Muscular dystrophies are a group of
inherited disorders characterized by progressive muscle
weakness and wasting. Anaesthetic management of patients with
muscular dystrophy undergoing surgery presents unique
challenges due to the potential for respiratory compromise,
cardiac dysfunction, and susceptibility to malignant
hyperthermia. We present a case of a middle aged female with a
diagnosis of Becker muscular dystrophy who underwent
laparoscopic hysterectomy. We discuss the anaesthetic
considerations, including preoperative evaluation,
intraoperative management, and postoperative care,
highlighting the importance of a multidisciplinary approach.
Additionally, we provide a comprehensive review of the
literature on anaesthetic management of patients with muscular
dystrophy undergoing laparoscopic surgery.
Introduction
Muscular dystrophies are a heterogeneous
group of genetic disorders characterized by progressive
skeletal muscle weakness and degeneration. Global incidence
is approximately I in 5000 individuals [1]. Becker muscular
dystrophy (BMD) is an X-linked recessive disorder, a milder
variant of Duchenne muscular dystrophy, caused by mutations
in the dystrophin gene [2]. Patients with BMD may present
with varying degrees of muscle weakness, cardiomyopathy, and
respiratory dysfunction.
Laparoscopic surgery has become a standard approach for
various gynaecological procedures, including hysterectomy,
due to its advantages of reduced postoperative pain, shorter
hospital stay, and faster recovery [3]. However,
laparoscopic surgery in patients with muscular dystrophy
poses unique challenges for anaesthesiologists due to the
physiological changes associated with the disease, effect of
muscular dystrophy on other organs, rhabdomyolysis and
hyperkalaemic response under stressful conditions, and the
potential for complications related to pneumoperitoneum and
positioning.
This case report describes the anaesthetic management of a
patient with BMD undergoing laparoscopic hysterectomy. We
also provide a comprehensive review of the literature on
anaesthetic considerations for patients with muscular
dystrophy undergoing laparoscopic surgery, emphasizing the
importance of a multidisciplinary approach to ensure patient
safety and optimal outcomes.
Case Presentation
A middle aged woman, with history of progressive quadriparesis
for 25 years and a known case of Becker Muscular Dystrophy,
presented to the gynaecology department with complaints of
heavy menstrual bleeding and dysmenorrhoea. Her BMD was
characterized by flaccid quadriparesis (which started with the
lower limbs and progressed to upper limbs), wasting and
contractures. Her elder sister also has the same disease.
There is a history of paternal consanguinity. Neurological
examination revealed power of 1/5 in upper limbs, 0/5 in lower
limbs and 2/5 in neck flexors. Deep tendon reflexes were
absent. Pre-operative ECG showed sinus rhythm. A
2D-Echocardiogram was performed, which revealed normal left
ventricular function with an ejection fraction of 60% and no
evidence of cardiomyopathy. Pulmonary function tests (PFTs)
could not be done as the patient had difficulty in performing
the test. Routine blood tests, including coagulation profile,
were within normal limits. Abdominal ultrasound confirmed the
presence of multiple uterine fibroids, correlating with her
symptoms.
Anaesthetic Plan and Informed Consent Due to the high risks
associated with anaesthesia, including the possibility of risk
of malignant hyperthermia and rhabdomyolysis and also
prolonged mechanical ventilation and worsening of neurological
status were explained. A multidisciplinary discussion
involving the gynaecologist, anaesthesiologist, and
neurologist was held. The decision was made to proceed with
spinal anaesthesia. The patient and her family were counselled
about the risks and benefits of both GA and spinal
anaesthesia, including the possibility of conversion to GA if
the spinal failed or if she could not tolerate the procedure
awake.
The anaesthetic and surgical management of the patient was as
follows. In the operating room, standard monitors, 5
lead ECG, ST segment interpretation, pulse oximeter, Non
Invasive Blood Pressure (NIBP), temperature probe
(Axilla) were attached. An intravenous line was secured
with 16G cannula. Radial Artery cannulation was done under
local anaesthesia for invasive blood pressure monitoring
(IBP). Under strict aseptic precautions and local anaesthesia,
in the left lateral position, a subarachnoid block was
performed at the L3-L4 interspace using a 25G Quincke needle.
3.8 ml of 0.5% hyperbaric bupivacaine with 60 mcg of
buprenorphine was injected. A sensory block upto the T6
dermatome was achieved. The patient was sedated with an
infusion of dexmedetomidine at 0.2-0.5 mcg/kg/hr. Her sedation
levels and respiratory rates were also monitored.
The patient was placed in a Trendelenburg position. Care was
taken to secure her position to prevent nerve injury or
slippage. A low-pressure pneumoperitoneum was maintained at 12
mmHg. A total laparoscopic hysterectomy with bilateral
salpingectomy was performed. The surgery lasted 90 minutes.
The patient remained haemodynamically stable throughout the
procedure.
The patient was transferred to the Post Anaesthesia Care
Unit (PACU) for further monitoring. Her motor and
sensory block regressed completely in 5-6 hours.
Post-operative analgesia was provided with intravenous
paracetamol 1g and tramadol 100mg was given IV 8th
hourly. Her recovery was uneventful, with no cardiac or
respiratory complications. She was discharged on the fourth
post-operative day. At her 2-week follow-up, she was
recovering well.
Discussion
This case report details the successful use of spinal
anaesthesia for a total laparoscopic hysterectomy in a middle
aged woman with Becker Muscular Dystrophy. The primary success
of this case lies in the complete avoidance of general
anaesthesia and its associated life-threatening risks in this
vulnerable patient.
The anaesthetic management of patients with muscular
dystrophies like BMD is challenging [4]. BMD is a congenital
muscle disorder caused by genetic mutations. The abnormal
dystrophin protein predisposes them to a range of
complications under GA. The foremost concern is the risk of
malignant hyperthermia, a hypermetabolic crisis triggered by
volatile anaesthetics and succinylcholine [5]. Rhabdomyolysis,
hyperkalaemia, and cardiac arrest is a well-documented risk in
patients with BMD. Therefore, a core principle of anaesthesia
in these patients is the strict avoidance of all known
triggering agents, making a total intravenous anaesthesia
(TIVA) or a regional technique preferable. When TIVA is used
as the only anaesthetic technique, the occurrence of
postoperative respiratory depression may increase in BMD
patients due to high sensitivity to opioids and muscle
relaxants. Moreover, propofol may lead to profound
hypotension, reduced organ perfusion, and violent coughing.
Our choice of spinal anaesthesia completely eliminated this
risk.
Laparoscopic surgery, particularly hysterectomy, is almost
universally performed under GA with endotracheal intubation.
This is because neuraxial anaesthesia presents its own set of
challenges for this type of surgery. The creation of a
pneumoperitoneum and the required Trendelenburg position can
lead to significant respiratory compromise and cephalad spread
of the anaesthetic. Furthermore, peritoneal and diaphragmatic
stretching from carbon dioxide (CO
2) insufflation
frequently causes referred shoulder tip pain, which is not
blocked by a mid-thoracic spinal level and can be intolerable
for an awake patient [6].
In our patient, we addressed these challenges through multiple
strategies. Firstly, we maintained a low-pressure
pneumoperitoneum (10-12 mmHg) to minimise diaphragmatic
irritation and reduce systemic CO
2 absorption.
Secondly, we used an infusion of dexmedetomidine for conscious
sedation. Dexmedetomidine is an ideal agent in this scenario
as it provides sedation and anxiolysis without causing
significant respiratory depression, a crucial benefit in a
patient with pre-existing restrictive lung patterns [7]. Its
analgesic properties also help obtund the visceral discomfort
and shoulder pain associated with laparoscopy under regional
anaesthesia [8]. The stable haemodynamics and patient comfort
observed in our case highlights the usefulness of
dexmedetomidine. Reports of major laparoscopic procedures
under neuraxial anaesthesia in patients with muscular
dystrophies are scarce in the literature. Most reports
describe regional techniques for lower limb or minor open
abdominal surgeries. For instance, spinal anaesthesia has been
successfully used for patients with other myopathies, like
Miyoshi myopathy, for procedures below the umbilicus [9].
However, reports on advanced laparoscopic surgeries like
hysterectomy in BMD patients are rare, making this case a
valuable addition to the existing evidence. Our successful
management demonstrates that with careful planning, the
benefits of minimally invasive surgery can be extended to this
high-risk population without resorting to GA.
It is important to acknowledge the limitations of this report.
This is a single case, and its success was contingent on the
patient's specific clinical profile, namely, preserved cardiac
function and only minor respiratory impairment. This approach
may not be suitable for patients with severe cardiomyopathy,
or significant respiratory muscle weakness.
Conclusion
Spinal anaesthesia, supplemented with conscious sedation and
managed with a low-pressure pneumoperitoneum, presents a safe
and effective alternative to general anaesthesia for
laparoscopic hysterectomy in carefully selected patients with
Becker Muscular Dystrophy. This strategy requires a thorough
preoperative multidisciplinary assessment and meticulous
intraoperative care but can significantly enhance patient
safety by circumventing the profound risks of general
anaesthesia in this population.
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