No other abnormality was observed in the examination. A n-CPAP (Positive end-expiratory pressure; PEEP:6 cmH2O) was then instituted as high PEEP therapy, and this led to the gradual resolution of his symptoms.
N-CPAP was continued for about one month. On day 43, he underwent a second FFB under sedation as before, which showed no obstructive findings in the absence of pressure support (Figure 2c). He had no hypoxic episodes while free of respiratory support for the next 2 weeks, and he was discharged on day 60.
Elongated soft palate is a common feature of brachycephalic airway syndrome in “flat-faced” dog and cat breeds1). In animals, an elongated soft palate is defined as a closed soft palate extending beyond the tip of the epiglottis toward the airway, but no clear definition exists in humans. Upper airway obstruction according to an elongated soft palate is presumably caused by anatomical obstruction with an abnormally long soft palate and functional obstruction due to immature pharyngeal muscles in the neonate. In addition, there have only been two cases of elongated soft palate diagnosed in neonates or infants with symptoms of upper airway obstruction2). Although all such cases had chromosomal or genetic abnormalities, the present baby had no obvious malformation, and there were no other findings that would raise suspicion of genetic abnormalities. Patients with functional abnormalities of the pharynx or larynx may have feeding difficulties as well as stridor and hypoxemia3), but the present baby had no feeding problems and was gaining weight well. Furthermore, including those of “elongated uvula”, most past cases were reported to have been treated surgically (e.g., with uvulopalatopharyngoplasty)4,5).
Uvulopalatopharyngoplasty is a surgical procedure to excise the uvula and soft palate and to resect a substantial amount of tissue from the lateral pharyngeal walls6). Fortunately, our patient improved with high PEEP therapy as a conservative treatment, which is a commonly used treatment for upper airway obstructive diseases3,7).
As in this case, if there are no specific symptoms of upper airway obstruction, it can be difficult to make a definitive diagnosis based on the clinical course alone. Therefore, FFB should be performed as soon as possible so that treatment can be undertaken within the first few months of life.
We experienced a very rare case of a baby diagnosed with an elongated soft palate. It is important to perform FFB for the accurate assessment and identification of clinical conditions affecting the soft palate to facilitate early intervention.
None.
The authors confirm that consent for submission and publication of this case report including images and associated text has been obtained from the patient’s guardians.
In addition, approval was obtained from the hospital’s Ethics Committee.
None declared.
We are grateful to Rise Japan LLC corporation for carefully proofreading the manuscript.
K.I. wrote the manuscript. H.H. performed laryngotracheal endoscopy. T.N. and D.H. provided conceptual advice. All authors read and approved the final manuscript.
