Giant Cystic Hygroma in a Neonate: Fixing the Trajectory – Manual Maneuver for Airway Rescue

INTRODUCTION

Cystic hygroma (CH), a congenital lymphatic malformation in the neck can cause significant airway compromise in neonates due to its proximity to vital airway structures and its potential for rapid enlargement.^[1] Management options include sclerotherapy or surgical excision, depending on the size of the mass and extent of airway involvement. Airway involvement can be in the form of lateral deviation of the trachea, with or without compression, anteroposterior compression of the trachea leading to airway narrowing, respiratory difficulty and hypoxemia, depending on the size and location of the cystic mass. Early surgery is preferred in large lesions causing airway compression and hypoxemia.^[2] The distorted anatomy makes conventional airway strategies unpredictable and technically challenging. In such cases, conventional direct laryngoscopy and intubation may not always be successful, and alternative methods must be kept ready.

Fiberoptic intubation, while considered a gold standard for managing distorted upper airways, requires a fully equipped tertiary-care setup with a neonatal-appropriate fiberoptic bronchoscope and experienced personnel. Moreover, in neonates, fiberoptic intubation is technically demanding and is rarely feasible as an emergency procedure.^[3] Definite airway, surgical tracheostomy under local anesthesia in neonates is hazardous due to the small size of airway structures, limited working space, poorly defined landmarks, and the difficulty of maintaining spontaneous ventilation in a struggling baby,^[4] and is the last option in neonatal airway management.

Video laryngoscopy (VL) has emerged as a more commonly employed modality.^[5] Although it improves glottic visualization, ease of intubation is determined largely by airway axis alignment rather than the laryngoscope device itself.^[6] Strategies for maintaining spontaneous ventilation are therefore essential.

This case emphasizes a less-discussed limitation of VL which is the failure of endotracheal tube (ETT) advancement despite good visualization in a neonate and presents a pragmatic solution. This limitation has been described, where improved glottic exposure does not always translate into easier and successful intubation in anatomically distorted airways.^[3,6] We report a neonate with a giant cervical CH in whom airway control was achieved by manual retraction of the mass, a maneuver that temporarily realigned the tracheal trajectory and enabled successful tracheal intubation. The technique needs gentle handling under continuous monitoring to minimize the risk of trauma to adjacent neurovascular structures, a potential hazard when manipulating large cervical masses.^[7]

CASE REPORT

A 20-day-old term male neonate, weighing 3.1 kg was born by normal vaginal delivery at our institute. Apgar scores were 8 and 9 at 1 and 5 min, respectively. The baby had a respiratory rate of 50–60 breaths/min, heart rate 140–150 beats/min, and oxygen saturation (SpO₂) of 85–88% on room air, which improved to >92% with oxygen at 2 L/min.

At birth, a soft, 5 × 6 × 5 cm swelling was observed on the anterior and lateral aspects of the neck. There was no antenatal diagnosis of cervical mass. The baby was admitted in neonatal intensive care unit (NICU) for observation and further radiological evaluation. The neonate did not require ventilatory support or invasive airway intervention. There were no episodes of apnea and cyanosis. A trial of the right and left lateral positioning was performed in the NICU to assess for positional relief of airway compression. There was no appreciable improvement in SpO₂ or reduction in oxygen requirement in either position. Due to the noted gradual increase in size of the swelling and oxygen requirement, magnetic resonance imaging (MRI) of the neck was planned and performed on day 14 of life to delineate the extent of the lesion, under graded ketamine sedation (0.25 mg/kg), supplemental nasal oxygen, and standard monitoring. No airway instrumentation was required during the procedure. MRI revealed a lobulated, multiseptated cystic mass measuring 7 × 10 × 7 cm, extending from the cervical region into the superior mediastinum, with encasement of the bilateral carotid arteries and internal jugular veins, and displacement of adjacent airway structures [Figure 1].

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Figure 1:

Pre-operative magnetic resonance imaging of the neck showing a large multiloculated cystic hygroma extending from the submandibular to the suprasternal region.

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In view of the large size of the mass and airway displacement, sclerotherapy was attempted in the NICU on day 16, under ultrasound guidance. Clinical response was assessed by reduction in mass size and decrease in oxygen requirement. However, over the subsequent days, the lesion showed progressive enlargement with no clinical improvement in respiratory parameters, prompting a decision for definitive surgical excision.

Pre-operative evaluation revealed normal hemoglobin, platelet count, renal function, and coagulation parameters. Echocardiography was normal, and there was no evidence of associated congenital anomalies.

A pre-operative multidisciplinary discussion was conducted involving the anesthesia, pediatric surgery, and otorhinolaryngology teams, and a stepwise airway management plan was formulated. Plan A – VL-guided intubation under inhalational induction, with maintenance of spontaneous ventilation. Plan B – in case of failed intubation with preserved ventilation, rigid bronchoscopy– guided airway control by the otorhinolaryngology team, rigid bronchoscopy/fiberoptic intubation if feasible. Plan C – emergency surgical airway for a cannot-intubate and cannot-ventilate scenario. A full difficult-airway cart was prepared, including age-appropriate ETTs, VL with Miller straight blade [Camera Macintosh blade (C-MAC) size 1], and rigid and fiberoptic bronchoscopes. All personnel required for each step were briefed, and available prior to induction. In view of anticipated blood loss, cross-matched packed red blood cells were reserved preoperatively. The maximal allowable blood loss was calculated to be approximately 50 mL. Our anesthetic plan prioritized maintenance of spontaneous ventilation until airway establishment. Hence, we preferred inhalational induction.

The baby was received in the operation theater from NICU, with a functioning intravenous (24G) line in the dorsum of the right hand. Intraoperative monitoring included electrocardiogram, non-invasive blood pressure, pre– post ductal saturation, and temperature. An inhalational induction was carried with a size 0 face mask and Jackson– Rees circuit, gradually increasing the concentration of sevoflurane from 2% to 6%, in oxygen at 6 L/min flow, while maintaining spontaneous ventilation. Once adequate depth of anesthesia was achieved (loss of eyelash reflex, absence of purposeful movement, regular spontaneous respiration, and tolerance of gentle mask ventilation), sevoflurane concentration was reduced to 3%. No intravenous induction agents were administered to avoid apnea and loss of airway tone. Injection fentanyl 6 mcg was given to reduce intubation response. A roll was placed under the shoulder. A total of three intubation attempts were made, all by a senior pediatric anesthesiologist using a VL, which provided an acceptable glottic view (Cormack–Lehane grade IIa). The first attempt was performed with the baby’s head slightly extended, using a 3.0-mm microcuffed ETT, but was not successful despite optimization maneuvers, such as repositioning of the head to neutral alignment and external laryngeal manipulation. ETT could not be advanced beyond the glottis despite a good glottic view. Bougie-assisted intubation was not attempted due to concerns of airway trauma and limited maneuverability in the distorted neonatal airway. A second attempt using a 3.5-mm uncuffed tube was also unsuccessful. All attempts were brief and adequate oxygenation and anesthesia were maintained using intermittent gentle mask ventilation with sevoflurane in oxygen while preserving spontaneous ventilation in between the attempts. During the attempts, the neonate-maintained heart rate between 140 and 155 beats/min and SpO2 at 90–92%, with no episodes of bradycardia, or laryngospasm. Given the persistent failure of tube advancement despite adequate glottic view, escalation to Plan B was considered. However, recognizing the obstruction may be more mechanical than visual, we decided to try manual traction on the cervical mass before proceeding to rigid bronchoscopy. We sought assistance from the surgical team. Two surgeons applied gentle anterolateral traction on the mass, to realign the airway. This maneuver magically succeeded, allowing successful intubation. The 3.5 mm uncuffed ETT advanced smoothly into the trachea without resistance [Figure 2]. Following confirmation of tracheal intubation by capnography and chest movement, anesthesia was maintained with sevoflurane in an oxygen–air mixture, atracurium (0.5 mg/kg), fentanyl infusion (2 mcg/kg/h) and controlled ventilation. Adequate depth of anesthesia was monitored using end-tidal anesthetic concentration, hemodynamic parameters, and absence of autonomic responses. The patient was then handed over to the surgeons [Figure 3]. Total duration of the surgical procedure was approximately 3 h.

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Figure 2:

Video laryngoscopic view showing visible edematous vocal cords and smooth passage of a 3.5-mm uncuffed endotracheal tube through the glottis after assistant-aided retraction of the cervical mass, enabling successful airway alignment.

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Figure 3:

Post-intubation positioning of the neonate.

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Throughout the procedure, the patient remained hemodynamically stable, with SpO₂ maintained at 92–94% on FiO₂ of 0.40. The patient received 45 mL of intravenous fluids and had urine output of 8 mL. No transfusion was required since the estimated blood loss was 20 mL and remained well within the calculated ABL of 50 mL. Active warming with a warming blanket and fluid warmer maintained temperature between 35.5 and 37°C. In view of anticipated tracheal edema from prolonged surgical handling, extubation was not planned. Tracheostomy was performed by the otorhinolaryngology team at the end of surgery, following which, oral ETT was removed, and the patient was transferred to NICU for ventilatory support [Figure 4]. The baby remained stable and was successfully weaned and decannulated on post-operative day (POD) 7, following which vitals and SpO₂ on room air remained stable. The patient was discharged on POD 10.

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Figure 4:

Intraoperative image following partial debulking of the cystic hygroma and placement of neonatal tracheostomy tube (size 3.0), confirming stable airway access and effective ventilation.

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DISCUSSION

Airway management in neonates with giant cervical masses such as CH presents a rare but life-threatening challenge, primarily due to the mechanical distortion, deviation, and compression of the airway.^[8] Pre-operative assessment included evaluation for positional airway improvement by the right and left lateralization of the neonate, which did not relieve compression, limiting the utility of positioning alone. Airway compression and distortion are more classically described with solid tumors. However, large cervical CHs have also been reported to cause significant anatomical displacement and distortion of the airway due to mass effect, rather than intrinsic airway pathology, particularly in neonates with highly compliant airway structures.^[9,10]

In this case, pre-operative MRI demonstrated mass extension into the oropharynx with resultant airway angulation, alerting the team for potential difficulty during intubation. Consequently, a multidisciplinary airway management plan was formulated involving pediatric anesthesia, otorhinolaryngology, and pediatric surgery teams, emphasizing maintenance of spontaneous ventilation, availability of advanced airway devices, and preparedness for surgical airway intervention. In accordance with pediatric difficult airway principles and the Difficult Airway Society, airway management was planned with a stepwise escalation strategy prioritizing oxygenation and minimizing airway trauma [Figure 5]. VL, while maintaining spontaneous respiration, was selected as the initial approach (Plan A), as it is a familiar, rapid technique that allows continuous assessment of ventilation. Fiberoptic intubation was reserved as a secondary strategy (Plan B) due to its technical complexity and limited feasibility in neonates during emergent airway management. Rigid bronchoscopy was also included in our plan B. Surgical airway intervention was planned as a rescue option (Plan C).

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Figure 5:

Stepwise airway management algorithm for a neonate with a large anterior cervical mass. MRI: Magnetic resonance imaging, VL: Video laryngoscope, ETT: Endotracheal tube, C-MAC: Camera macintosh, IV: Intravenous.

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While difficulty with tube advancement despite good laryngeal visualization was not predicted, the possibility of airway axis distortion due to mass effect was anticipated based on MRI findings. The assistant-aided retraction maneuver employed in this case was not a predefined step in the airway plan but was adopted as a pragmatic response to persistent difficulty in ETT advancement despite adequate glottic visualization. This maneuver provided a temporary mechanical correction of airway axis distortion caused by the mass effect. To the best of our knowledge, such assistant-aided mass retraction has not been formally described in neonatal airway management literature and represents a simple adjunct based on anatomical reasoning rather than an established technique. Applying gentle anterolateral traction on the cystic mass temporarily corrected the airway axis and enabled ETT passage. This maneuver differs from the external laryngeal maneuver or backward, upward, rightward pressure as it addresses extrinsic compression rather than the glottic view. Moreover, the fragility of neonatal tissues necessitates a gentle, coordinated effort by trained personnel to minimize the risk of trauma to adjacent vital structures. In our case, two experienced surgeons performed symmetric retraction of the mass under anesthesia team supervision, ensuring control of the airway and preventing venous compression.

Existing literature tends to focus on advanced devices such as fiberoptic bronchoscopes or surgical airway approaches.^[11] However, in time-critical neonatal airway situations, the immediate availability of appropriately sized equipment and personnel with advanced neonatal airway expertise may be limited, which can restrict the feasibility of these techniques. The simplicity, safety, and immediacy of assistant-aided manual retraction make it an invaluable addition to the difficult-airway algorithm for neonates with cervical masses.

Tracheostomy is not routinely performed in neonates and is reserved for selected situations. In our case, the decision to perform tracheostomy was taken at the end of surgery, due to anticipated tracheal edema from surgical handling, and risk of airway compromise in the post-operative period.

CONCLUSION

This case describes an anatomy-driven adjunct that facilitated ETT advancement in a neonate with a large cervical mass despite adequate VL visualization. In retrospect, this maneuver could have been anticipated and discussed during pre-operative planning in cases of significant extrinsic airway compression. The experience underscores the importance of anticipatory planning, multidisciplinary coordination, and dynamic assessment of airway mechanics in complex neonatal airway management. From a learning standpoint, this case illustrates a practical, anatomy-driven maneuver that may aid endotracheal intubation and serve as a pragmatic bridge between visualization-based techniques and invasive airway interventions in selected neonates with large cervical masses. We feel that this is a novel, adjunct derived from real-time anatomical assessment rather than a pre-established or recommended maneuver and is easy to adopt and apply in emergency situations.