Introduction
Tuberous sclerosis (TS) or Bourneville disease is a rare
autosomal dominant neurocutaneous disorder with an overall
prevalence of 1 in 29,000 [1]. TS usually manifests as a triad
of seizures, mental retardation and adenoma sebaceum, but may
also involve other organs such as kidney, heart, lung and
brain [2]. It offers a significant challenge for
anaesthesiologists because of variable clinical presentation
and multi organ involvement. A thorough pre operative
evaluation to assess the extent of involvement of various
organs and careful intraoperative management and post
operative monitoring are crucial for safe management of such
patients. We report the successful anaesthetic management of a
case of TS for laparoscopic myomectomy with cystectomy under
general anaesthesia.
Case Report
A young female, suffering from TS, weighing 60 kg
presented with complaints of bleeding per vagina for the past
1 year. She was mentally retarded. She had seizures from
childhood, on regular multiple antiepileptics (Lacosamide,
Carbamazepine, Clobazam, Sodium Valproate) and the last
episode was 1 month back. She had undergone right partial
upper polar nephrectomy due to angiomyolipoma 4 years back
under general anaesthesia with epidural and she developed
seizure postoperatively and was managed in the ICU.
Physical examination revealed adenoma sebaceum in the face and
large adenoma on the occiput which interfered with lying
supine. Airway examination was found to be normal except
limited extension of the neck. Laboratory investigations
revealed haemoglobin of 11.0 g% and all other parameters
including renal function tests were within normal
limits. Echocardiography showed normal left ventricular
function. Contrast enhanced computed tomography (CECT) abdomen
showed multiple angiomyolipoma in both kidneys, bulky uterus
with multiple fibroids and endometriotic cyst in left ovary.
High risk consent was taken, Patient was advised to continue
all antiepileptics and given injection Levetiracetam 1.5 gm on
previous day and the morning before surgery as per
neurologist's advice. On arrival at the operation theatre,
after attaching monitors, premedicated with injection
midazolam 1 mg, injection fentanyl 120 microgram and
anaesthesia was induced with injection propofol 120 mg
intravenously (IV). Injection vecuronium 4 mg IV facilitated
tracheal intubation without any difficulty and was followed by
maintenance with air and oxygen with 1-2% sevoflurane and
injection paracetamol and fentanyl was used for intraoperative
analgesia
Throughout the intraoperative period, haemodynamic and
ventilatory parameters remained stable. Intramural vasopressin
was given by the surgeon and blood pressure was stabilised
with small boluses of injection nitroglycerine. Laparoscopic
myomectomy with cystectomy was done with minimal blood loss.
At the end of surgery, injection ondansetron 4mg was given.
Anaesthesia was reversed with injection sugammadex 120 mg and
extubated and shifted to post anaesthesia care unit (PACU) for
monitoring. Postoperative analgesia was given with fentanyl
infusion and buprenorphine patch (5mcg/hr). After 1 hour,
patient developed seizure in the PACU and was managed with
injection midazolam 2 mg and injection levetiracetam 1 gm IV.
Postoperative haemoglobin, blood sugar, and serum electrolytes
were normal. She was discharged on the 3rd post
operative day.
Discussion
Tuberous sclerosis is an autosomal dominant genetic disorder
characterized by a wide spectrum of symptoms owing to
multisystem involvement [3]. Pre anaesthetic assessment of the
patient should focus on various abnormalities secondary to the
disease process involving the neurologic, pulmonary,
cardiovascular and renal system. Anaesthetic considerations in
these patients depend on the site, extent, and severity of the
disease and type of surgery
Neurologic involvement is characterized by sub ependymal
nodules, cortical tubers, and subependymal giant cell
astrocytoma [4]. Though seizures and mental retardation are
the most common symptoms encountered, approximately 50% of
patients have normal intellect [5]. This patient was mentally
retarded on multiple antiepileptics for seizure since child
hood. Patient was advised to continue antiepileptics and given
Inj levetiracetam 1.5 gm on previous day and morning of
surgery to avoid perioperative seizures. Most anti-epileptic
drugs have interactions with anaesthetic drugs through
induction or inhibition of cytochrome p450 iso enzyme in
hepatic metabolism. Typically, almost all anaesthetic agents
have both proconvulsant and anticonvulsant properties, and
lower doses of these agents are proconvulsant. We avoided
drugs which precipitate seizures and was ready with loaded IV
antiepileptics.
Cardiac signs and symptoms are characterised by congestive
cardiac failure, conduction abnormalities, refractory
arrhythmias, and severe hemodynamic compromise [6].
Preoperatively the patient was assessed with ECG, and echo
cardiogram and found to be normal. Patient was cautiously
monitored for conduction abnormalities and hemodynamic
derangements while administering vasopressin into myoma.
Renal lesions include angiomyolipoma, renal cysts, and renal
cell carcinoma [7]. Renal lesions can lead to renal failure
affecting drug pharmacokinetics. Preoperatively patient's
renal function tests, urine output, and blood pressure were
normal. Intraoperative management involves avoiding renal
insults and maintaining normovolemia and normotension to avoid
decreases in renal perfusion and cardiac output.
Pulmonary involvement is rare (1%) and patients may present
with severe and progressive dyspnoea, spontaneous
pneumothorax, haemoptysis, and respiratory failure [6].
We administered general anaesthesia with maintaining a low
tidal volume and low peak inspiratory pressure to avoid
pneumothorax due to barotrauma and nitrous oxide was avoided
to prevent rupture of lung cysts, even though our patient
didn't have pulmonary involvement.
Oral lesions such as nodular tumours and papilloma may be
present on the tongue, palate, pharynx and larynx [8]. Airway
management might be complicated by these lesions, and
alternatives to direct laryngoscopy should be taken into
consideration [9]. Our patient's airway examination was normal
apart from limited neck extension. We did direct laryngoscopy
with muscle relaxant as vecuronium without any difficulty
because of the adequacy of mask ventilation and availability
of sugammadex.
Various post operative complications such as seizures, severe
hypertension and bradyarrhythmias have been reported. Our
patient had one episode of postoperative seizure which was
managed with Inj levetiracetam and midazolam.
Conclusion
Understanding the involvement of disease in various organs and
knowledge of their anaesthetic implications is mandatory for
successful management of patients with TS.
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