Papillary thyroid cancer (PTC) is the most common type of well differentiated thyroid cancer. Thyroid cancer affects over 300 individuals in New Zealand every year with an incidence of 5.2 per 100,000 population.[[1]] Papillary thyroid cancer is staged according to the American Joint Committee on Cancer, which will classify the extent of disease at diagnosis, guides treatment, and which informs prognosis.[[2]] With appropriate treatment, five-year survival for localised PTC or PTC with only regional nodal spread is around 99%.[[3]] The American Thyroid Association recommends total thyroidectomy and adjuvant radioactive iodine in the setting of bulky nodal metastatic disease and the British Thyroid Association also similarly advocates for a total thyroidectomy.[[4–5]] However, it may not always be possible to follow these treatment guidelines.

We present the unique case of a young man with severe Autistic Spectrum Disorder (ASD) who presented with Stage I PTC with bulky nodal disease. While generally, advanced papillary thyroid cancer with nodal metastases can be effectively treated and cured—particularly in young patients—cases that are compounded by co-morbid severe intellectual disability can require departure from gold standard treatment. There is little published on this subject to date.

We explore the multiple challenges around managing this unique case and the inventive, flexible approaches that were required to provide the best outcome for the patient. This case provides several useful learning points to help inform future clinical management of young cancer patients with co-morbid severe intellectual impairment.

A 25-year-old male with severe ASD was referred to the Department of Otolaryngology, Head and Neck surgery at Waikato Hospital for a right-sided level V neck mass. This mass had been noted by carers for approximately six months prior to presentation and it was felt that it was increasing in size. There were no obvious associated aerodigestive, systemic or constitutional symptoms.

His medical background included severe ASD, which meant that he was non-verbal and employed only very basic communication techniques. He lived in a permanently staffed residential care facility. Functionally, he mobilised independently in the care facility and generally was directable in following commands. He was non-compliant with medical examination. His size and weight added further complexity to his perioperative management. His weight and height were 86kg and 188cm, respectively, meaning that patient transfer and restraint if necessary was considerably more challenging.

Due to the severity of his ASD, his first clinical assessment had to be performed at the entrance of the hospital due to his reluctance to come inside the hospital. Physical examination was a very brief palpation of the neck mass which felt solid, measuring approximately 3cm by 4cm and corresponded to the right level V region. The complete history, although limited, was obtained from his main carer. A decision was made to complete further comprehensive assessment under general anaesthesia (GA). This required considerable coordination with the surgical and the anaesthetic team, and to ensure the availability of an ultrasound (US) machine, radiologist, and pathologist. Under GA, an US scan of the neck by the surgical team revealed multiple abnormally enlarged right sided lymph nodes in levels III–V and a suspicious, hyperechoic ill-defined right thyroid nodule with microcalcifications. A comprehensive oral and panendoscopic examination was unremarkable. A fine needle aspirate (FNA) of the thyroid nodule and a core biopsy of the abnormal lymph nodes was completed, which was examined in theatre and reported as abnormal cells consistent with a neoplastic process. The patient was then taken intubated to the radiology department for a computed tomography (CT) scan under GA.

Figure 1: A) Coronal CT slice demonstrating right neck lymphadenopathy with cystic component (u) and thyroid nodule (→) in the superior aspect of the right thyroid lobe. B) Axial CT slice of the right thyroid lobe nodule (→). C) Axial CT slice demonstrating right neck lymphadenopathy with evidence of central necrosis and scattered calcification (u).

This confirmed multiple right sided pathologic lymph nodes—many of which were cystic—involving levels II–V and level VI, complete internal jugular vein effacement, and sparing of the common carotid artery and trachea. He also had a number of blood tests while under GA, which revealed normal thyroid function tests.

Histological analysis was consistent with metastatic papillary thyroid carcinoma (PTC), radiologically staged as T1b N1b M0.

Extensive multidisciplinary team (MDT) discussions included the family; carers; local head and neck cancer MDT; members of the surgical, anaesthetic, palliative care, radiation oncology, and intensive care teams; a medical physicist; and a clinical nurse specialist. The challenges with radioactive iodine (RAI / I[[131]]) included a lack of confidence that the patient would swallow the capsule or liquid I[[131]]. Spitting of the I[[131]] if swallow was attempted or with endoscopic placement of I[[131]] would lead to likely exposure of staff members. The challenges of isolation for radiation protection in the immediate days after consumption were considered. Ultimately the risks of radiation exposure to public or staff were deemed too high. In addition, it became clear that the patient was likely to interfere with the surgical wound, dressings and drains, and there was a serious concern with the ability to nurse him in the immediate post-operative period. Non-compliance with blood tests and need for lifelong medication, especially in the setting of total thyroidectomy, were also explored.

Eventually, it was decided that a right hemithyroidectomy and right lateral (I–V) and central (VI–VII) neck dissection would be completed, level VII representing paratracheal fibro-adipose tissue below the level of the suprasternal notch. To manage the perioperative period a plan was devised, with collaboration of the intensive care unit (ICU), to keep him intubated for approximately seven days until his surgical drains could be removed and his wound had largely healed. An anaesthetic plan was also devised to minimise preoperative distress with a considered medication regiment with specific dosing. He received 40mg of Diazepam at home pre-operatively, and 800mg of Ketamine in the hospital transit lounge 25 minutes prior to the first observation in the operating theatre. He was then transferred from his car seat to a pink evacuation sheet to the operating theatre with monitoring on and gas induction was initiated once he arrived in theatre. The operation took place three weeks after this discussion, he remained in the ICU for eight days and for a further four days in the ward before being discharged home without complication. His surgical drain was removed while he was still intubated in ICU.

The main tumour was 23mm in maximum diameter with extension into but not beyond the extra-thyroid fat pad and there was 0.02mm clearance at the closest resection margin; however, adjacent level VI tissue was excised as a separate specimen. Ten of 47 lymph nodes were positive for PTC, and the largest lymph node was 45mm in size. The final histological diagnosis confirmed pT2 N1b metastatic papillary thyroid carcinoma. His case was re-discussed post-operatively in the local head and neck cancer multidisciplinary meeting, with a consensus for him to undergo surveillance neck US assessment under GA every six months. The total duration of surveillance is still to be decided.

It has now been 12 months since his surgery, and he has remained well without any evidence of disease recurrence on post-operative follow-up, with clinical and ultrasonographic examination and blood tests including thyroglobulin tumour markers under GA.

Discussion

This case highlights the unique challenges of managing cancer in individuals with severe intellectual impairment such as with severe ASD. While the gold standard therapy in this case would have been total thyroidectomy, neck dissection, and RAI,[[6]] multiple issues beyond oncological standard of care had to be considered.

Verbal communication can be a significant problem for patients with severe ASD, with nearly 80% being unable to express their needs verbally.[[7]] Interacting with the healthcare setting can predictably provoke anxiety for an individual with ASD, and this can often be severe.[[8]] The combination of anxiety, difficulty with communication, and not being able to understand and fully participate in their own health problems necessitate extensive multidisciplinary team input. The unique challenges in this case demanded an inventive approach to manage the post-operative period. This ultimately led to the joint decision to keep him intubated in the ICU until the initial wound healing was complete and drains were removed.

Some of the challenges in this case, such as non-compliance with RAI and radiation risk to others, were unavoidable and have been previously documented in similar cases.[[9–10]] In this case, challenges that were explored included whether RAI therapy would be completed in hospital or in residential care; the risk of chewing and spitting out the RAI tablet; and exposing healthcare workers, carers or co-residents to radiation. Involvement of the medical physicists helped guide decision making in this area. Patient self-care while isolating such as feeding, washing and dressing were important issues that also had to be explored, as well as the psychological distress of change to routine and isolation.

However, other issues can be managed and optimised. A systematic review of perioperative management of paediatric surgery patients with comorbid ASD identified three important themes. These were:

• collaborating with the caregiver;

• establishing a system to communicate to the perioperative staff the information gathered from the caregivers;

• adopting an individualised approach to managing the perioperative environment based on the information gathered from the caregivers.[[11]]

These recommendations should be considered for all patients with ASD presenting to the hospital for surgical pathology. Additionally, we would advocate for institutions to establish guidelines to help manage surgical patients with ASD with attention to the unique demographic needs of their patient population. In New Zealand, for example, additional effort should be made to deliver culturally appropriate care to Māori, as well as the multitude of other cultures within New Zealand.

While conventional gold standard treatment guidelines should be considered and adhered to where possible, these should be customised in light of the unique challenges that some patients with ASD present with. In this case, these included concerns regarding ability to adhere to lifelong requirement for medication, non-compliance with regular blood tests, and risk of radioactive harm to both patient and others. Risk of radioactive harm due to non-adherence to strict self-isolation during RAI treatment is a unique consideration for thyroid cancer management.[[9–10]] This further necessitated deviation from conventional treatment in this case. The key lessons learned from this case are the importance of being patient- and caregiver-centric, being inventive, and having regular MDT involvement and a willingness to deviate from conventional treatment guidelines when managing surgical pathology in patients with ASD.