Arthrogryposis multiplex congenita: An anesthetic challenge

Abstract

Children with arthrogryposis multiplex congenita often require multiple orthopedic corrective procedures. We present a case of a child with arthrogryposis multiplex congenita posted for contracture release of both lower limbs that were successfully managed with total intravenous anesthesia and caudal epidural analgesia with Bupernorphine as an additive.

INTRODUCTION

Arthrogryposis multiplex congenita (AMC) is a rare condition characterized by multiple joint contractures found throughout the body at birth.[1] AMC may be associated with multiple congenital anomalies.[2] Patients of AMC require anesthesia most often to correct orthopedic deformities and for muscle biopsy for definitive diagnosis. AMC is a challenge to the anesthesiologist as it is associated with multiple anesthetic implications.

The following is a case report of successful management of a 10-year-old girl with AMC posted for orthopedic corrective surgery.

CASE REPORT

A 10-year-old girl with AMC presented for release of ankle and knee contractures of both lower limbs. The parents were well informed about the disease and had been fully worked up elsewhere. The most significant problems were lower limb and upper limb deformities. She was confined to the wheel chair and had to be mobilized by her parents.

Her past history included two orthopedic surgeries done at the ages of 2 and 4 years, the anesthetic details were unavailable. The patient required post-op ventilation after both the surgeries for a day.

On examination patient had a mallampati grade-3 airway. Her weight was 25 kgs and securing a good IV access seemed difficult.

After discussion of all the possible options it was decided to manage the case with total intravenous anesthesia (TIVA) with caudal epidural analgesia.

The patient was premedicated with syrup Promethazine 125 mg on the night and morning of surgery. The routine monitoring was instituted and a 22 G IV cannula was inserted in the left forearm with difficulty. The patient was given inj Midazolam 1 mg IV for smooth separation from the parents. The difficult airway cart was kept ready, patient was induced with inj Fentanyl 50 mcg, inj Propofol 50 mg iv and after ensuring smooth mask ventilation inj Rocuronium 25 mg iv was given. Direct laryngoscopy done with MAC 2 blade, Lehane and Cormack grade-3 view was seen, which improved to grade-2 view with backward and downward pressure on the larynx. The child was intubated with a six cuffed endotracheal tube.

Anesthesia was maintained with an infusion of inj Propofol started at 5 mg/Kg/hr to achieve a BIS level of 50–60. She was ventilated with 33% oxygen and 66% nitrous oxide mixture with ventilation to maintain end tidal carbon dioxide between 30–33%. The caudal epidural space was identified with a 23 G hypodermic needle and 20 mL of 0.20% Bupivacaine and 30 mcg of Bupernorphine.

The left external jugular vein was cannulated with 18 G IV cannula. The vitals were stable throughout the 3 hr procedure. The Propofol infusion was reduced to 3 mg/Kg/ hr 20 min after of the start of surgery. The total additional inj Rocuronium used was 15 mg. Inj Paracetamol 500 mg IV was infused. A convection warmer was used through out surgery and the skin temperature was between 34–36°C.

The limbs were operated upon sequentially and tourniquet used for both. The Propofol infusion was tapered and stopped at the last suture. The total duration of the surgery was three hours. The neuromuscular blockade was reversed with inj Neostigmine 1.25 mg and inj Glycopyrollate 0.3 mg and extubated after full reversal of neuromuscular blockade.

The patient was comfortable and had no pain she was monitored in the recovery room for an hour and was then sent back to her room. The patient did not require any additional analgesics for the next 8 hr. NSAIDs were used for postoperative analgesia. The child had an uneventful postoperative course and discharged after 2 days.

DISCUSSION

Patients with AMC may come for multiple surgeries most often for correction of orthopedic deformities. The challenges faced by the anesthesiologist are as follows.[1]

1. Difficult airway – micrognathia, high arched palate, cervical spine instability (under development of first and second cervical vertebra).[2]

2. Malignant hyperthermia (MH) – avoid triggering agents such as Succinyl choline and inhalational anesthetic agents (some variants have been associated with MH).[3]

3. Myopathy – increased sensitivity and prolonged duration of neuromuscular blockers.

4. Cardio-respiratory – increased sensitivity to induction agents. They may have a high risk of postoperative respiratory depression and are prone to aspiration.

5. Difficult regional blockade – spine anomalies, difficult access to nerves due to joint contractures.[4]

6. Difficult IV access – reduced subcutaneous tissue and tense skin.

7. Difficult positioning – joint contractures and reduced muscle mass.

In our patient we did not anticipate a difficult airway, but we had to use a muscle relaxant and intubate the patient as the surgeon wanted to position the patient prone or lateral if he had difficulty during the surgery. But the surgeon could complete the surgery in supine position. We encountered difficult IV access and had to cannulate the external jugular vein. We avoided triggering agents for MH[3,5] and we successfully managed the case using TIVA with propofol and caudal epidural analgesia.[6] Ketamine infusion has been used for management of these patients, we chose propofol for better cardiovascular stability.[7]

The use of regional anesthesia in these patients is controversial as one of the etiologies of AMC is a self-limited anterior horn cell disease and may be technically difficult due to anatomical abnormalities. Caudal analgesia has been successfully used for the management of postoperative pain in Freeman Sheldon syndrome a variant of AMC.[8] Regional anesthesia in the form of Fascia iliaca block has been successfully used for muscle biopsy in patients with AMC without any neurological sequale.[9] We have used a caudal analgesia for supplementing TIVA, thus reducing the requirement of Propofol, muscle relaxants and opiates. We used Bupernorphine as an additive to prolong post operative analgesia; our patient was pain free for 8 hr without any complications. Intrathecal morphine has been used for the management of post operative pain in patients with AMC. We chose Bupernorphine over Morphine as the duration of action is longer and without any serious side effects.[10] We could extubate the child and she did not require post operative ventilation or ICU care.

In conclusion, AMC can present many challenges for anesthetist especially in children. The combination of TIVA and caudal epidural analgesia with Bupernorphine appears to be a suitable anesthetic technique for patients with AMC. This technique provided good conditions for rapid recovery from anesthesia with good postoperative analgesia.