Bali Journal of Anesthesiology

CASE REPORT
Year
: 2020  |  Volume : 4  |  Issue : 3  |  Page : 132--135

Anaesthetic management and challenges involved in goldenhar's syndrome; A series of two cases


Sudha Puhal, Sudhir Kumar Bisherwal, Rahul Saini, Anju Rani 
 Department of Anaesthesia, Pain and Critical Care, PGIMS University of Health Sciences, Rohtak, Haryana, India

Correspondence Address:
Dr. Sudhir Kumar Bisherwal
Department of Anaesthesia, Pain and Critical Care, PGIMS University of Health Sciences, Rohtak, Haryana
India

Abstract

Goldenhar's syndrome is a congenital defect caused by maldevelopment of the first and second brachial arch. This causes maldevelopment of the ears, nose, soft palate, mandible, and vertebral column. These patients pose a unique challenge to anesthetists in having difficult management of the airway and neuraxial blockade. This report describes two patients of Goldenhar syndrome who came for the surgery under anesthesia and their management.



How to cite this article:
Puhal S, Bisherwal SK, Saini R, Rani A. Anaesthetic management and challenges involved in goldenhar's syndrome; A series of two cases.Bali J Anaesthesiol 2020;4:132-135


How to cite this URL:
Puhal S, Bisherwal SK, Saini R, Rani A. Anaesthetic management and challenges involved in goldenhar's syndrome; A series of two cases. Bali J Anaesthesiol [serial online] 2020 [cited 2021 Mar 5 ];4:132-135
Available from: https://www.bjoaonline.com/text.asp?2020/4/3/132/290092


Full Text



 Introduction



Goldenhar's syndrome is a birth defect resulting from the maldevelopment of the first two branchial arches, with incomplete development of the ear, nose, soft palate, lip, and mandible. The phenotype is highly variable.[1]

The classic triad is mandibular hypoplasia resulting in facial asymmetry, ear and/or eye malformation, and vertebral anomalies.[2] Goldenhar's syndrome is often characterized by a combination of anomalies, including dermal epibulbar cysts, auricular appendices, and malformations of the ears. It has also been described as having an association with cardiac, pulmonary, renal, and vertebral anomalies.[3],[4],[5],[6] Oral manifestations in this syndrome are clearly heterogeneous and range from malocclusion to a more complex phenotype with a complete absence of the mandibular ramus and temporomandibular joint.[5] The syndrome presents at birth, and the exact etiology is unknown.

These patients pose a unique challenge to anesthetists because of their facial deformity with abnormal vertebral curves which renders their spine and neckless flexible for intubation and spinal anesthesia. Our first case was a 45-year-old male presenting with avascular necrosis of the hip posted for the hip replacement surgery. He was managed with a combined spinal–epidural (CSE) technique. Our second case was a 3-month-old baby posted for the excision of limbal dermoid. He was given general anesthesia with a supraglottic device.

 Case Reports



Case 1

A 45-year-old male, married, belonging to low socioeconomic status, washerman by occupation presented with a chief complaint of pain in the right hip for 3 years and limping for 2 years. The patient was evaluated by orthopedics and diagnosed as having avascular necrosis of the hip joint and subsequently posted for total hip replacement.

The patient had no medical or surgical history. On examination, he had an asymmetric face with mandibular hypoplasia [Figure 1]. He had reduced mouth opening of 1 cm with bucked and malaligned teeth. Mallampati grade (MPG) could not be assessed because of a small mouth opening. He also had a deviation of the jaw on mouth opening, with a normal full range of the neck movement. On vertebral examination, he was found to have lumbar scoliosis and had difficulty in flexing his spine without elicitation of any tenderness [Figure 2]. He also gave a history of a small skin tag in front of the left ear, which was removed when he was a teenager. However, no scar was visible near the ear. His echocardiography report was immaculate, as was his renal and liver function tests.{Figure 1}{Figure 2}

The patient was administered ranitidine and metoclopramide on the night before and on the morning of the surgery for aspiration prophylaxis. He was taken to the operating room (OR) and was planned for CSE. General anesthesia was employed as a backup plan should the CSE fails. Difficult intubation cart and cricothyroidotomy set were ready for immediate use in case of any emergency.

After attaching electrocardiogram leads, noninvasive blood pressure (NIBP) cuff, and pulse oximetry probe, the patient was positioned in the lateral position with the affected side down for CSE. An epidural needle was inserted with difficulty and by insertion point in the midline and directing the needle laterally. The epidural catheter was inserted with 16G Tuohy's needle in L3–L4 space with a median approach, and then a 25G quincke spinal needle was inserted in L3–L4 space and 15 mg of 0.5% bupivacaine heavy was injected after confirmation of free flow of cerebrospinal fluid. Epidural test dose was given with 3 ml of 2% lignocaine with adrenaline (1:200,000). The patient was kept in the same lateral position for 10 min after spinal and was handed over for the surgery after adequate effect was observed.

The patient was stable intraoperatively. The surgery finished within 2 h, and hence no epidural top-up was given in the OR. Postop analgesia was given with 5 ml of 0.125% bupivacaine with 25 μg fentanyl through epidural space and 1 g paracetamol intravenously. Epidural was left in situ for pain control and was removed 2 days later. There was no complication in the postoperative period, and the patient was discharged on day 10 after the removal of skin sutures.

Case 2

A 3-month-old male baby (6 kg) born to nonconsanguineous healthy parents by normal vaginal delivery at the full gestational period was referred to the ophthalmology department for removal of skin tags near the lateral angle of the left eye. There was no history of delayed crying on birth or any admission to the neonatal unit after birth. There was no history of cyanosis on crying or feeding, no history of any recurrent respiratory infection or seizure. On examination, he had a deviation of angle of the mouth with multiple skin tags on the left side of face largest being near the eye [Figure 3]. On palpation, there was hypoplastic ramus and body of mandible on the left side. The vertebral spines on palpation appeared normal.{Figure 3}

His skin tag near the eye was examined and found to be arising from the limbus and was diagnosed as limbal dermoid. His echocardiography was done to rule out any associated cardiac pathology which came normal.

His mother was explained about fasting on the night before surgery, and the child was taken to the prewarmed OR. The child was given oral midazolam as premedication. In OR, the child was given a mixture of halothane and oxygen, after which a 24G venous cannula was inserted. He was then given fentanyl 15 μg, and a size-1, I-Gel laryngeal mask airway (LMA) was inserted after deepening the patient with propofol. He was ventilated with Mapleson circuit F with a 50–50 mixture of oxygen and nitrous oxide with halothane. The surgery took 10 min, and the I-gel was removed after adequate spontaneous breathing was observed. Paracetamol injection was given for postoperative analgesia. There was no significant intraoperative cardiovascular or difficult ventilation event. The patient was discharged the next day.

 Discussion



The incidence of Goldenhar's syndrome has been reported to be from 1:3500 to 1:5600, with a male:female ratio of 3:2.[7] Patients with any malformation of face or airway should be suspected as a case of the difficult airway.[8] The anesthetic management of such a patient can be challenging as they may pose difficulty while securing the airway during general anesthesia or in case of any catastrophic intraoperative event requiring intubation. The scoliotic spine also poses a unique challenge for the anesthetist and may complicate regional anesthesia. The patient may often require general anesthesia in case of failed or patchy block.

Goldenhar syndrome is characterized by a wide range of congenital anomalies, including ocular, auricular, cranial, vertebral, and cardiac anomalies. Mandibular hypoplasia and vertebral anomalies often result in limitation of neck movement.[9] During the preoperative evaluation, a suitable method of airway management should be selected considering the patient's special needs. LMA is an adjunct that can be used in case of difficult mask ventilation.[10] Short-acting neuromuscular blockers should be used for intubation. These patients must also be carefully evaluated for other associated anomalies (especially cardiac and pulmonary) during preoperative evaluation.

Tilting and rotation of vertebra in scoliosis make it difficult for the needle to reach epidural or subarachnoid space by the median approach. One might even have to use other techniques to get to space. As the vertebral body rotates toward the convex-side of the scoliotic curve, a direct path to the neuraxial spaces occurs on the convex-side when using a paramedian approach. Therefore, if the spinous process is palpable or can be identified with a needle, then a paramedian approach could be attempted on the convex side of the curve.[11] If a midline approach is used, the spinal needle should be angled in the transverse (axial) plane towards the convex-side of the curve.[12]

Because of the abnormal curves of the vertebral column, there may also be a patchy or unilateral or inadequate effect of the block in spite of a successful lumbar puncture. To prevent this, our patient was kept in the lateral position with the affected side down to have a unilateral effect and to minimize the chances of inadequate effect. Utilizing ultrasonography may improve the success rate.[13] Computed tomography (CT) scans can also be used to aid in spinal anesthesia.[14]

Difficult intubation is not infrequent in patients of Goldenhars' syndrome.[15] Difficult mask ventilation has also been reported in such patients. Moreover, children are more prone to develop airway related complications such as laryngospasm and bronchospasm. Mask ventilation may cause insufflation of the stomach with air, which may increase the chances of aspiration. Supraglottic devices are easy to place and need lesser muscle relaxation and anesthesia than endotracheal intubation. These devices are associated with lesser complications.

 Conclusion



Meticulous preparation is the key to manage difficult cases in anesthesia. Spinal deformity poses a challenge during spinal anesthesia. However, an out of box thinking can buy success for these cases. One should not hesitate to make use of a CT scan or ultrasound for spinal needle placement.

In the pediatric population, spontaneous ventilation is very important because of the rapid development of hypoxia. LMA can easily be placed by deepening the patient with an inhalation agent or any intravenous agent without paralyzing the child. Muscle relaxants can be kept on hold until the airway is secured.

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.

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