A multidisciplinary approach to the management of temporomandibular joint ankylosis in a sickle-cell anemia patient in a resource-limited setting.

This report describes the multidisciplinary management of a 35-year-old female sickle-cell anemia patient who had unilateral bony ankylosis of the left temporomandibular joint secondary to septic arthritis. She was managed by a team comprising of maxillofacial surgeons, anesthetists, otorhinolaryngologist, and hematologist. Unilateral left interpositional arthroplasty and ipsilateral coronoidectomy through a postrami approach were done and followed by aggressive jaw physiotherapy in the postsurgical period. No perioperative morbidity was encountered. Mouth opening of 3.5 cm was achieved and maintained 7 months after surgery. Challenges and rationale for the use of a multidisciplinary team approach in treatment of such cases were discussed.

INTRODUCTION

Sickle-cell disease (SCD) is a collective term for a number of genetic disorders in which hemoglobin is structurally abnormal, resulting in the episodic formation of sickle-shaped red blood cells (RBCs) and a wide range of clinical manifestations. It is an autosomal-recessive disorder that produces hemolytic anemia related to abnormal hemoglobin and erythrocytes. Sickle-cell trait is common in Africa, the Middle East, the United States of America, the West Indies, Brazil, and around the Mediterranean Sea.[1]

The underlying abnormality is a single nucleotide substitution in the gene for β-globin on chromosome 11, resulting in the replacement of a glutamic acid residue with valine on the surface of the protein Haemoglobin S (termed HbS). The resulting polymers align into bundles, causing distortion of the RBC into a crescent or sickle shape and reducing flexibility and deformability, which impairs passage of the cells through narrow blood vessels.

SCD is a combination of sickle-cell gene with any other abnormal β-globin gene. Sickle-cell anemia Haemoglobin SS (HbSS) is the commonest and the most severe form of the SCD. Other SCDs include sickle Beta zero-thalassemia, Hemoglobin SC, Sickle beta plus-thalassemia. Sickling can be precipitated by environmental factors such as hypoxia, acidosis, cold, and dehydration, as well as adhesion molecules and cytokines associated with infections.

Septic arthritis in children with SCD ranges from 0.2% to 5.4% and SCD has been complicated by osteomyelitis, osteonecrosis, septic arthritis, and multiple ankylosis.[2] These conditions can affect all the joints in the body including the temporomandibular joint (TMJ). Despite the current advancement in patients’ care, the perioperative mortality rate in patients with SCD is estimated to be 1 in 100 and this is several times greater than that reported in nonsickle adults (approximately 1 in 300,000). Mortality rate in nonsickle children (1 in 50,000–80,000) is also much lower.[3] Patients with HbSS disease, in general, have lower hemoglobin levels and are at greater risk under general anesthesia (GA). Intermittent episodes of vascular occlusion cause tissue ischemia resulting in acute and chronic organ failure. As a result of this, HbSS patients require special considerations to minimize perioperative complications due to hemolysis and vaso-occlusion. The outcome of patients with HbSS requiring surgery can be improved by careful attention to the cardiorespiratory, hemodynamic, hydration, infection and nutritional status of the patient. This report highlights the various precautionary measures taken to reduce hemolysis, vaso-occlusive crises, and infection in sickle cell anemic patients undergoing complex surgeries like TMJ ankylosis under GA.

CASE REPORT

A 35-year-old, known HbSS patient presented in the Orthopaedic Department of the Ondo State Trauma and Surgical Centre, Ondo on May 12, 2014, with a 17-year history of inability to walk properly. Following examination and preliminary investigations, a diagnosis of total fusion of the hip joints, knee joints, and ankle joints was made. Because of inability to open the mouth, the orthopedic team referred her to the Maxillofacial Surgery Department of the same hospital. She presented in our department on September 9, 2014, with inability to open the mouth of 16 years duration. She gave a history of blood transfusion during one of her vaso-occlusive crises at about the age of 18 years after which she developed an infection in many joints including the left TMJ. The infection was managed with antibiotics, and she had a prolonged hospital stay. Two years later, she was unable to open her mouth properly. She presented at a private hospital where high condylectomy was done in the left TMJ. However, she failed to comply with an aggressive jaw exercise and had since been unable to open her jaws.

On examination, there was some transmitted movement in the right TMJ but with no movement at all in the left TMJ. The mouth opening (interincisal distance) was 1 mm [Figure 1].

Figure 1

Preoperative mouth opening

The scout cranial computed tomography (CT) showed an enlarged diploe [Figure 2a] confirming the characteristic radiographic evidence of sickle-cell and CT scan with three-dimensional reconstruction revealed complete fusion of the left condyle to the temporal bone with enlargement into the sigmoid notch [Figure 2b]. The axial CT shows an enlarged left condyle [Figure 2c].

Figure 2

(a) Posteroanterior view scout computed tomography showing enlarged diploe. (b) Three-dimensional computed tomography showing bony fusion of left temporomandibular joint. (c) Axial computed tomography section showing enlarged left condyle

A diagnosis of bony ankylosis of the left TMJ in a known HbSS patient with bilateral ankylosis of hip, knee, and ankle joints was made.

Work up for surgery

Maxillofacial surgeons

She was planned for interpositional arthroplasty of the left TMJ with/without bilateral coronoidectomy under GA.

Hematologists

Following hematologists review, the requested investigations included;

Leukocyte count: 13.1 × 103/mm3

Packed cell volume (PCV): 17%

Hemoglobin: 6.5 g/dL

Erythrocytes: 2.18 × 106/mm3

C-reactive protein: nonreactive (immuno-turbidimetric method)

PTTK: 30 s (Dade Behrings, Actin FSL kit)

PTTK control: 32 s (Dade Behrings, Actin FSL kit)

PT: 12 s (Dade Behrings, Thromborel S kit)

PT control: 10.95 s (Dade Behrings, Thromborel S kit)

INR: 1.10 (Dade Behrings, Thromborel S kit).

Based on the PCV, the hematologists requested that patient should be optimized with 1 unit of packed red cell 48 h before surgery and that provision be made for 2 units of fresh frozen plasma intraoperatively.

Anesthetists

The anesthetist planned for a fiberoptic nasotracheal intubation but advised on possible tracheostomy should that fail. Also based on the low PCV, the patient was preoperatively admitted into the Intensive Care Unit (ICU) where preoxygenation and optimization was done and she was planned for ICU care in the immediate postoperative period. The preoperative blood chemistry of the patient included;

Na+: 139.0 mmol/L

K+: 4.33 mmol/L

Cl−: 126.3 mmol/L

HCO3: 29.8 mmol/L

Urea: 4.55 mmol/L

Creatinine: 62.4 mmol/L.

Otorhinolaryngologist

The ear, nose and throat (ENT) team advised against fiberoptic nasotracheal intubation to avert the problem of a compromised airway postoperatively. It was then concluded that airway management should be by tracheostomy.

Surgical chronology

Patient was admitted to the ICU as suggested by the anesthetists and transfused with 1 unit of packed red cell as requested by the hematologist.

She was preoxygenated and intravenous (IV) line was inserted for medication delivery. Patient had tracheostomy under local anesthesia and sedation on the operating table by the ENT surgeons. She had left TMJ ankylosis release via a postrami incision [Figure 3a] by gap arthroplasty of 1 cm [Figure 3b]. About 1 cm of mouth opening was achieved, and ipsilateral coronoidectomy was performed to further improve the mouth opening. Following ipsilateral coronoidectomy, about 1.5 cm mouth opening was achieved intraoperatively. The gap was then interposed with a strip of the masseter muscle [Figure 3c]. Contralateral coronoidectomy was re-scheduled as a stage 2 surgery following the advice of the anesthetists that patient had stayed 2 h 15 min under GA. Estimated blood loss was 350 ml (70 ml from tracheostomy and 280 ml from surgery). She was transfused with another pint of whole blood at the end of surgery. Final tracheostomy tube removal was performed by the ENT team after reversal of GA. She was thereafter transferred to ICU where the following medications were administered:

Figure 3

(a) Postrami surgical incision. (b) Gap arthroplasty of about 1 cm. (c) Interposition of masseter muscle

IV cefuroxime 750 mg 8 hourly

IV metronidazole 500 mg 8 hourly

IV pethidine 20 mg 6 hourly

IV dexamethasone 8 mg start then 4 mg 8 hourly.

She was also placed on the following medications for immune optimization as suggested by the hamatologists:

Tablets immunace 12 hourly

Tablets maxomega 8 hourly

Tablet immunocal daily.

Active jaw exercise was commenced on the 1st postoperative day with 1.6 cm mouth opening. Tizanidine tablet (2 mg) was added to medication as muscle relaxant. She was transferred to regular ward from ICU on postoperative day 5. Mouth opening on postoperative day 5 was 2 cm. Patient was discharged home on postoperative day 6 on oral medications. Review appointment was 2 weeks and following review, mouth opening was 2.3 cm. At the 7 months review, mouth opening was 3.5 cm [Figure 4] and the patient was satisfied. Postoperative scar was minimal both from previous and recent surgeries [Figure 5].

Figure 4

Postoperative mouth opening

Figure 5

Postoperative scar

DISCUSSION

The treatment of TMJ ankylosis poses a significant challenge because of technical difficulties and high incidence of recurrence.[4] It becomes more challenging in patients with other co-morbidities like SCD. Patients with SCD have a high incidence of perioperative morbidity and mortality. Earlier studies[4567] of patients with SCD undergoing surgery noted perioperative morbidity and mortality >50%. Perioperative mortality has reduced steadily in concert with overall improvements in anesthetic care. Individuals with life-limiting illnesses often have complex and multifaceted needs. In most cases, these needs are best managed using a multidisciplinary approach to care that includes opportunities for multidisciplinary discussions and care planning.[8] Effective multidisciplinary care uses collaborative and patient-centred approaches to treatment planning and provision and leads to the achievement of goals that are unlikely to be achieved by health professionals acting in isolation.[8]

Benefits of a multidisciplinary approach to care include: increased patient perception that their care is being managed by a team, greater likelihood of the delivery of care in accordance with national standards and clinical practice guidelines, increased patient satisfaction with care, increased access to information, and practical support for patients. In our case, the specialists involved in the management included; oral and maxillofacial surgeons, anesthetists, otorhinolaryngologist, and hematologist.

The ankylosis in this patient could be a complication of septic arthritis as well as avascular necrosis. Multiple joints in her body were affected, and similar but few cases have been reported in the literature. Our patient had transfusion of a unit of packed red cells because of preoperative anemia. An aggressive transfusion model was not employed because HbSS patients are known to have adaptive mechanisms to tolerate anemia better than normal population. Aggressive transfusion for the purpose of dilution of sickle-cells has also not been shown to have benefited over a more conservative approach aimed at only increasing the hematocrit.[7] She maintained a postoperative PCV of 22% for 3 months after surgery and thereafter PCV fell to 19% 5 months after surgery.

Perioperative risks increase as an HbSS patient ages.[7] Accumulated effects of vasoocclusive crises compromise a number of organs including the lungs. Sickle cell chronic lung disease will eventually lead to lung fibrosis and restrictive lung defect in the later stages. A consequence of this will be right-sided cardiac hypertrophy and cor pulmonale at the end stage. This chronic condition may precipitate clustering of acute chest syndrome (ACS). Health of the lungs and ventilation are important for delivery of uneventful anesthesia, and these were effectively managed in this patient by the protocol used. A protocol for management of TMJ ankylosis advocated by Leonard et al.[9] includes early identification and surgical intervention, resection of ankylotic mass and creation of 1–1.5 cm gap, ipsilateral coronoidectomy, and mouth opening of 3.5 cm and if this gap is not achieved, contralateral coronoidectomy, lining of the articular surfaces with TMJ facscia/muscle, and aggressive physiotherapy. The temporalis muscle flap (TMF) through an Al-Kayat and Bramley[10] incision has been used as interpositional material for correcting TMJ ankylosis due to its simplicity of handling, closeness to the temporal joint, good functional results, successful clinical results, and least complications.[1112] However, the usefulness of the TMF technique in supplying interpositional material is not certain and failures may occur.[11] Postrami incision was utilized in our patient because it was a case of re-ankylosis with ankylotic mass extending to the ramus of the mandible. Furthermore, because the temporalis muscle has been used as interpositional material in the previous surgery, we decided to use masseter muscle. Temporalis muscle as interpositional material has been reported to fail due to contraction of the muscle postoperatively. Because of this reason, Balaji[13] has modified the TMF as interpositional material in TMJ ankylosis by submandibular anchorage. This modification will inhibit the contraction of the temporalis muscle postoperatively.[13] Although the gap created in our case was 1cm and the mouth opening achieved was 1.5 cm soon after ipsilateral coronoidectomy, we, however, achieved a postoperative mouth opening of 3.5 cm following 7 months aggressive physiotherapy using stacked wooden spatula. The choice of ICU for the immediate postoperative management of the patient was to monitor closely for the occurrence of any pulmonary complication that can progress to ACS. This was also necessary to deliver adequate postoperative care to a high-risk patient in a resource-scarce setting.

CONCLUSION

Multidisciplinary approach by teamwork in the management of TMJ ankylosis in a sickle-cell anemia patient led to a good outcome. The main advantage of multidisciplinary team care in our case was achieving the goal of functional efficiency, patient satisfaction, and improved quality of life.

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.