Anaesthesia for adult patients with Down syndrome
Shalini Subramanian, Poornima Shivanna
Narayana Nethralaya NN-2, NH Health city, Bommasandra, Bangalore 560099
In the last six months we had four adult patients with Down syndrome (DS) coming for ocular surgery. The average life expectancy of individuals with DS is now 60 years. Dental, ENT and ocular problems requiring surgical intervention have a greater prevalence in this population. Hence it is appropriate to understand the anaesthetic concerns in these patients.
The first one was 51 years old, diagnosed with DS in early childhood. She had bilateral mature cataract. She was otherwise a cheerful person, co-operated with our examination and blood tests. She was however obstinate in refusing an echocardiogram and refused to lie down for an A- scan. She was able to carry out her usual activities of daily life (ADL) and even attended dance classes. Her cataract was identified after prolonged investigation for her recent increase in stubbornness and other behaviourial changes. She weighed 57 kg, was a snorer and preferred sleeping in the prone position. Her room air SpO2 was 98%. She had a short, thick neck, receding mandible, mouth opening > 3cm and Mallampati score of Class 3. She was euthyroid. At the request of her parents, both the eyes were operated simultaneously
On the morning of surgery, we secured an intravenous line after applying EMLA cream. Anaesthesia was induced with midazolam 1 mg, Fentanyl 60 µg, Propofol 50 mg. After ensuring adequate mask ventilation (required an oropharyngeal airway), neuromuscular blockade was achieved with atracurium 20 mg. Airway was secured with an Ambu laryngeal mask airway (Ambu LMA) (Size 3) in the first attempt. Her blood pressure (BP) dropped to 70/40 mmHg post induction and this was managed by administering mephentermine 6 mg intravenously.. She was given a bilateral peribulbar block under general anaesthesia (GA) for post-operative analgesia. The surgery lasted 30 minutes and she was extubated awake. There was no delay in awakening. She was quite cryful in the post-operative period but had enough clarity of consciousness to say that it was the eye plaster that was troubling her. She was shifted to the ward in a fast track manner to allow her to be in the company of her family members. She continued to remain adamant but finally settled down, smiled and even thanked us once the eye plaster was removed two hours later. She was discharged home the same day. Her parents later noticed a marked improvement in her temperament once her vision improved.
The second patient was 36 years old and required a C3 F8 compression suture for acute hydrops of the cornea secondary to pellucidal degeneration. She had already undergone a similar procedure under GA 5 years earlier for the other eye. A review of her previous anesthesia records showed that it had been uneventful. She had a history suggestive of obstructive sleep apnea (OSA) with room air SpO2 of 93% and was a known hypothyroid on thyroxine therapy. Her effort tolerance was good and no echocardiogram was done. She also had a short neck, retrognathia and Mallampati class 3 score. She had an intravenous induction with Fentanyl 100 µg, propofol 60 mg and atracurium 20 mg. Airway was secured with as supraglottic device (I-gel #3). The procedure lasted 40 minutes and she was extubated awake. The post-operative period was uneventful.
The third patient was a 35 year old male with bilateral mature cataract. He had been diagnosed with Down’s syndrome at birth. He was employed in a unit that helped special children lead a socially productive life. The diminution in vision was identified when he was unable to assemble nuts and bolts at work. He was euthyroid, a snorer and had grade 1 aortic regurgitation with good ejection fraction (61%) on echocardiogram. He had already undergone three dental restorative procedures under general anaesthesia. He was extremely co-operative. We did an intravenous induction for him and secured airway with an Ambu LMA#4. He received a Sub-Tenon’s block for post-operative analgesia. The anaesthesia and recovery were uneventful. The second eye was operated for cataract two weeks later and the same anaesthesia technique was followed.
The fourth patient was 45 years old and also had bilateral mature cataract. He also had bilateral nasolacrimal duct obstruction. Hence bilateral dacryocystectomy was done under GA and then bilateral cataract surgery a month later. His thyroid functions and echocardiogram were normal. He had an intravenous induction with slight hypotension after induction (BP 74/50 mmHg) requiring fluid resuscitation. He also required an OPA for mask ventilation and two attempts at inserting the Ambu LMA #3. Recovery was uneventful and he was discharged home the same evening.
None of them had a formal IQ assessment. They had impairment of cognition and adaptive /functional skills to call them Intellectually Disabled (ID). [DSM 5]
There are certain clinical conditions that occur more commonly in DS. These include:
- Behaviourial disorders:
Mental illness may affect 30% of DS. Depression and Alzheimer’s may both present as behaviourial changes, memory loss, sleep disorder or incontinence. The caregivers or parents are the best persons to say if the post-operative alertness and responsiveness are the same as baseline for that person. Hearing loss is quite common in DS (70%). They also have a better sense of understanding than expressing as they lag behind in speech and language development. We need to be patient and allow time for their reply. Self talk is common and is not generally considered hallucinatory.
They sometimes are wary of accepting certain new sensations like BP cuff or saturation probes, requiring patience and reassurance. These patients are able to perceive anxiety and agitation among caregivers and may react to them with hostility. It is useful to establish confidence and encourage family members to remain calm and positive.
In cases of limited communication, the family members may be helpful in identifying the expressions or reactions to pain or discomfort of the patient. It would be greatly beneficial to use regional techniques for post-operative analgesia.
- Cardiovascular disease:
They may have been operated in childhood for congenital heart disease and may have residual defect/ conduction defects after cardiac surgery. Adults without CHD may also develop mitral valve prolapse (57%) or valvular regurgitation (17%). An echocardiogram may be useful if the effort tolerance is poor but may not always be possible without sedation. A clinical decision about its requirement needs to be taken.
- Obstructive sleep apnea (OSA): OSA is quite common and may affect 50 % of adults with DS. Macroglossia, oropharyngeal hypotonia, micrognathia, adenotonsillar hypertrophy and dysfunctional central ventilatory drive may all be contributory. It may manifest as snoring, unusual sleeping positions, daytime somnolence, re-appearance of napping in adults, apneic spells as well as irritability and other behaviourial changes. Untreated, it may even lead to pulmonary hypertension or cor pulmonale.
- Respiratory disease: Lower airway problems may be secondary to hypotonia, obesity, OSA, gastro-esophageal reflux disease (GERD), smaller airways, thick mucus and some degree of pulmonary hypoplasia.
- Atlantoaxial instability (AAI): Any symptom or sign of AAI including gait disturbance, neck pain, weakness, bladder /bowel disturbance, increased deep tendon reflexes or spasticity may prompt evaluation with cervical radiographs. If AAI is confirmed, neutral positioning of the neck is required throughout anesthesia and recovery.
- Endocrine disturbances: Hypothyroidism and diabetes are more prevalent in DS and need to be evaluated.
- GERD: It is quite common and may be suspected from symptoms like vomiting, heartburn, night time cough or aspiration pneumonia. GERD may contribute to reactive airway disease and wheezing.
- Difficult airway: Adults with DS may not have the typical facial features that are so characteristic of children with DS. These patients may have short neck, truncal obesity, cervical spine abnormalities, and subglottic stenosis. Macroglossia, hypoplasticity of the maxilla/mandible, palatal abnormalities, and mandibular protrusion are the usual airway abnormalities encountered in patients with DS. Tracheal stenosis may be present even in adults. A review of previous anaesthetic records is very useful, if available. Equipment to manage difficult mask ventilation as well as difficult intubation should be available before administering GA.
- Others: Patients with DS have weak immune function and are prone to infections. They also have poor thermoregulation and are prone to hypothermia.
- There are certain conditions that are less likely to affect DS. This includes asthma, coronary or peripheral vascular disease and hypertension.
- Catecholamine response to atropine: These patients show markedly increased (twice normal
or greater) sensitivity to the cardio accelerator effects of atropine. One needs to exercise caution in using this drug which might nevertheless be useful in view of its antisialagogue effect and vagolytic effect. The mydriatic response to ocular atropine is also exaggerated.
- The low central nervous system catecholamine levels may be responsible for lower MAC value of inhalational agents and lesser requirement of anaesthetic drugs. Bradycardia and hypotension are not uncommon after anaesthetic induction and may be due to the low circulating catecholamine levels. This may occur across all ages including adults.
- Difficult intravenous access: This may be due to obesity or xerodermia. We used the EMLA cream to secure intravenous lines in all the patients.
- Consent: Consideration should be given to assessing the mental capacity to give consent in adults with DS. In three of our patients, the parents were the ones to give consent. In the fourth patient (case 4), consent was obtained from the caregivers (relatives).
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