Critical Case Discussion
Online case based discussion
Case 1 – CCD
A 3-year-old male presented to the emergency room (ER) by his mother with a complaint of bloody diarrhea and was admitted in the pediatric intensive care unit (PICU). Initially, The child was alert but fussy, appears pale, and was non-toxic. His conjunctiva was pale, pharynx and tonsils appear normal, and tympanic membrane was intact. His neck was supple without adenopathy. In heart examination, there was a regular rhythm with tachycardia and a grade II/VI vibratory systolic ejection murmur at the left sternal border without radiation. No heaves, lifts, thrills, rubs, or gallops were present. His lungs were clear with good aeration and abdomen was flat, soft, and non-tender, with the liver edge palpable 3 cm below the right costal margin. The spleen was non-palpable. His pulses and perfusion were good. There was no edema, rash, or petechiae. Vital signs were as follows: temperature 37.7C (99.9F), heart rate 150 bpm, respiratory rate 28 breaths per minute, blood pressure 100/45 mmHg, and oxygen saturation 100% on room air. Weight is 13 kg (75th percentile).
A basic metabolic panel, complete blood count, and urinalysis were obtained. A blood peripheral smear showed schistocytes, helmet cells, and polychromasia.
White blood cell count – 16 000/mm3 |
Hemoglobin – 6.9 g/dL |
Hematocrit – 22.4% |
Platelet count – 37 000/mm3 |
Neutrophils – 56% |
Lymphocytes – 27% |
Sodium – 133 mEq/L |
Potassium – 5.8 mEq/L |
Chloride – 106 mEq/L |
Carbon dioxide – 13 mEq/L |
Blood urea nitrogen – 48 mg/dL |
Creatinine – 3.82 mg/dL |
Glucose – 145 mg/dL |
Calcium – 7.8 mg/dL |
Explanation:
If a child presents with microangiopathic hemolytic anemia, thrombocytopenia, and renal failure preceded by abdominal pain and diarrhea, the diagnosis can be made for Shiga toxin-associated hemolytic uremic syndrome (HUS). This accounts for 90% of HUS cases in children.
Management:
Therapy for HUS is supportive and includes volume repletion, hypertension control, and managing complications of renal insufficiency, including dialysis when warranted. Red blood cell transfusions may be indicated. Platelet transfusions should be avoided since they may contribute to the thrombotic microangiopathy, and are indicated only by active hemorrhage or prior to a procedure. Early hydration during the diarrheal phase may lessen the severity of renal insufficiency. Most children with Shiga toxin-associated HUS survive the acute phase and recover normal renal function
Case 2 – CCD
A 6-month-old female, with no significant medical history, admitted to the pediatric intensive care unit (PICU) with one day of fever over 38.8 0C (102F), bilious non-bloody emesis, generalized abdominal pain, and decreased oral intake. On examination, she appeared ill with dry cracked lips, sunken eyes, and flat anterior fontanelle. Extremities were cool to the touch, with warm head and trunk. She has decreased bowel sounds with abdominal distension. Nursing staff have witnessed an episode of emesis while breast-feeding, but she has easy work of breathing, without adventitious sounds. Vital signs on arrival in ER were temperature 38.6 C (101.5F), heart rate 148 bpm, blood pressure 114/49 mmHg, respiratory rate 38 breaths per minute, and oxygen saturation 96% on room air.
She was admitted in the PICU, and with concern for an abdominal obstruction and fever in an infant, an abdominal radiograph, urinalysis, and a respiratory viral nasal swab were done. Because of signs of dehydration, a peripheral intravenous catheter is placed, blood work obtained, and rehydration therapy started following an initial 20 mL/kg of normal saline. While in the ER, she showed a positional preference with her head, and no longer turned it to the right (with negative meningeal signs). She has undergone computed tomography (CT) of the neck without pertinent findings. She continues to have fever and blood cultures were obtained and a single dose of ceftriaxone administered. An abdominal ultrasound was done for further evaluation of her emesis, but that was unremarkable.
Table 1: CBC
White blood cell count – 15 000/mm3 |
Hemoglobin – 9.9 g/dL |
Hematocrit – 30.8% |
Platelet count – 528 000/mm3 |
Neutrophils – 66% |
Eosinophils – 1% |
Monocytes – 11% |
Table 2: Chemistry
Sodium – 136 mEq/L |
Potassium – 5.1 mEq/L |
Chloride – 103 mEq/L |
Carbon dioxide – 23 mEq/L |
Blood urea nitrogen – 7 mg/dL |
Creatinine – 0.2 mg/dL |
Glucose – 118 mg/dL |
Calcium – 10.2 mg/dL |
Table3: Urine Examination
Color – Normal |
Appearance – Cloudy |
Specific gravity – 1.025 |
pH – 5.5 |
Leukocyte esterase – Negative |
Nitrate – Negative |
Protein – <30 mg/dL |
Glucose – Normal |
During the first day of her inpatient stay, she continues to be febrile and tachycardic, responsive to antipyretics. She continues to favor her left side and does not rotate her head to the right. She is irritable with poor oral intake, but is soothed when held by parents. During her afternoon feed, she starts to cough which is followed by a large emesis. An hour following the emesis, the nurse is called to the room to find patient with retractions, tachypnea, and grunting. A provider is called to bedside, pulse oximetry placed, and suction attempted. Oxygen saturation readings are in the 70s, respiratory rate in the 70s, and she is pale and mottled with weak thready pulses. The rapid response team is called to evaluate the patient. The rapid response team arrive and initiate bag mask ventilation, and child was intubated successfully.
After intubation, the Focused neurologic examination produces the following results. Her eyes are closed and she does not respond to voice or tactile stimuli other than with triple flection to noxious stimuli in the right lower extremity. Her pupils are 1 mm, round and minimally responsive to light. Oculocephalic reflex not clearly present. She has symmetric facial movement with a midline tongue. Her four limbs are flaccid. She has no response to noxious stimuli in bilateral upper extremities or left lower extremity. She has a 1+deep tendon reflex (DTR) at the bilateral brachioradialis. Trace DTR at the patella and achilles bilaterally. No clonus. Her toes are mute on the left, down going on the right with plantar stimulation.
After 24 hours in the PICU, she developed decreased lung compliance and worsening respiratory examination, likely due to aspiration pneumonia.
The infant is placed on minimal conventional ventilator settings with a dexmedetomidine infusion for sedation and analgesia. Antibiotic coverage is broadened to cover for sepsis, aspiration pneumonia, and viral encephalitis. A CT scan of the head and lumbar puncture was done that reported normal.
Table4: CSF Examination
Appearance – Clear |
Color – Colorless |
Xanthochromia – Absent |
Red blood cell count – 9/mm3 |
Leukocytes – 108/mm3 |
Protein – 21 mg/dL |
Glucose – 101 mg/dL |
Herpes simplex virus – Negative |
An electroencephalogram (EEG) is placed for continued evaluation of brain activity. 1-day EEG recording showed an abnormal awake and asleep pattern with no seizure activity. Magnetic resonance imaging (MRI) of the brain is completed due to progression of neurologic symptoms. Findings on MRI suggest rhombencephalitis (likely viral) due to bilateral symmetric signal abnormalities involving the brainstem and extending to the cervical cord.
Diagnosis: Brainstem (rhombencephalitis) encephalitis
The terms “brainstem encephalitis” and “rhombencephalitis” are synonymous. Causes include infectious, autoimmune etiology, or paraneoplastic syndromes. The most common infectious agents are Listeria, enterovirus 71, and herpes simplex virus. Behçet’s dis-ease, systemic lupus erythematosus, and relapsing polychondritis have also been documented to cause rhombencephalitis. MRI is the gold standard for evaluation
What is the difference between encephalopathy and encephalitis?
Encephalitis is defined as altered level of consciousness lasting for 24 hours or more, plus two or more of the following: fever (temperature >38 C), seizures (generalized or partial) or abnormality on EEG, pleocytosis (white blood cells in CSF≥5/mm3), neuroimaging consistent with encephalitis, or detection of autoantibodies in CSF. When the criteria for encephalitis are not met due to lack of inflammatory criteria (e.g. absence of fever, CSF pleocytosis, and/or imaging changes) and with altered mental status lasting more than 24 hours, it is defined as encephalopathy
Management:
High-dose steroids and intravenous immunoglobulin (IVIG) were administered without improvement. She received multiple rounds of antibiotics. There was partial improvement. Patient ended up with tracheostomy due to prolong intubation. She has neurological sequalae and had long term neurological follow up.