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Combined Spinal–Epidural Anesthesia With Dexmedetomidine-Based Sedation for Multiple Corrective Osteotomies in a Child With Osteogenesis Imperfecta Type III: A Case Report

Gupta, Anju MD*; Kamal, Geeta MD*; Gupta, Nishkarsh MD; Aggarwal, Anil MS

doi: 10.1213/XAA.0000000000000527
Case Reports: Case Report
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Osteogenesis imperfecta (OI) is a rare disabling genetic connective tissue disorder. General anesthesia in these patients is associated with increased risks. Regional anesthesia is favored wherever feasible, but there are limited reports of use of a sole regional technique in OI in pediatric patients. Moreover, combined spinal–epidural anesthesia has never been described previously. We are reporting the use of combined spinal–epidural anesthesia for a prolonged surgery (multiple osteotomies) of lower limbs in a 10-year-old wheelchair-bound child with OI type III. Preoperative counseling, ultrasonography guidance, titrated local anesthetic dosage, and dexmedetomidine sedation helped establish optimum surgical conditions.

From the *Department of Anesthesiology, Chacha Nehru Bal Chikitsalya, Geeta Colony, New Delhi, India; Department of Onco-Anesthesiology and Pain Medicine, DRBRAIRCH, AIIMS, New Delhi, India; and Department of Orthopedics, Chacha Nehru Bal Chikitsalya, Geeta Colony, New Delhi, India.

Accepted for publication January 23, 2017.

Funding: None.

The authors declare no conflicts of interest.

Address correspondence to Anju Gupta, MD, Department of Anesthesiology, Chacha Nehru Bal Chikitsalya, Geeta Colony, 437 Pocket A, Sarita Vihar, New Delhi 110076, India. Address e-mail to dranjugupta2009@rediffmail.com.

Osteogenesis imperfecta (OI; brittle bone disease) is a rare hereditary disorder of collagen production and manifests in approximately 1 in every 20,000 births.1,2 It is characterized by skeletal deformities, teeth anomalies, hearing disorder, blue sclera, macrocephaly, kyphoscoliosis, thrombocyte function disorder, respiratory dysfunction resulting from chest and spine deformity, metabolic disorders, and short stature.2,3

It has been classified into 4 types, of which type III is the most severe phenotype in children beyond the neonatal period and leads to extreme short stature with progressive bone deformities.1 Such patients generally require multiple orthopedic surgeries for management of bone fractures. They are believed to be especially susceptible to hyperpyrexia episodes under general anesthesia (GA).2 Anesthetic risk is further increased by positioning related injuries, a potentially difficult airway (DA), risk of atlantoaxial dislocation, basilar invagination, and mandibular or cervical fracture during airway manipulation for intubation.2–4 After weighing all the pros and cons, we decided in favor of regional anesthesia. We report for the first time the use of ultrasound (US)-guided combined spinal epidural (CSE) anesthesia with dexmedetomidine (DXM) sedation in a patient with OI type III posted for a prolonged lower limb surgery.

The patient’s mother reviewed the case report and gave written permission for the authors to publish the report.

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CASE REPORT

A 12.4-kg, 10-year-old short-statured girl (height: 72 cm), diagnosed case of OI type III, was posted for corrective osteotomies of bilateral lower limbs. She had a history of multiple recurrent fractures (since age 2.5 years), was wheelchair-bound, and could walk only with the assistance of her hands. She was managed conservatively for fractures and was receiving injection pamidronate, oral calcium, and vitamin D supplements. There was no history of mental retardation or previous anesthesia exposure. On examination, she had triangular facies with macrocephaly, multiple skeletal deformities of upper and lower limbs, thoracic kyphoscoliosis, and pectus carinatum (Figure 1A). Radiographs of upper and lower limbs showed multiple malunited old fractures, whereas chest radiography showed severe kyphosis in the upper thoracic spine and scoliosis in the lower thoracic spine with rib crowding on the right side (Figure 1B). She was able to lie comfortably in the supine position and had 98% oxygen saturation on room air. Echocardiography ruled out cardiac abnormalities, but a pulmonary function test revealed moderate restrictive lung disease. Preoperative investigations including platelet count and coagulation profile were also within normal limits.

Figure 1.

Figure 1.

Figure 2.

Figure 2.

Airway examination revealed a mouth opening of 3.2 cm, modified Mallampati class III, a short neck, limited neck mobility, and irregular dentition. She and her mother were counseled regarding regional anesthesia and informed high-risk consent and assent were obtained. EMLA cream was applied 60 minutes before at the IV line, arterial cannulation, and over 3 lumbar intervertebral spaces for CSE (cumulative total dose <10 g). In the operating room, after careful transfer and positioning, routine monitors were attached. DA cart inclusive of a McCoy laryngoscope, Truview Picture Capture Device Videolaryngoscope (Truphatek Int, Netanya, Israel) and various laryngeal mask airway (classic and Ambu® AuraOnce™, Baltorpbakken, Ballerup, Denmark) were kept ready according to the age of the child (rather than her body weight). A 20-G IV line and an arterial line (US-guided) were inserted on the first attempt. Thereafter, a scout scan of the lumbar spine was performed using a 2- to 5-MHz 60-mm curved array probe (Fujifilm Sonosite, Inc, Bothell, WA) in the left lateral position. The midline depth of the epidural space and the inclination angle (100˚–105˚) were determined at intervertebral spaces (L2–3, L3–4, and L4–5) with the aid of a sterile protractor (Figure 2). Under all aseptic precautions, a 19-G (5 cm) Tuohy needle (Portex; Smiths Medical, Olomoucka, Hranice, Czech Republic) was introduced at the L3–4 interspace and the epidural space was identified at 3.0 cm using a loss of resistance technique. Thereafter, a 20-G epidural catheter was inserted and fixed at 7 cm. After this, subarachnoid block was performed at the L4–5 interspace with 1 mL 0.5% hyperbaric bupivacaine and 10 μg fentanyl (total volume 1.2 mL) injected over 30 seconds. The maximal sensory level of T6 was achieved at 20 minutes. DXM infusion was started and titrated (3-μg bolus over 15 minutes followed by infusion of 6 μg/h) to achieve a Ramsay sedation score of 2 to 3. When the level of sensory block receded below T10 (after 90 minutes), an epidural test dose (1.2 mL of 1% lidocaine with 1: 200,000 epinephrine) was injected. After confirming a negative response, a bolus dose of 4 mL of 0.5% plain bupivacaine was administered in an incremental fashion. There was transient hypotension resulting from increased blood loss (total 300 mL) near the end of surgery, which responded to a bolus of lactated Ringer’s solution and 180 mL of packed red blood cells. The surgery lasted 3 hours 40 minutes, and the child was transported to the intensive care unit for further monitoring. The postoperative period was uneventful and the child was transferred from the intensive care unit the next morning and discharged home on postoperative day 3.

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DISCUSSION

We have described the successful anesthetic management of a child having OI type III posted for bilateral corrective osteotomies under CSE. It is characterized by blue sclera at birth (which normalize during childhood), multiple fractures, and progressive bone deformities.1,2 The anesthetic management in such a patient is challenging (Table).

Table.

Table.

Our patient was extremely short-statured, wheelchair-bound, and had multiple skeletal deformities (Figure 1, A and B). These patients have increased fragility of bones and are prone to easy fractures during transfer, positioning, and continuous noninvasive blood pressure measurement.2,3 Because our patient was awake during the positioning for central neuraxial block and surgery, such complications were avoided. Invasive blood pressure monitoring and Esmarch bandage were chosen instead of a pneumatic cuff to avoid the risk of fractures.

The child had an anticipated DA as a result of macroglossia, short neck, limited neck mobility, and thoracic kyphoscoliosis. Also, laryngoscopy in such patients may increase the risk of odontoaxial dislocation in addition to cervical or mandibular fractures.2,3 Moreover, the small stature of the patient made the equipment selection difficult. The endotracheal tube size was determined by the size of the patient’s nostril instead of her age.

It has been reported that patients with OI have an increased incidence of hypermetabolic state (metabolic acidosis and hyperthermia) under GA.4–6 This was previously mistaken as malignant hyperthermia (MH).4,6,7 However, most such events were self-limited, not associated with hypercarbia or muscle rigidity, and there was no conclusive muscle biopsy proof suggestive of MH.7–9 Hence, these episodes are now believed to be the result of a disorder of central thermoregulation rather than MH.7–9 Nonetheless, as a result of the rarity of OI and lack of randomized trials, literature remains equivocal on the safe GA technique. So, to avoid triggering agents, total IV anesthesia (TIVA) has been preferred.2–5

In one report of TIVA in a child with OI, lactic acidosis developed after a short-term (150 minutes) propofol infusion.10 Propofol infusion syndrome is a highly fatal complication of propofol infusion, and several reports have triggered debate over its safety for children.11 Keeping all these considerations in mind, we were wary of choosing GA and endotracheal intubation.

Central neuraxial blocks have occasionally been used in adults with OI.12,13 In previous large case series, TIVA/inhalational techniques were used in all children with OI and none described use of regional anesthesia.4,5 There is only 1 recent report mentioning the use of epidural anesthesia for short (40 minutes) femur surgery in a 11-year-old child with OI type I (least severe phenotype).14 In this case, the epidural catheter was inserted after inhalational induction, thus counterfeiting many of its advantages.

These patients may have coagulation abnormalities as a result of increased capillary fragility, decreased levels of factor VIII, and deficient platelet aggregation.2,3 In our case, the child was extremely cooperative, could lie supine, was posted for a lower limb surgery, and had no demonstrable coagulopathy, so a central neuraxial block was a feasible option. We chose CSE to gain advantage of faster onset dense subarachnoid block and flexibility of epidural anesthesia. Peripheral nerve block, although possibly safer, was not selected because it was a bilateral procedure and an local anesthetic dose would have exceeded the safe limits. Also, hip and limb deformities would have made the procedure extremely challenging. There was minimal blood loss until the last osteotomy on the femur when sudden increased blood loss was observed and packed red blood cells were transfused.

US guidance has established its role in facilitating placement of central neuraxial block in anticipated difficult procedures.15

Preprocedural US of the spine is invaluable in these patients to delineate relevant anatomy (determining epidural depth, midline, adequate windows for procedure, and inclination angle) and hence reduce the puncture attempts and increase success rate.15 In our case also, US guidance helped ensure atraumatic CSE with minimal puncture attempts. Accounting for her short stature, the spinal dose was accordingly reduced and an epidural dose injected in an incremental fashion (after the spinal effect receded) to achieve desired sensory block without undesirable side effects.

In this case after instituting CSE, a DXM infusion was started. The fact that the child remained hemodynamically stable and drowsy but easily arousable throughout the surgery affirms its safety and utility in our case.

Preoperative counseling, application of EMLA cream before IV, arterial line, and epidural placement, and DXM sedation minimized discomfort and ensured smooth conduct of the surgery.

In conclusion, anesthetic management of patients with severe OI can be challenging. With our experience, we conclude that after careful preoperative preparation, US-guided CSE may be considered as a safe and effective approach for relevant surgery in such children.

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DISCLOSURES

Name: Anju Gupta, MD.

Contribution: This author helped care for the patient, acquire data, and write and edit the manuscript.

Name: Geeta Kamal, MD.

Contribution: This author helped acquire the data.

Name: Nishkarsh Gupta, MD.

Contribution: This author helped write and edit the mansucript.

Name: Anil Aggarwal, MD.

Contribution: This author helped care for the patient and acquire data.

This manuscript was handled by: Mark C. Phillips, MD.

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REFERENCES

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