Emergency Medicine
Board Review

Educational Objective:

Discuss possible presentations of an atraumatic subarachnoid hemorrhage in pediatric patients.

Key Point:

Symptoms may include a "thunderclap headache", double vision, confusion, and loss of consciousness. Symptoms tend to evolve rapidly and may sometimes be preceded by a centinel bleed a few days earlier.

Explanation:

Bleeding occurs in the subarachnoid space between the arachnoid mater and the pia mater.

Computed tomography of the head is the usually the first imaging modality indicated. A lumbar puncture may be helpful if the CT scan is negative and a high suspicion remains and the child does not have signs of significantly increased intracranial pressure. Angiography may be indicated to identify an additional site of bleeding.

The prognosis for an atraumatic subarachnoid hemorrhage is poor. Many people with a subarachnoid hemorrhage die before reaching the hospital. One-third of the remaining cases die soon after reaching the hospital. Surgery and controlling the patient's blood pressure are the mainstays of treatment. Survivors are often at increased risk for another rupture.

References:

Carlo WA. Nervous system disorders. In: Kliegman RM, et al, eds. Nelson Textbook of Pediatrics. 19th ed., 2011.

deVeber G. Pediatric stroke syndromes. In: Kliegman RM, et al, eds. Nelson Textbook of Pediatrics. 19th ed., 2011.

In LGL, James fibers bypass the AV node, directly connecting the atrial conduction system to the His bundle, causing a short PR interval and supraventricular tachycardia.

Multiple types of cardiac bypass tracts exist. The most common ones are:

Kent fibres – atrioventricular bypass pathway – Wolff-Parkinson-White syndrome

James fibres – atrium to HIS bundle bypass pathway – Lown-Ganong-Levine syndrome

Mahaim fibres -  HIS-bundle to ventricle bypass pathway – variation of Wolff-Parkinson-White syndrome

The tachy-brady syndrome is just one of a group of syndromes collectively know as the ”sick sinus syndrome”.

Educational Objective:

Discuss the workup of a stable patient with possible epiglottitis.

Key Point:

When epiglottitis is in the differential, the epiglottis should be visualized one way or the other in order to avoid a missed diagnosis. A lateral neck X-Ray will confirm the diagnosis in 90% of patients. If the X-Ray is non-diagnostic but clinical suspicion is present (e.g. significant sore throat with normal retropharyngeal area on simple visual inspection), the literature recommends viewing the epiglottis via direct laryngoscopy or CT scan. Adult mortality from epiglottitis is HIGHER than that of children, possibly due to missed diagnosis of the early presentation (as in the above patient) of this disease without signs of airway compromise.

Explanation:

Adult epiglottitis is becoming more common, with the milder, stable cases (such as the above patient) most often misdiagnosed as  "strep throat" (a diagnosis that is an attempt to explain the severe pharyngeal pain). Misdiagnosis as “strep throat” leads to discharge with a prescription of ineffective antibiotics (covering streptococci) rather than admission to the ICU with IV antibiotics covering H. influenza (the most likely pathogen in adult epiglottitis). Possibly due to the rate of missed diagnosis of milder cases, adult mortality from epiglottitis is HIGHER than that of children. 

The literature states that in stable patients (no drooling or tripod position) with significant throat pain and a muffled voice and normal (red flag!) visual exam of the retropharynx, some type of direct assessment of the epiglottis is necessary. In 90% of adult patients, a simple lateral neck x-ray will yield the diagnosis. In the 10% where the x-ray is normal or non-diagnostic, a direct look with the laryngoscope is recommended by multiple authors. 

For the laryngoscopic look, Rosen (2018) recommends a conscious sedation procedure with setup for a rescue airway even in the stable patient.

If the supraglottic/epiglottic area is swollen and erythematous, the patient should be admitted (usually to the ICU) and antibiotics such as cefotaxime or ceftriaxone that cover Hemophilus influenzae should be initiated empirically until cultures return.

Review – Adult Epiglottis:

In adults, the term supraglottitis would capture the disease process better because the epiglottis itself may be normal in the setting of severe supraglottic swelling that may involve the base of the tongue, vallecula, aryepiglottic folds, arytenoid soft tissues, and the lingual (not the pharyngeal!) tonsils. Though a variety of viral and bacterial disease agents may be the culprit, Hemophilus influenza continues to be associated with the most severe disease process in adults. 

Rarely, an epiglottic abscess may be present, in which case Streptococcus and Staphylococcus species are the predominant organisms. Occasionally, epiglottitis may also be secondary to thermal injury. The disease occurs slightly more frequently in males, has no seasonal peak, and does not involve infraglottic structures.

Usually, patients have experienced a prodromal upper respiratory tract infection that may have started anywhere from a few hours to 7 days before presenting to the health care system. At the time of presentation, classic signs and symptoms are dysphagia, odynophagia, and a muffled or "hot potato" voice (similar to the hot potato voice of peritonsillar abscess, and different from the duck quack muffled voice of retropharyngeal abscesses).

Although some patients present with simultaneous pharyngeal pathology, including pharyngitis, tonsillitis, peritonsillar abscess, and deep space infections, the majority of patients with epiglottis have no oropharyngeal findings on exam, making it important to maintain a high degree of suspicion for epiglottitis in any patient presenting with a muffled voice, dysphagia, and odynophagia. 

Drooling and dysphonia are signs of imminent airway collapse. The sniffing position is the last sign to appear immediately before respiratory arrest.

Pearls: In the classroom, hot potato voice is often taught to be associated with peritonsillar abscess, This leads to a missed diagnosis in the clinic when the hot potato voice to due to something other than a peritonsillar abscess especially if no pathology is visible upon inspection of the oropharynx. All of the following space-occupying lesions can present with a Hot Potato Voice:

1. Lymphoid masses

2. Quinsy – peritonsillar cellulitis or abscess

3. Epiglottitis

4. Tumors of vallecula between the epiglottis and the base of the tongue

5. Lingual thyroid gland

6. Foreign body

Hoarseness, on the other hand, is typically ABSENT in epiglottitis. If the patient presents with hoarseness, laryngitis is the more likely diagnosis.

References:

Melio RM. Upper Respiratory Tract Infections (Chapter 65).  In: Walls R, et al. Rosen’s Emergency Medicine: Concepts and Clinical Practice. 9th ed., 2018: 857-870.e1.

Gompf SG. Epiglottitis Workup. https://emedicine.medscape.com/article/763612-workup (Updated April 28, 2020) (Accessed December 22, 2020).

Lindquist B, Zachariah S, Kulkarni A. Adult Epiglottitis: A Case Series. Perm J. 2017. 21

Bellis M, Herath J, Pollanen MS. Sudden Death Due to Acute Epiglottitis in Adults: A Retrospective Review of 11 Postmortem Cases. Am J Forensic Med Pathol. 2016 Dec. 37 (4):275-8. 

Discussion of hot potato voice with a youtube example of a hot potato voice (in this case, from a peritonsillar abscess) https://epomedicine.com/clinical-medicine/hot-potato-voice/

Strongyloidiasis is endemic to the tropics, subtropics, and the southeastern United States. Humans are the principal hosts. Strongyloides is usually acquired in endemic regions by direct skin penetration by larvae after coming into contact with infected soil. Most people who become infected with Strongyloides are asymptomatic. Female Strongyloides release their eggs into the small intestine of the host, which is where the larvae hatch and are then excreted into the feces.

The parasite completes a life migratory cycle that involves traveling to the lungs and ascending to the pharynx. It is eventually swallowed and reaches the intestine.

In immunosuppressed patients, hyperinfection can occur and manifest with creeping rashes, respiratory manifestations with lung infiltrates, and eosinophilia. Gram-negative sepsis is a common complication because it is believed that the larvae may predispose the translocation of bowel bacteria.

Hyperinfection syndrome caused by Strongyloides infection usually occurs in people who are chronically infected and become immunosuppressed. Larvae migrate throughout the body via the systemic circulation to distant organs. Migration of the larvae into the central nervous system has been associated with bacterial meningitis. One of the most important and potentially fatal complications that can occur is gram negative bacteremia or meningitis mainly from pathogens such as Streptococcus bovis, Escherichia coli, Streptococcus fecalis, Klebsiella pneumoniae, or Enterobacter sp. as they become blood borne when the larvae penetrate the intestine.

Ivermectin is the antiparasitic of choice; however, patients with sepsis or meningitis secondary to the hyperinfection require antibiotics. Use of corticosteroids may worsen the hyperinfection.

Reference:

American Academy of Pediatrics. Red Book: Report of the Committee on Infectious Diseases. 29th ed., 2012.

Amyotrophic lateral sclerosis (ALS) is the most common form of adult-onset motor neuron disease and the third most common neurodegenerative disease overall. It is not an entrapment neuropathy. Entrapment neuropathy is one of the most common causes of peripheral mononeuropathy, and is often related to injury, overuse or repetitive minor trauma. Mononeuropathies are either isolated or multiple. Isolated mononeuropathies are usually caused by trauma to or entrapment of a particular nerve. Isolated mononeuropathies may be acute, chronic, constant, or intermittent.

Nerves commonly entrapped are the radial nerve, ulnar nerve (in the cubital canal of the forearm or Guyon’s canal at the wrist), median nerve, sciatic nerve, lateral femoral cutaneous nerve, and the common peroneal nerve.

References:

Renton, Alan E., Adriano Chiò, and Bryan J. Traynor. "State of play in amyotrophic lateral sclerosis genetics." Nature neuroscience 17.1 (2014): 17-23.

Schmid, Annina B., et al. "The relationship of nerve fibre pathology to sensory function in entrapment neuropathy." Brain (2014): awu288.