Hx: A 17 month old male presents with a seizure. He is driven to the emergency department by his parents after they witnessed him having the seizure. According to parents, the child has been well until today. No fevers, no illness, and no history of prior seizures. Additionally, there is no family history of seizures.
While in triage, the nurse notes the patient has multiple bruises and brings the patient back immediately concerned for abuse.
PMHx: None
SocHx: One older healthy sibling.
Exam:
- Vitals: Pulse 140, RR 25, BP 90/60, Temp 98.6F (37C)
- General: poorly responsive, no distress, cool to touch
- Skin: multiple ecchymoses on all 4 extremities in various stages of healing, two small ecchymosis on forehead and face.
- HEENT: Normocephalic, normal TMs, pupils equal and sluggish bilaterally, pharynx clear
- Resp: Clear to auscultation, mild tachypnea
- Cardiovascular: tachycardia, regular, normal pulses in all extremities
- Abdomen: Soft non-tender, no masses, bowel sounds present.
- Extremities: no deformities
- Neuro: arousable to loud noises, will not follow commands, no abnormal flexion or withdrawal to pain, cranial nerves grossly intact but patient unable to participate in exam. Will move extremities purposefully.
Differential:
- Meningitis
- Sepsis, septic shock
- DIC
- Child Abuse, Trauma
- Malignancy – leukemia, lymphoma
- Idiopathic Thrombocytopenia (ITP)
- Aplastic Crisis, pancytopenia
- Toxic Ingestion
ED Course:
- Immediate IV access, supplemental oxygen, and monitoring.
- Shortly after initial examination, the patient had a generalized seizure. A dose of lorazepam is given with termination.
- A few minutes later, the patient has a second tonic clonic seizure and is given a second dose of lorazepam.
- After CT the patient has a third tonic clinic seizure. At this point, rapid sequence induction is undertaken and the patient is placed on a propofol infusion and give a loading dose of Keppra IV.
- Shortly after labs and CT results have returnees, the patient is admitted to the pediatric ICU.
Diagnostics:
- CT Head – multiple bilateral cortical petechial hemorrhages. No subdural or epidural hematomas. No skull fracture.
- Significant labs – WBC 57,000, HGB 9, Platelets 30,000.
Hospital Course:
- The patient is admitted to the pediatric ICU then transferred to a pediatric hematology center.
- Induction chemotherapy is initiated and the patient ultimately does well.
Dx: Status Epilepticus due to Acute Lymphoblastic Leukemia.
Brief Discussion: Leukemia is the most common cancer in children, making up almost 30% of all childhood cancers . Among them, Acute Lymphoblastic Leukemia (ALL) is the most common, with rates 5 times that of Acute Myeloid Leukemia (AML). Peak incidence is between 2 and 5 years of age and prevalence in the US is highest in caucasians, followed by hispanics, then black Americans.. The current 5 year survival rate is 85%, showing a dramatic improvement within the past 30 years.
First presentation may include
- Hepatomegaly (64%) or splenomegaly (61%) which can be associated with abdominal pain, distention, or poor appetite
- Lymphadenopathy (50%) which is painless and unresponsive to antibiotics
- Persistent bone pain present in long bones (21-38%)
- Headache and other intracranial manifestations occur with CNS infiltrations (<5%)
- Mediastinal mass
- WBC <10,000 (50%), WBC >50,000 (20%)
- Anemia is very common due to bone marrow infiltration
- Platelets <100,000 (75%)
- Bleeding, petechia, purpura (50%)
- Disseminated Intravascular Coagulation (DIC) (10%)
Initial treatment is focused on stabilization and resuscitation. In our case example, emergent control of seizures was required prior to transfer to an ICU setting and eventually a pediatric cancer center.
Pediatric status epileptics is defined as continuous seizure activity for 5 minutes or repetitive seizures without return to baseline for 30 minutes. Multiple treatment algorithms exist and include the rapid escalation of therapy. In general, benzodiazepines (lorazepam, diazepam) are the accepted initial or first line therapies. If no response is obtained to these medications, anti-epilipetic therapy is initiated (dilantin, keppra, valproate, phenobarbital). Typically this second line therapy is given in conjunction with induction of a coma through continuous infusion of propofol, midazolam, or pentobarbital. Once seizure control is achieved, focus is placed on determining the underlying cause through laboratory testing and imaging.
References:
Up-To-Date ALL
Medscape – ALL
Medscape – Pediatric Status Epilepticus
Up-To-Date Pediatric Status Epilpeticus