Acta Clin Croat (Suppl. 1) 2016; 55:90-93 Case Report doi: 10.20471/acc.2016.55.s1.13 AIRWAY MANAGEMENT WITH DIRECT LARYNGOSCOPY IN A CHILD WITH GOLDENHAR SYNDROME Bibiana Vitković and Morena Milić Clinical Department of Anesthesiology and Intensive Care Medicine, Dubrava University Hospital, Zagreb, Croatia SUMMARY – Goldenhar syndrome, also known as oculoauriculovertebral dysplasia, is a rare congenital condition characterized by facial, cranial, vertebral, ocular, auricular and cardiac abnor- malities. This syndrome is associated with hemifacial microsomia due to inadequate growth of the mandible and vertebral anomaly of the cervical part of the spine. For anesthesiologists, airway ma- nagement is of great interest because of facial and oral abnormalities such as mandibular hypoplasia and limitation of neck movement. Considering different conditions of Goldenhar syndrome, every patient should be preoperatively evaluated in order to make the plan for the anticipated difficult airway management. We report a case of a 2.5-year-old boy undergoing palatoplasty, who required general anesthesia and orotracheal intubation. Our decision to intubate with direct laryngoscopy and with slight external pressure on the larynx proved successful. Key Words: Airway Management; Airway Obstruction; Goldenhar Syndrome; Anesthesia; Palato- plasty Introduction epibulbar dermoid cyst, and unilateral maxillary hy- poplasia. Eyelid colobomas, microphthalmia, genito- Goldenhar syndrome was first described in 1952. urinary anomalies and deafness may also be included. The syndrome is sometimes hereditary, autosomal re- Cleft palate can be found in 10% of these children. cessive or dominant transmitted, but may also occur The consequence of unilateral mandibular hypoplasia randomly. The pathogenesis can be explained with and vertebral anomalies are limitations of neck move- inadequate vascular supply of the first two brachial ments and changes in the airway anatomy. Accord- branches, resulting in malformation of structures in ingly, anesthesiologist must be aware of difficult ven- fetal development. The incidence is estimated be- tilation and difficult intubation1. tween 1/3000 and 1/5000 live births, and the male/ We describe successful orotracheal intubation female ratio is 2:1, with male predominance. in a 2.5-year-old child with Goldenhar syndrome, Goldenhar syndrome is also known as oculoauric- scheduled for plastic reconstruction of the cleft pal- ulovertebral dysplasia or hemifacial microsomia. The ate. Despite predicted difficult airway management, syndrome include the following conditions: hemifa- we successfully intubated the child using direct laryn- cial microsomia, vertebral anomalies of different size goscopy. and shape, microtia, preauricular skin tags, central nervous system malformations, cardiac malformation, Case Report A 2.5-year-old boy, body weight 10 kg, was pre- Correspondence to: Bibiana Vitković, MD, Clinical Department of Anesthesiology and Intensive Care Medicine, Dubrava Univer- sented for palatoplasty. He was delivered as a prema- sity Hospital, Avenija Gojka Šuška 6, HR-10000 Zagreb, Croatia ture due to fetal suffering, birth weight 1420 g, Apgar E-mail: [email protected] score 7/7. Immediately after birth, oxygen saturation Received February 27, 2015, accepted: September 9, 2015 was low, consequently nasotracheal intubation was 90 Acta Clin Croat, Vol. 55, (Suppl. 1) 2016 Bibiana Vitković and Morena Milić Airway management of Goldenhar syndrome performed. After caffeine citrate therapy in intensive was confirmed by auscultation and capnography. Af- care unit (ICU), the child was extubated and stayed in ter thoughtful fixation, a small amount of throat pack the hospital for 58 days. The boy was diagnosed with was put in the oropharynx as additional fixation of the Goldenhar syndrome. endotracheal tube and capture for saliva and blood. Physical examination performed preoperatively re- Electrocardiography, peripheral oxygen satura- vealed micrognathia and hypoplasia of the right side tion, noninvasive blood pressure, body temperature of the mandible; furthermore, the coronoid process and capnography were monitored. Stethoscope was and the mandibular condyle did not exist; complete attached with a patch on the left side of the thorax. clefts of the soft and the last third of the hard pal- The intravenous cannula was inserted on the left foot. ate were visible. Ears were underdeveloped and there At the beginning of surgical procedure, the opera- were two bilateral preauricular tags. Additionally, tion field was infiltrated by the surgeon with a mix- there was a coloboma, a minor defect on the medial ture of lidocaine and adrenaline, and correct position half of the right upper eyelid. of the orotracheal tube was confirmed once again after Cervical spine x-ray showed hemivertebra C7 and introducing the mouth gag. The anesthesia was main- megastransversus C7 and Th 1. Consequently, the tained with sevoflurane 2 vol %. During the surgery, examination showed torticollis with concavity on the the patient was hemodynamically stable, peripheral right side and flat physiological lordosis. oxygenation was 100%, and body temperature was X-ray of thoracic organs, abdomen ultrasound normal; the patient received prophylactic cefuroxime and brain ultrasound were normal. Ultrasound of the 30 mg/kg and dexamethasone 4 mg. Palatoplasty, fre- heart revealed foramen ovale apertum and small tri- nectomy and removal of preauricular tags were per- cuspidal regurgitation; electrocardiography showed formed. At the end of 60-minute operation, the boy sinus rhythm of 150/min; the cardiologist confirmed started breathing spontaneously and was awake soon. good heart performance. Postoperative period was uneventful and the patient Laboratory findings were as follows: hemoglo- was discharged from the hospital after 6 days. bin 12 g/dL, hematocrit 33%, and leukocyte count 8.5x109/L. There were no known allergies and no Discussion history of previous surgery. According to airway as- The presence of cleft palate without cleft lip in a sessment, since the child was not cooperative at the newborn is often correlated with other birth defects moment of examination, the Mallampati score was and syndromes3. The best known syndromes corre- 2 not possible to evaluate . Movements of the neck were lating with isolated cleft palate are Pierre Robin se- limited. He was classified as the American Society of quence, Treacher Collins syndrome, Goldenhar syn- Anesthesiologists (ASA II) physical status. drome, and velocardiofacial syndrome. Premedication was administered 30 min before Children born with congenital disorders deserve surgery: 1 mg midazolam and 0.15 mg atropine, in- special attention of anesthesiologist when airway tramuscularly. Anesthesia was induced by using 100% management and general anesthesia are required. oxygen with sevoflurane which was slowly increased. Every patient should be carefully examined. It is im- Additionally, when ventilation was satisfactory, thio- portant to notice morphology of the head and neck, pental 40 mg, fentanyl 0.03 mg and vecuronium 1 micrognathia, jaw movements, dentition, and pos- mg were administrated to deep anesthesia and to al- sible dysmorphic features2. After preoperative ex- low best condition for laryngoscopy. After 3 minutes amination, anesthesiologists should prepare the plan of uneventful ventilation, laryngoscope (Macintosh for tracheal intubation, i.e. difficult airway manage- blade number one) was slowly introduced, the tip of ment, and consider using special devices. Orotra- the epiglottis was shown. Slight external pressure on cheal intubation (flexible tube with cuff) is the safest the larynx was performed by anesthetic technician, approach to protect airway in plastic reconstruction visibility of aditus was improved, and arytenoids were of cleft palate. visualized. Flexible tube number 3.5 I.D. with sty- If the risk of intubation is greater than the benefit let was placed. The position of the orotracheal tube of the procedure, anesthesiologist, surgeon and par- Acta Clin Croat, Vol. 55, (Suppl. 1) 2016 91 Bibiana Vitković and Morena Milić Airway management of Goldenhar syndrome ents have to decide whether to abandon or proceed Conclusion with the surgical procedure. The only wise approach to the management of the The standard technique for expected difficult- in anticipated difficult airway in a child with Goldenhar tubation is awake intubation with flexible fiberoptic syndrome is clear strategy that provides satisfactory bronchoscope (FOB) using topical anesthesia if the oxygenation, ventilation and good surgical visualiza- patient is cooperative. The children cannot be coopera- tion. Orotracheal intubation with direct laryngoscopy tive and sedation or general anesthesia before fiberop- and slight external pressure on the larynx was a good tic intubation is necessary. FOB is expensive and easily choice in this case. We decided to administer muscle damaged, especially those of small pediatric diameter; relaxant to facilitate intubation, only after confirma- the usage of FOB needs special education and training. tion of adequate ventilation. Recent studies have shown that fiberoptic intubation An individual management plan should be se- via laryngeal mask airway (LMA) is acceptable tech- 4 lected considering the special conditions and different nique for a child with Goldenhar syndrome . Com- airway anatomy of every child with Goldenhar syn- parison of FOB intubation and intubation with rigid drome.