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.