Infantile Cystic Hygroma: An Unusual Perioperative Course.

Airway management of an infant with a giant cervical lump may be a difficult task. The anesthesiologist must be prepared to face associated challenges during securing the airway in such patients. We report our experience with One year old infant who presented with huge cystic hygroma in the cervical region leading to recurrent episodes of respiratory tract infection and distress. Surgical removal was needed as sclerotherapy proved ineffective in reducing its size. Proseal laryngeal mask airway was used as a conduit after inhalational induction since airway could not be maintained with bag and mask. The child was tracheostomized postoperatively and also had a prolonged Intensive Care Unit stay. Difficulties encountered in intubation and postoperative management of this child are discussed in this report.

INTRODUCTION

Anesthetic management of the airway may be a challenge in neonates and young infants with a giant cervical lump. Cystic hygroma, a benign, congenital malformation of the lymphatic system is one such example. The prevalence rate is 1:6000–16,000 newborn in the US.[1] Cystic hygroma manifests anywhere in the body; however, 70–80% of cases arise in the neck, particularly in the left posterior cervical triangle.[2] Anesthetic concerns include extrinsic or intrinsic pressure on the airway causing distortion and obstruction of the airway. Therefore, a strategy needs to be developed to anticipate and manage patients in such difficult scenarios. We describe the difficulties encountered in the preoperative airway management and postoperative care of pediatric patients with special reference to Proseal laryngeal mask airway (PLMA) as a rescue device.

CASE REPORT

A One year old male child weighing 6 kg presented with a cystic mass arising from left posterior triangle and extending to right posterior triangle across the midline. Inferior extension was up to supraclavicular region, bilaterally [Figure 1a]. The mass progressively increased in size since birth. On local examination, the swelling was soft, nontender, and measured 12 cm × 10 cm. The infant presented with repeated episodes of infection leading to stridor but no dysphagia. Surgical excision of the mass was planned after no response to repeated intralesional injection of bleomycin.

Figure 1

(a) Child in preoperative period, (b) Child after intubation.

Concurrent anomalies such as Down's syndrome, Turner syndrome, and congenital heart defects were ruled out in preoperative evaluation. Size and extent of swelling were defined clearly by magnetic resonance imaging to avoid any potential airway compromise during intubation. X-ray chest (posterior-anterior view) showed the minimal deviation of trachea to the right side without any compression and no intrathoracic extension of the mass. No signs of lower respiratory tract infection were seen in X-ray. The child had a history of a cough with expectoration 2 weeks ago for which he was treated with antibiotics. In view of repeated episodes of stridor and infection, surgical excision of the mass was planned despite incomplete resolution of Upper respiratory tract infection (URI). Informed consent for anesthesia was taken and parents were explained about the risk of anticipated difficult airway and postoperative ventilation. Difficult airway cart was kept ready. Because of nonavailability of pediatric (fiberoptic bronchoscope [FOB]), it was decided to proceed with the conventional method for securing the airway. Pulse oximeter, electrocardiograph, and noninvasive blood pressure monitor were applied and baseline parameters recorded. 24-G intravenous cannula was already in place and child was premedicated with injection glycopyrrolate 0.01 mg/kg and injection fentanyl 2 µg/kg intravenously. Supine position was maintained using a shoulder roll because of prominent occiput for optimal laryrgoscopic position [Figure 1b]. Anesthesia was induced with graded concentration of sevoflurane (2–6%) in 100% O2. Mask ventilation was found to be difficult as anticipated despite the insertion of the oropharyngeal airway (OPA) as jaw-thrust was not possible due to cervical lump. During induction, the child went into respiratory obstruction and desaturated (SpO2 79%). The child was turned to left lateral position immediately to allow the mass to fall away from the midline. However, this maneuver did not help much. Since airway could not be maintained with bag and mask, spontaneous/assisted ventilation was facilitated by inserting PLMA size 1.5 and adequate anesthetic depth achieved successfully. Direct laryngoscopy was then attempted without using muscle relaxant in view of anticipated difficult intubation. Glottic opening was found to be deviated to right toward 2 o’clock position and Grade II Cromack and Lehane laryngoscopic view was noted. Trachea was successfully intubated in the first attempt with size 4.5 uncuffed portex endotracheal tube (ETT) with slight external laryngeal manipulation. Injection atracurium 0.5 mg/kg and injection dexamethasone 2 mg, then followed intravenously. Paracetamol suppository 80 mg was inserted for perioperative analgesia. Anesthesia was maintained with 66% N2O in O2, sevoflurane (1–2%) and intermittent doses of atracurium. Intraoperative period was uneventful which lasted for 2 h. Cysts were multiloculated and could not be excised completely due to the vicinity of a cyst to major vessels. In view of preoperative chest condition, anticipated increase in edema and respiratory obstruction in the postoperative period, it was decided to electively ventilate the child in Intensive Care Unit (ICU). Only morphine sedation was continued for tube tolerance after 24 h. All hemodynamic parameters remained stable throughout the ICU stay. In the following 72 h, child was alert, active but could not be extubated due to gross bilateral facial edema (left > right) [Figure 2] and excessive secretions in ETT despite appropriate antibiotics and steroids. Feeding through the nasogastric (NG) tube was started on the 3rd postoperative day (POD). On the 4th POD, child accidently self-extubated. Mask ventilation was again difficult even with OPA due to edema and dressing. Laryngoscopy revealed marked swelling in the hypopharynx and laryngeal inlet. Trachea could be successfully intubated with size 4.0 portex uncuffed ETT with a minimal acceptable leak around the tube confirming airway edema. On the following day, X-ray chest revealed a pneumonitic patch in the left middle zone and child developed a fever. The decision for tracheostomy was taken on the 7th POD due to nonresolution of edema of the upper airway and poor chest condition. Patient demonstrated signs of improvement after adequate pulmonary toileting following tracheostomy. Arterial blood gas was maintained throughout this period. Gradually, child was weaned and was transferred to ward on the 15th POD with tracheostomy tube in situ.

Figure 2

Child in Intensive Care Unit showing edematous face and neck.

DISCUSSION

Cystic hygroma is a congenital, developmental malformation of lymphatic system with no malignant potential. Mostly it presents in infancy as a gradually increasing mass which may produce stridor or dysphagia. Treatment choices include repetitive suction, injection of curing agent into the mass, radiation therapy, and radiofrequency treatment.[3] However, the only radical treatment method is surgical removal.[4] In our case, repeated bleomycin injections proved unsuccessful. In addition, sudden increase in size of mass due to infection and inflammation led to respiratory compromise. Eventually, there was no choice but to take up the patient despite residual respiratory infection.

Pediatric difficult airway is always challenging for anesthetist. The mass leads to several anesthetic problems such as difficult ventilation, intubation, intraoperative hemorrhage, and postoperative airway complications. It is mandatory for the anesthetist to understand the extent of invasion into respiratory tract by the cyst and prepare for airway management accordingly.

Compression of upper or lower airway due to mass can cause retention of secretions and hence these children are prone to pneumonia.[5] Our child also presented with a history of repeated respiratory infection secondary to retained secretions. Preoperative chest infections should adequately be treated before planning for surgery to reduce morbidity and mortality. Obstruction of the airway is common following the loss of consciousness in huge cervical cystic hygroma. It can be improved by turning the patient to lateral or even to semi-prone position.[6] It proved to be of no help in our case. Principle of safe induction of anesthesia is to maintain spontaneous ventilation until trachea is secured. Awake FOB is the technique of choice but difficult and traumatic in infants. Tracheostomy is also not a feasible option due to indistinguishable landmarks. Inhalational anesthesia remains the preferred technique for management of difficult pediatric airway.[7] Supraglottic devices can be life savior for maintaining spontaneous ventilation and deepening the level of anesthesia when mask ventilation is difficult due to a huge mass. Insertion of PLMA at the right moment helped us to maintain the airway. Sinha et al. also observed improved oxygen reserve by the use of PLMA as a conduit before laryngoscopy in morbidly obese patients.[8] External laryngeal manipulation allowed successful intubation despite deviated glottic opening. The presence of expert assistance and all standby preparations are very crucial for successful airway management in such cases. Respiratory complications can occur in postoperative period due to reactionary edema or residual lesion as observed in our case Sharma et al. also experienced similar problems in their case.[5] Preoperative chest condition was also a contributing factor in this patient. Direct communication between surgeon and anesthesiologist is a must for better patient outcome. Our patient continued to remain on ventilatory support due to gross facial, pharyngeal, and laryngeal edema. Superadded chest infection in ICU along with edema necessitated tracheostomy in this child. Ameh and Nmadu carried out a retrospective study about the perioperative complications in 41 children with cervical cystic hygroma. They observed preoperative life-threatening complications in 80% of children and 24% of patients experienced complications such as edema, respiratory obstruction, and chest infection in the postoperative period.[9]

Another postoperative concern reported in 20% of cases of excision of cystic hygroma, is damage to 7th, 11th, and 12th cranial nerve.[10] This can lead to the floppy tongue and obstructed the airway. This was not observed in our case.

CONCLUSION

Cervical cystic hygroma is a known difficult airway, but difficult mask ventilation is less reported. Cervical cystic hygromas are challenging at every stage of their management. In this case report, we aimed to describe the role of adjunct airway equipment for maintaining ventilation and airway and the complications encountered in the perioperative period. The importance of proper preparation for such cases and anticipation of possible postoperative complications can never be overemphasized. A definite plan for cannot ventilate, cannot intubate situation with available equipment before proceeding with further management is essential to avoid morbidity and mortality.

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.