Case Presentation
The patient was a 41-year-old, gravida 3 with pycnodysostosis scheduled
for an elective cesarean delivery at week 37 + 5 of pregnancy. Previous
2 cesarean sections were done, trying with her spinal anesthesia, but
the patient was not cooperative due to labor pain converted to general
anesthesia. General anesthesia was difficult during intubation and
post-extubation; the patient developed aspiration pneumonia and was
managed with antibiotics without admission to the ICU.
The patient was 140 cm in height, weighed 60kg, and had a Body Mass
Index (BMI) of 35.9. she is characterized by a short neck, small
mandible, and Mallampati classification (MPC), TMD thyromental distance.
She had known allergies to Butamirate® (cough suppressant) and Rhinopro®
(Pseudoephedrine & Carbinoxamine) drugs and was not taking any
prescription medications.
She had a history of the bilateral femur pathological fractures
(Figure-1) and underwent closed reduction and intramedullary (nail) left
femur early in 2020, under spinal anesthesia Ultrasound showed three
hyperechoic subcutaneous lesions 19x7mm in the post aspect, 12x7mm, and
16x10mm in the medial aspect of the right side. Moreover, the left side
showed four well-defined hyperechoic subcutaneous lesions measuring
20x12mm in the lateral aspect, 10x7mm in the medial aspect, 12x9mm, and
9x6 mm in the posterior aspect. All show no septation, no calcification,
and no vascular by doppler.
She planned for a second surgery for the right femur, but it was not
done because of the COVID-19 pandemic, and after that, she became
pregnant. The patient had severe Obstructive sleep apnea (AH1) diagnosed
by a sleep study in 2014 and mild asthma since childhood advice to be on
continuous positive airway pressure (CPAP), but she refused. The
presence of obstructive sleep apnea may have a risk of airway
obstruction in the postoperative period; thus, it should be carefully
addressed. She had a family history of the same disease in maternal and
paternal cousins (familial), dwarfism, her sister, and her maternal
grandmother.
Physical examination of the spine revealed multilevel disc dehydration;
multiple disc bulges are identified at multiple levels starting in the
cervical and lumbar spine. The most marked changes are seen at the C5-C6
level, where there are left posterior disc bulges causing indentation
over the thecal sac with mild narrowing of the central canal (Figure-3).
On the 1st-day post-Lower Segment Caesarean
section(LSCS), she developed a cough, palpitation, and tachycardia; the
patient desaturated Oxygen demand gradually increased from 3 liters to 8
liters, then she had sustained cardiac arrest, CPR was performed for 2
minutes, with 1 dose of adrenaline, developed later to multiple comorbid
Cardiac arrests was resuscitated and intubated for 4 days, then
extubated and shifted to the inpatient ward.
Later, she developed multi myoclonus jerking, both upper arm spasms with
upward rolling of eyes due to PAID syndrome during her ICU stay. In ICU,
ECG showed sinus tachycardia, T wave inversion in leads V1, V2, V3, V4,
previous ECG dated back to 2022 showed T wave inversions in leads V1,
V2. At the return of spontaneous circulation (ROSC), an arterial blood
gas shows PCO2:70, after 10 minutes of intubation and ambobaging.
Repeated ABG showed lactate: 2.4, PCO2: 45, PO2:65 ON 50% FIO2. EEG
shows no intracranial mass effect, hemorrhage, or gross ischemic
infarction area.
EEG is indicative of paroxysmal nonepileptic events and a mild-moderate
diffuse encephalopathy secondary to sedative medications. Few lower
extremity jerks were induced only with stimulation recorded with no EEG
correlates. MRI whole spine revealed multi-disc disease but no abnormal
signal in the cord, no major pathology. She was currently admitted under
the impression of post-hypoxic cortical myoclonus.
Computed tomography arterial portography (CTAP) reported bilateral lower
lobes consolidation, more on the left side. Bilateral lung changes could
be related to atelectasis.
The patient complains of adjustment disorder due to physiological
consequences of the current health changes following her delivery as
manifested with low mood; the patient denied that; however, there were
episodes last week in which she was angry, shouting, and refusing
medical care but not anymore. The psychiatrist recommended no need for
psychotropic medication. However, it is important to reassure and
involve her in any intervention and procedure frequently.
The patient shifted to a private room accompanying her newborn and
reports improvement regarding her myoclonic movements, affecting her
lower limb mainly occur whenever she takes her clonazepam and worsens
during evening time (in between the doses). She was unable to sit
without support, unable to mobilize, and difficult to hold her legs in
flexed posture or against gravity due to myoclonus. The patient still
has an involuntary movement of the lower part of the body. On the other
hand, she is alert and oriented with comprehensive speech. The discharge
plan includes a wheelchair with a Zimmer frame, a home CPAP machine, and
home exercises’ instructions; the physiotherapist advised not to
discharge the patient and keep her at the hospital.