Airway management of palatoglossal bands - A challenge to an anaesthesiologist.

Palatoglossal bands are one of the very rare congenital anomaly with very few documented cases worldwide. They can present with respiratory distress which requires immediate surgical intervention, or with feeding difficulties. The management of such a patient is a challenge to any anaesthesiologist because of inability to perform conventional laryngoscopy and associated cardiac or digital anomalies. We discuss here the management of such an infant who presented at 18 months with feeding difficulties. Copyright:

Palatoglossal bands are rare congenital anomalies with very few reported cases.[1] They present with either respiratory distress at birth or feeding difficulties. They are usually associated with a variety of congenital defects such as cleft lip and palate, microglossia, micrognathia and temporomandibular disorders.[2]

An 18-month-old child presented in the paediatric outpatient department with chief complaints of difficulty in feeding. She had recurrent episodes of vomiting after feeds. On oral examination, tongue was found to be adhered to soft palate with a small central defect of around 5 mm. A diagnosis of palatoglossal bands was made. The cause of these bands is still unknown and presumed to be genetic, teratogenic, or mechanical insult in early gestation.[3] An experimental study on the aetiology suggested persistence of buccopharyngeal membrane, presence of amniotic constriction bands in the region of developing first branchial arch, environmental insults and drugs such as meclizine and large doses of vitamin A as possible causes; however, they failed to prove any association for the same.[4] These defects are commonly associated with many congenital syndromes such as Moebius' syndrome, hypoglossia–hypodactylia syndrome, Hanhart's syndrome, glossopalatine ankylosis syndrome, splenogonadal fusion syndrome, Charlie M syndrome, Van der Woud's syndrome, orofaciodigital syndrome, cleft palate alveolar synechiae, oromandibular limb hypogenesis syndrome and cleft palate medial synechiae syndrome.[56] Our patient was a case of nonsyndromic palatoglossal bands as she did not have any other associated congenital anomaly.

Congenital palatoglossal bands may consist of epithelium and varying amounts of connective tissue. If the bands involve only soft tissues, the condition is termed as synechiae, and if bone is also involved, the term syngnathia is used.[7] The affected children usually present with respiratory distress at birth or difficulty in feeding and failure to gain weight later. If the patient presents with airway problems at birth, the surgical intervention has to be done as soon as possible.[1]

The definitive surgery includes excision of palatoglossal bands under anaesthesia.[8] Anaesthetic management of such a case is a challenge and includes meticulous preoperative evaluation to rule out any associated congenital conditions and thorough evaluation of airway. A thorough preanaesthetic evaluation revealed normal facial features, low weight, delayed motor milestones, active cry and no cyanosis. Oral examination revealed presence of intraoral palatoglossal bands extending from the soft palate to the tongue with a central perforation [Figure 1]. Our patient did not have any other associated congenital condition. On the day of surgery after giving premedication, anaesthesia was maintained on air/oxygen/sevoflurane with spontaneous respiration. Adequate analgesia was provided with fentanyl and paracetamol. The ideal airway management in the presence of palatoglossal bands is through nasotracheal fibreoptic intubation.[9] We did not have a paediatric fibreoptic bronchoscope available with us. After thorough consultation with the surgeons, we utilised flexible fibreoptic nasopharyngoscope to visualise the oropharynx and glottis through one nare of the infant under spontaneous ventilation. Through the other nare, an experienced anaesthesiologist inserted an uncuffed nasotracheal tube which could be visualised on the nasopharyngoscope monitor. Through external laryngeal manipulation and neck extension, we attempted that the endotracheal tube could be guided through the vocal cords. When intubation was confirmed visually through the nasopharyngoscope and by capnography, we administered muscle relaxants. Elective tracheostomy was kept on standby at all times. This was a novel approach such that patients' safety was not compromised as the patient was on spontaneous ventilation with adequate depth of anaesthesia. Second, we could guide our nasotracheal tube under vision into the larynx, thus avoiding any trauma and bleeding. After return of spontaneous respiration, the patient was successfully extubated after the surgery. During the follow-up period, the baby thrived well with adequate intake of milk.

Figure 1

Palatoglossal bands extending from the tongue to the palate with a small central perforation of around 5 mm

To conclude, the use of a flexible fibreoptic nasopharyngoscope-guided intubation under spontaneous ventilation is a novel option for securing airway in such cases. A meticulous preoperative evaluation, thorough communication with the surgical teams and having a safe anaesthetic plan go a long way in improving outcomes in such patients.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.