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CASE REPORT
Year : 2023  |  Volume : 17  |  Issue : 1  |  Page : 83-86

Anesthetic challenges in the management of Larsen syndrome: A rare congenital anomaly


1 Department of Trauma and Emergency, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
2 Department of Anesthesiology and Critical Care, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India
3 Department of Orthopedics, All India Institute of Medical Sciences, Bhubaneswar, Odisha, India

Correspondence Address:
Subhasree Das
Department of Trauma and Emergency, All India Institute of Medical Sciences Bhubaneswar, Odisha - 751 019
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/sja.sja_518_22

Rights and Permissions
Date of Submission13-Jul-2022
Date of Decision14-Jul-2022
Date of Acceptance14-Jul-2022
Date of Web Publication02-Jan-2023
 

  Abstract 


Larsen syndrome is a rare inherited disease associated with dislocations of multiple joints, typical syndromic facies, and multiple spine abnormalities. They often required multiple corrective orthopedic surgeries to regain their functional ability, thus needing repeated anesthesia. Apart from skeletal deformities, they have predicted difficult airway and need extreme care during intubation and positing of the patient. Abnormal posturing due to spinal deformity and poor pulmonary reserve due to kyphoscoliosis creates an extremely challenging situation for the anesthetist to manage the case during the perioperative period. Here we are describing the perioperative anesthetic management of a patient with Larsen syndrome.

Keywords: Difficult intubation, kyphoscoliosis, Larsen syndrome, poor pulmonary reserve


How to cite this article:
Das S, Panda A, Jena SS, Jain M. Anesthetic challenges in the management of Larsen syndrome: A rare congenital anomaly. Saudi J Anaesth 2023;17:83-6

How to cite this URL:
Das S, Panda A, Jena SS, Jain M. Anesthetic challenges in the management of Larsen syndrome: A rare congenital anomaly. Saudi J Anaesth [serial online] 2023 [cited 2023 Feb 3];17:83-6. Available from: https://www.saudija.org/text.asp?2023/17/1/83/364866




  Introduction Top


Larsen (or Larsen's) syndrome is a rare connective tissue disorder associated with variable clinical presentations like characteristic facial features (prominent forehead, flattened nasal bridge, and hypertelorism), dislocations of multiple joints, deformities of the limbs, along with various vertebral anomalies like scoliosis, spinal stenosis and vertebral instability resulting in neurological deficits.[1],[2],[3] Dysmaturity of collagen bundles results in a reduction in rigidity of cartilages of the larynx, trachea, and rib cage.[4] All these abnormalities pose a tremendous challenge for anesthetists. Here, we have described the perioperative anesthetic risks associated with Larsen syndrome, how to prevent the complications and to manage the adverse events promptly.


  Case Report Top


A 17-year-old male with Larsen syndrome suffered from myelopathy due to cervical kyphosis and neuromuscular scoliosis (NMS) that made him unable to sit erect without support [Figure 1]a. On examination, he was conscious with average intelligence, carrying typical syndromic facies with congenital dislocation of multiple limb joints [Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e, including the bilateral knee and elbow that made him dependent on others (his mother) for all his daily activities. Previously he could sit erect with a fixed and locking attitude of his limbs, but for a year, he was unable to do so, needing support because of the NMS.
Figure 1: Patient unable to sit without support. Notice the locked posture while sitting in (a), and dislocation in the bilateral foot (b), knees (c), elbow(d), and wrist (e)

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Staged surgeries were planned for occiput to pelvis fixation to make him able to sit. Cervical anterior decompression with combined anterior–posterior instrumentation was scheduled as the first surgery.

His chest was clear on auscultation, but his breath-holding time was significantly low, probably due to restrictive lung disease, for which incentive spirometry and chest physiotherapy were advised earlier. Airway was difficult due to abnormal head position because of cervical kyphoscoliosis, large tongue, and limited neck extension. All the laboratory parameters, along with electrocardiogram (ECG) and echocardiogram (ECHO), were within normal limits. The radiograph of the patient's spine and deformed joints is shown in [Figure 2]a,[Figure 2]b,[Figure 2]c,[Figure 2]d. The spine's magnetic resonance imaging (MRI) revealed cervical and thoracic kyphoscoliosis [Figure 2]e with severe spinal canal stenosis (AP diameter was 2 mm at C4-C5).
Figure 2: X-ray confirms a long C-type scoliosis (a), with multiple joint dislocations (b–d); the severe cervical kyphosis and cord compression can be appreciated in this T-2 weighted sagittal MRI (e)

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On the day of surgery, the patient was carefully shifted to the operating table, and all the standard monitors were attached. Due to cervical-thoracic kyphoscoliosis, only the left lateral position was possible. It was challenging to place peripheral intravenous lines due to the laxity of skin and contracture deformity of almost all joints. Under local anesthesia, the left internal jugular line and radial arterial cannulation were inserted using ultrasound (USG) guidance. Awake fiberoptic nasal intubation was performed with a 6.5 mm ID reinforced tube. Surgery was started with anterior decompression of the spinal cord. Both C-3 and C-4 corpectomies were performed, which lasted for 3 h, intraoperative hemodynamic was maintained, and the blood loss was minimal.

After completing the surgery on the anterior aspect, the patient was repositioned to prone for posterior decompression of the cervical spinal cord. After 20 min of prone positioning, the airway pressure increased gradually from 18 to 30 H2O. Meanwhile, the patient went into bradycardia, followed by cardiac arrest. Immediately patient was made supine after tight packing of the surgical incision, and cardiopulmonary resuscitation (CRP) started. Return of spontaneous circulation (ROSC) was achieved after two cycles of CPR and 1 mg adrenaline bolus. Following ROSC, ventricular tachycardia persisted. So, amiodarone infusion was continued after a bolus dose, and gradually rhythm reverted to normal sinus rhythm. The rest of the surgical procedure was abandoned following this event, and the patient was shifted to the intensive care unit (ICU) with minimal inotropic support, which was gradually tapered and stopped over 4 h.

The patient became conscious in 2 h and was extubated within the next 24 h. On the third postoperative day in the ward, he developed breathing difficulty and desaturated due to postoperative lung atelectasis. The patient was again shifted back to ICU, where he gradually recovered and got discharged on the tenth postoperative day. He was advised to continue chest physiotherapy at home. Eventually, the patient underwent second and third corrective spine surgery [Figure 3]a,[Figure 3]b,[Figure 3]c after 3 and 4 months, respectively, and recovered well without any anesthetic and surgical complications, but unfortunately, he succumbed to COVID because of the severe respiratory compromise.
Figure 3: Sagittal CT following first surgery showing corpectomy with cage and plate (a); the final postoperative radiograph is shown in AP view (b), Lateral view (c)

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  Discussion Top


There were many challenges in managing this case in the perioperative period. The difficulties were encountered while securing venous access, managing the airway, and during patient positioning.

Atelectasis and pneumonia are possible complications of thoracic deformities in patients with Larsen syndrome.[5] So, chest physiotherapy and frequent pulmonary toileting were required during the perioperative periods. In our case, poor respiratory reserve and micro-aspiration of oral secretions led to postoperative lung atelectasis. Similarly, reports have suggested that patients with Larsen's syndrome develop early hypoxia due to poor pulmonary reserve.[6]

We have performed awake fiberoptic intubation to minimize neck movement in this patient. Tobias reported that Larsen syndrome patients are associated with cervical instability and are prone to cord compression. So, they suggested that the cervical spine of these patients should be assessed radiologically, and intubation should be performed in the neutral position after stabilizing the head and neck.[5]

Pavan Malick et al. established somatosensory evoked potential (SSEP) monitoring before tracheal intubation and prone positioning in two such patients and observed a change in evoked potential during intubation in one patient. So, they proposed that early establishment of SSEP monitoring is helpful in this type of patient who has a high chance of cord compression during intubation.[7] Careful handling is required during patient positioning because of skeletal deformities, limb contractures, and cervical instability to prevent further cord compression.[7]

The peak airway pressure dramatically got elevated following prone positioning because of poor chest wall compliance leading to increased intrathoracic pressure. This, in turn, causes decreased venous return and a low cardiac output state contributing to cardiac arrest.

Quadriplegia and death due to minor trauma to the cervical spine has already been reported in the literature.[8] So, another cause of cardiac arrest in our case could be spinal cord compression following a prone position. Monitoring SSEP before anesthesia induction and or before positioning the patient could have helped us to identify cord compression with no delay.


  Conclusion Top


Many of these patients have normal intelligence despite multiple organ systems involvement and can lead a normal life with proper corrective surgeries. Careful and vigilant monitoring during the perioperative period is the key to early recognition and management of complications. Difficulties related to intubation should be anticipated prior, and postoperative respiratory problems should be addressed carefully by proper monitoring.

Anesthesia for children with Larsen syndrome can be extremely hazardous, so these patients need proper systemic evaluation to conduct surgery under anesthesia.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Larsen LJ, Schottstaedt ER, Bost FC. Multiple congenital dislocations associated with characteristic facial abnormality. J Pediatr 1950;37:574-81.  Back to cited text no. 1
    
2.
Murphy-Ryan M, Psychogios A, Lindor NM. Hereditary disorders of connective tissue: A guide to the emerging differential diagnosis. Genet Med 2010;12:344-54.  Back to cited text no. 2
    
3.
Wieisenbach J, Melegh B. Vertebral anomalies in Larsen's syndrome. Pediatr Radiol 1996;26:682-3.  Back to cited text no. 3
    
4.
Rock MJ, Green CG, Pauli RM, Peters ME. Tracheomalacia and bronchomalacia are associated with Larsen syndrome. Pediatr Pulmonol 1988;5:55-9.  Back to cited text no. 4
    
5.
Tobias JD. Anesthetic implications of Larsen's syndrome. J Clin Anesth 1996;8:255-7.  Back to cited text no. 5
    
6.
Critchley LAH, Chan L. General anaesthesia in a child with Larsen syndrome. Anaesth Intensive Care 2003;31:217-20.  Back to cited text no. 6
    
7.
Malik P, Choudhry DK. Larsen syndrome and its anesthetic considerations. Pediatric Anaesthesia 2002;12:632-6.  Back to cited text no. 7
    
8.
Stevenson GW, Hall SC, Palmieri J. Anesthetic considerations for patients with Larsen's syndrome. Anesthesiology 1991;75:142-4.  Back to cited text no. 8
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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