Coblation of paediatric cystic laryngeal lymphovascular malformations: a safe and effective alternative to tracheostomy
- 1 Anatomy, Division of Health Sciences, University of Edinburgh, Edinburgh, UK
- 2 Department of Paediatric Otolaryngology, NHS Lothian, Edinburgh, UK
- Correspondence to Christopher St Clair Gaston Thompson; christhompson3@nhs.net
Abstract
We describe the case of a 12-hour-old, full-term newborn girl referred to the Ear, Nose and Throat emergency team with increased work of breathing and stridor present at birth. Flexible nasendoscopy revealed a cystic laryngeal lesion obstructing the glottis that prompted securing of the airway with intubation and transfer to a tertiary paediatric centre. On further investigation with MRI and direct visualisation, the lesion was identified as a mixed macro/microcystic laryngeal lymphovascular malformation. The patient successfully underwent a series of microlaryngo–bronchoscopy and coblations of the laryngeal lesion with the aim of avoiding a tracheostomy. We describe the presentation, diagnosis and management of this rare condition in a paediatric case, along with a literature review of the subject.
Background
Paediatric congenital laryngeal cysts are extremely rare, and their management when presenting with significant respiratory compromise very early in life is not well described.
The established gold standard is to first secure the airway with either endotracheal intubation or tracheostomy followed by surgical excision using either an endoscopic or open approach. However, recent advances such as the introduction of radiofrequency ablation, or coblation, may potentially offer superior outcomes.
Case presentation
A 12-hour-old, full-term newborn girl was referred to the ENT emergency team at a district general hospital with increased work of breathing and stridor. This had been present since birth, requiring continuous positive airway pressure and monitoring on the special care baby unit. She had been delivered via emergency caesarean section for fetal bradycardia. No prenatal concerns for upper airway concern were identified by obstetric ultrasound prior to birth.
Investigations
Initial examination demonstrated accessory respiratory muscle use and stridor. Flexible nasendoscopy (FNE) revealed a large cystic mass obscuring the glottis, extending to the level of the supraglottis on the right side. After further review by a consultant paediatric Ear, Nose and Throat surgeon and discussion with the paediatric and anaesthetic teams, emergency intubation was performed with a Hopkins rod fibreoptic scope loaded with a size 3.5 endotracheal tube in order to secure the airway prior to transfer to a tertiary paediatric centre.
Subsequent general anaesthetic MRI demonstrated a right-sided mixed macro/microcystic transglottic laryngeal lesion (figure 1).
Pre-operative general anaesthetic coronal (A) and axial (B) MRI images, demonstrating a right-sided laryngeal, mixed macro/microcystic lesion.
Differential diagnosis
Initial differential diagnoses included a laryngeal cyst (simple mucus retention or more complex lymphovascular malformation), laryngeal tumour (benign or malignant), laryngeal web, laryngocele, laryngomalacia and subglottic stenosis.
Following both the MRI and endoscopic findings, a type 1 mixed micro/macrocystic lymphovascular malformation was considered more likely due to its complex relationship with the larynx.
Treatment
The patient was initially resuscitated with fluids and started on antibiotics and dexamethasone. Following multi-disciplinary team discussion, she was taken to theatre for microlaryngoscopy and bronchoscopy (MLB) and coblation of the laryngeal lesion with the aim of avoiding a tracheostomy, at around 48 hours after initial presentation (figure 2). Direct visualisation demonstrated the presence of a cystic mass causing obstruction of the glottis, consistent with a type 1 laryngeal cystic lymphovascular malformation.
Endoscopic images from microlaryngoscopy and bronchoscopy of the laryngeal lesion with endotracheal tube pre-coblation (A) and extubated post-coblation (B).
She was extubated successfully after 2 days and maintained on Optiflow with a nasogastric tube in situ for feeding and medications. However, she subsequently deteriorated over the following 3 days, with an increased work of breathing and stridor. Re-examination with FNE revealed a reaccumulation of the cyst, prompting repeat MLB and coblation.
She went on to have two further MLB+coblations at 2 and 3 weeks following initial presentation, each with good effect in draining/marsupialising the lesion (figure 3). Subsequent MLB 2 weeks later revealed no evidence of reaccumulation of the lesion and she was successfully extubated. After a period of observation, she remained well and was discharged home with an NG tube in place to continue medications and feeding. A gastrostomy tube was not considered due to the anticipated short-term nature of feeding supplementation.
An endoscopic image following repeat microlaryngoscopy and bronchoscopy and coblation demonstrating the true vocal cords and cyst cavity.
Outcome and follow-up
A repeat MLB and a further MRI at 9 weeks postpresentation showed no reaccumulation of the lesion (figures 4 and 5). The patient was followed up several times over the following months and was found to be thriving, enabling removal of the NG tube. She had no reported problems with feeding, was gaining weight appropriately and had a strong cry.
An endoscopic image from the repeated microlaryngoscopy and bronchoscopy at 9 weeks showing resolution of the cyst and only minor right-sided scar tissue formation.
Repeated coronal (A) and axial (B) general anaesthetic MRI images at 9 weeks further demonstrating resolution of the lesion.
Discussion
Congenital cysts of the larynx are an unusual cause of airway obstruction in newborns with an estimated incidence of 1.82/100 000 live births.1 They are thought most commonly to arise from either obstruction of the laryngeal epithelial mucous glands or atresia of the laryngeal ventricle orifice itself, leading to mucus retention. Alternatively, they are thought to be more complex,2 such as congenital lymphovascular malformations of either or both a macro/micro cystic nature. They can be classified into cysts confined to the larynx (type I) or those with extralaryngeal extension (type II).2
The severity of presenting symptoms and timing of presentation of laryngeal cysts are related to their location and size. Larger cysts, as in this case, may present with respiratory distress and airway obstruction immediately after birth, whereas smaller cysts may produce more subtle problems with mild stridor, a weak muffled cry and feeding difficulties. Flexible fibreoptic endoscopy enables dynamic assessment of the larynx in a bedside setting, however direct laryngoscopy under general anaesthesia is the gold standard for confirming diagnosis. Antenatal diagnosis with ultrasonography and fetal MRI has also been reported in the literature, optimising immediate postnatal management.3
There is a lack of clear consensus as to the most appropriate surgical method to treat congenital laryngeal cysts due to their rarity. Securing the airway is the priority if it is compromised, and this is usually achieved with either endotracheal intubation, or in some cases, a tracheostomy. Removal of laryngeal cysts can be achieved either endoscopically or via an external approach. Endoscopic excision with complete cyst wall removal with microinstruments or CO2 laser is the mainstay of treatment for type I cysts, whereas type II cysts or those which recur following endoscopic removal require an external surgical approach.1 4 5 Needle aspiration may also be used to decompress the cysts and relieve the airway obstruction, however, this is associated with a high rate of recurrence, as the mucous glands in the epithelial lining of the cysts will continue to secrete mucus.6 In one series of 17 infants and children with saccular cysts, 11 (65%) underwent a tracheostomy due to concerns about laryngeal oedema and cyst recurrence following initial treatment and after multiple rapid recurrences.7 Conversely, another review of 20 patients found only a 15% recurrence rate across a variety of management options, with no tracheostomies required and scarring seen as a longer-term complication.8
In this case, although considered as part of her ongoing management, our patient did not undergo tracheostomy due to the significant improvements made following each coblation, the well-documented risks and morbidity of the procedure and parental wishes.
Low-temperature radiofrequency ablation (commonly known as coblation) has emerged as a novel technique in paediatric ENT surgery. It involves passing a radiofrequency bipolar electrical current through a medium of normal saline to produce a plasma field of sodium ions that disrupt intercellular bonds, leading to vaporisation and coagulation. Radiofrequency ablation heats tissue to only 60–65 degrees, resulting in less bleeding and damage to surrounding structures and improved visualisation of the operative field compared with conventional techniques.9 A retrospective study by Wang et al found that low-temperature radiofrequency ablation was associated with a shortened operation time, decreased blood loss and lower 1-year recurrence rate in their study population of 84 children diagnosed with pharyngolaryngeal cysts.10 Its use in the management of laryngeal cysts was supported in a case series of six patients, which found the removal of recurrent saccular cysts could be achieved safely and effectively with improved surgical precision, minimal bleeding and better healing.11 Additionally, coblation has been used successfully in several other cases of neonatal laryngeal cysts.12 13
Indeed, it can be suggested that in this case, coblation of a more complex laryngeal cyst proved to be safe and effective in both treating the underlying pathology and avoiding the morbidity of a tracheostomy. Additionally, no complications were encountered throughout her management, with only the need for several general anaesthetics as part of a balanced risk-benefit analysis. We feel that this is one of the first cases presenting as an emergency managed in this way, with an excellent outcome.
Learning points
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Congenital laryngeal cysts remain rare but should remain differential diagnoses when assessing neonates or infants presenting with stridor.
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Numerous surgical modalities have been used to treat laryngeal cysts, often requiring frequent, repeated treatments.
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Low-temperature radiofrequency ablation should be considered as a safe and effective alternative to tracheostomy and conventional therapy in the treatment of congenital laryngeal lymphovascular malformations.
Footnotes
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Contributors AS, CSGT: Clinical, Case identification. LQL: Literature search. AS, CSGT, LQL: Manuscript writing, Manuscript checking.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Competing interests None declared.
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Patient consent for publication Parental/guardian consent obtained.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.
References
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