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M2E Too! Mellick's Multimedia EduBlog by Larry Mellick, MD

​The M2E Too! blog presents important clinical pearls using multimedia. By its name, M2E Too! acknowledges that it is one of many emergency medicine blogs, but we hope this will serve as a creative commons for emergency physicians.​

Friday, March 31, 2023

My twin brother, a neurology pain specialist, discovered in the late 1990s that cervical injections with bupivacaine were effective in relieving headache and orofacial pain. We subsequently wrote three papers describing my emergency department experience with this procedure in treating adult and pediatric headaches and orofacial pain. (Headache. 2006;46[9]:1441; https://bit.ly/3ZtbP3V; J Orofac Pain. 2008;22[1]:57; Pediatr Emerg Care. 2010;26[3]:192.)

The bilateral injection of 1.5 mL of 0.5% bupivacaine in the paraspinous muscles at around the sixth cervical spinous process has demonstrated effectiveness in treating a spectrum of headaches, from migraines and tension headaches to subarachnoid bleed headaches, meningitis (bacterial and viral), hemiplegic, trigeminal neuralgia, and post-concussion headaches. All have shown a therapeutic response to this technique in the two decades I've been using it.

Corneal abrasions, toothaches, painful shingles of the face, TMJ pain, and other orofacial pain conditions have also responded. The spectrum of headaches and conditions responding to this treatment has convinced me that various headache etiologies probably have a common mechanism and pathophysiology.

I recently saw my first pediatric patient, an 11-year-old girl, with a classic cluster headache and successfully treated it with bilateral bupivacaine injections with 40 mg of solumedrol added. Cluster headache is rare in children, and it isn't common in adults in my experience either. Consequently, I was delighted and proud of the complete resolution of this patient's headache within 15 to 20 minutes using this technique.

What's new with paraspinous cervical injections? A medical student and I just recently submitted for publication a systematic review of anesthetic and saline injections and dry needling for headache patients. We reviewed 440 articles and selected seven for review, including retrospective case studies of patients with headaches or orofacial pain who received paraspinal cervical or other intramuscular injections. A total of 683 of the 841 patients with headache pain in the studies received anesthetic injections. The remaining patients received dry needling therapy or saline injections using the same trigger points.

Significant pain relief from intramuscular or paraspinal cervical injections was observed in 83.7 percent of the anesthetic and dry needle patients. Surprisingly, dry needle intramuscular injections also had a very high success rate—93 percent in 44 patients—while only 37 percent of patients who received saline injections reported pain relief. Saline injections were similar in effectiveness to a previously reported placebo effect of subcutaneous needle injections (32.4%). (J Neurol. 2000;247[3]:183.)

The total number of patients was small, but we were surprised at the success of dry needle intramuscular injections. Evidence suggests that acupuncture has a role in treating headaches, and dry needling seems similar in technique and may have similar mechanisms. (Cochrane Database Syst Rev. 2016;4[4]:CD007587; https://bit.ly/3EMJJJ9; Evid Based Med. 2016;21[5]:183; Pain Res Manag. 2020;2020:3825617; https://bit.ly/3IFZtia.) Dry needling for headaches and orofacial pain deserves further investigation.

We are no closer to understanding the mechanism for headache and orofacial pain relief, but our proposed mechanism continues to be the most rational explanation. Evidence suggests an antinociceptive effect on the trigeminocervical complex may be part of the pathway in addition to reducing the second order neuron cell activity. Both the cervical and trigeminal afferents are known to converge at the brainstem, and the periaqueductal gray, nucleus raphe magnus, and the rostroventral medulla also synapse the trigeminocervical complex, providing a strong antinociceptive effect. This complex may serve as the drug target.

Watch a video of patients talking about the pain relief they experienced after cervical injections with bupivacaine.

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Wednesday, March 1, 2023

​Celine Dion, one of the best-selling musical artists of all time, recently announced that she had been diagnosed with a rare condition called stiff person syndrome. My first introduction to this condition occurred in 2017 when a 54-year-old woman with stiff person syndrome presented to my emergency department.

Stiff person syndrome was first reported and initially labeled stiff man syndrome in 1956. (Proc Staff Meet Mayo Clin. 1956;31[15]:421.) The authors described a series of patients with fluctuating and progressive rigidity and painful muscle spasms causing gait difficulties, falls, and a wooden appearance.

The name was changed to stiff person syndrome a few years later to acknowledge that men and women are affected with this autoimmune condition. Stiff person syndrome is most frequently associated with antibodies against glutamic acid decarboxylase, which is the underlying pathophysiology of this condition. Glutamic acid decarboxylase is an important enzyme in the synthesis of gamma-aminobutyric acid (GABA), an amino acid critical for maintaining inhibitory pathways. The decline in GABA levels in the CNS causes a loss of neural inhibition and increased muscle activity. In other words, the resulting low levels of GABA, a major neurotransmitter responsible for inhibitory synaptic signaling, cause consequent disinhibition and the symptomatology of stiff person syndrome. This can present classically, as focal, segmental, and jerking variants, or as progressive encephalomyelitis with rigidity and myoclonus.

The onset is typically insidious with intermittent muscle tightness or achiness. Hypertonia or rigidity caused by sustained antagonist muscle groups co-contraction results over time in restricted range of motion, slowed voluntary movements, hypertrophy of muscles, and abnormal postures. Emotional upset, startle from various stimuli, or sudden movements may trigger muscle spasms. Pain is a common complaint, and other neurologic findings may include oculomotor disturbances, exaggerated startle responses, and paroxysmal dysautonomic crises (hypertension, tachycardia, hyperthermia, and diaphoresis). Stiff person syndrome is often associated with type 1 diabetes mellitus (30%) and other autoimmune disorders. It can also be part of a paraneoplastic syndrome. Psychiatric comorbidities include agoraphobia, depression, alcohol abuse, and anxiety, and their presence can result in a delayed or less timely diagnosis. (J Acad Consult Liaison Psychiatry. 2021;62[1]:3.)

Therapy includes symptomatic relief and immune modulation. The GABA agonists, diazepam and oral baclofen combined or alone, are usually effective for treating rigidity and muscle spasms. Unfortunately, progressively higher doses are often required, resulting in intolerable side effects. Intrathecal baclofen may also be used as rescue therapy for severe stiff person syndrome. Other medications such as antiepileptic drugs including levetiracetam are also used with some success. Immune modulation therapies include intravenous IgG and are initiated when symptomatic relief is inadequate with the GABA agonists. If patients do not respond to IVIG, they may respond to other therapies such as plasma exchange or B-cell depletion with rituximab.

Unfortunately, many patients' symptoms persist, and significant disability and quality of life is severely affected despite these therapies. 

Watch a video and hear the story of a patient with stiff person syndrome​.​

Mellick Stiff Person Syndrome.jpg

Wednesday, February 1, 2023

Streptococcosis or streptococcal fever is one of those little known, single-paragraph diseases mentioned almost in passing during discussions of group A streptococcal disease (GAS). We are taught that children under 3 rarely get GAS pharyngitis, but instead they may have a protracted low-grade febrile infectious process presenting with nasal discharge and congestion and tender anterior cervical lymph nodes. Babies under a year old may present with fussiness, decreased appetite, and low-grade fever as well as older siblings or daycare contacts with GAS infection.

This GAS symptom complex is called streptococcosis or streptococcal fever. Even though the authoritative Red Book published by the American Academy of Pediatrics as well as the Centers for Disease Control and Prevention and trusted resources like UpToDate all accept the existence of this condition, the supporting evidence is impressively sparse. (Red Book-2021-2024 Report of the Committee on Infectious Diseases. January 2021; https://bit.ly/3X1neal; CDC. June 27, 2022; http://bit.ly/3X6UuNm; UpToDate. Feb. 1, 2022; http://bit.ly/3iC5Hqd.)

The challenge is that the symptom complex is practically indistinguishable from viral upper respiratory tract infections. A 1999 commentary noted that most cases of acute rheumatic fever do not commonly have a memorable episode of pharyngitis. (Pediatr Infect Dis J. 1999;18[8]:663.) The implications of this are that streptococcal infections of the upper respiratory tract are often entirely subclinical or very mild and suggestive of a viral upper respiratory tract infection.

This commentary underscored the role of GAS as a cause of generalized mucositis in the upper respiratory tract in children of all ages. In other words, GAS results in viral-like upper respiratory tract infections and may predispose to acute otitis media and acute bacterial sinusitis caused by the other most common pathogens. A streptococcal infection rather than a viral infection can predispose to inflammatory conditions that lead to obstruction of the sinus ostia and ultimately acute bacterial sinusitis.

Perhaps that explains why I feel this is an imaginary disease no one has ever seen when I talk to colleagues about streptococcosis or streptococcal fever. On the other hand, we have all seen this disease but not recognized it if GAS also presents as an upper respiratory tract infection. Nevertheless, we should balance this discussion by acknowledging that the Infectious Diseases Society of America guidelines specifically recommend against testing for GAS in children with URI symptoms despite awareness of this GAS symptom complex. (Clin Infect Dis. 2012;55[10]:1279.)

Others have also attempted to discern markers for simple carriers of group A streptococcus versus active infection. One report noted that the presence of cough and nasal congestion among children with a positive GAS test was 95.4 percent specific (95% CI: 87.1-99.0%) for being a GAS carrier. (Pediatr Infect Dis J. 2020;39[6]:483; https://bit.ly/3X7KTpr.)

Streptococcosis or streptococcal fever deserves to be in our differential for febrile runny-nosed toddlers presenting to our EDs. That is especially true if the toddler presents with a protracted low-grade febrile infectious process with nasal discharge and congestion and tender anterior cervical lymph nodes. The threshold for testing should also be lowered if the child has older siblings with classic GAS pharyngitis signs and symptoms. An associated cough, on the other hand, should rapidly dampen your enthusiasm for GAS testing.

Watch this video of a child with a scarlet fever rash, pharyngitis, and rhinitis indistinguishable from an upper respiratory tract infection.​

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Sunday, January 1, 2023

Abdominal pain often brings children to the emergency department, and its most common cause is constipation.

         The physiologic causes of functional constipation include:

    • Painful defecation (withholding, which starts a vicious cycle)
    • Irregular bowel habits (distraction, public restrooms)
    • Dietary causes (lack of fiber, inadequate hydration, cow's milk)
    • Decreased physical activity (obesity)
    • Psychological and behavioral (ADHD, autism, sexual abuse)
    • Toilet training (toileting refusal)
    • Genetic factors (family history of constipation)
    • Parental factors (low educational status, social status, mental health)

         Constipation is a clinical diagnosis based on patient history, physical examination, and meeting the Rome criteria. (Rome Foundation. Jan 16, 2016; https://bit.ly/3h8sSHV.) No further routine diagnostic testing is needed if there are no signs of organic disease.
         Plain films are overused to diagnose constipation. Multiple prospective studies and systematic reviews show a lack of intra- and interobserver reliability in interpretating plain radiographs. They also do not support a diagnostic association between clinical symptoms and fecal loading on abdominal radiographs.
         Another common problem is that reliance on an abdominal radiograph for evaluating constipation in patients with abdominal pain frequently resulted in the wrong diagnosis or overlooking other diagnoses. (J Pediatr. 2014;164[1]:83.) Others have noted that children with functional abdominal pain are frequently diagnosed as having constipation and are treated inappropriately with laxatives. Radiographs also add to the medical costs and cause radiation exposure. A single abdominal radiograph exposes a child to 0.46 mSv per film while the average annual ionizing exposure is 1.5-3.5 mSv. (J Pediatr. 2017;191:179.) Unfortunately, children commonly get repeat films with every visit.
          Should constipation diagnosed in the emergency department be treated with a disimpaction maneuver such as an enema or suppository before starting oral management or should we send the child home with oral medications? The evidence shows that the outcome is the same at about five days, but the symptoms improved more frequently in those who had an enema or suppository. (Pediatr Emerg Care. 2012;28[2]:115.) If there is any abdominal pain and my diagnosis is constipation, the abdominal pain needs to have been relieved by a bowel movement before I am comfortable discharging the patient home. I have managed this condition with an enema or glycerin suppository in the emergency department for years.
         We recognize that children with constipation frequently have associated urinary tract infections or evidence of bladder dysfunction. Anorectal and lower urinary tract function are physiologically related, and constipation is frequently associated with bladder dysfunction. In fact, this is referred to as the bowel and bladder dysfunction or dysfunctional elimination syndrome. (Drugs Context. 2021;10:2020; https://bit.ly/3VFgIW0.)
         Disimpaction or an initial cleanout is essential before starting maintenance therapy. Oral or rectal medications or a combination can be used. The oral route may be better tolerated, but it may take a few days to work. The enema or rectal approach is faster but more invasive. First-line oral therapy is polyethylene glycol with or without electrolytes (PEG 3350), 1-1.5 g/kg/day for three to six days. PEG without electrolytes is better accepted by children because it is more palatable than PEG with electrolytes. (Drugs Context. 2021;10:2020; https://bit.ly/3VFgIW0.)
         The second element is maintenance. Start infants with nondigestible, osmotically active carbohydrates, such as sorbitol-containing juices (prune juice, apple-prune juice, pear juice). The use of osmotic laxatives and glycerin suppositories are also options. The most effective laxative in children is PEG (0.4-0.8 g/kg/day) with or without electrolytes. Transient and mild side effects include diarrhea, bloating, flatulence, nausea, and abdominal cramps.
         Lactulose is the preferred alternative if PEG is not available. The oral osmotic laxative should be given daily, and the dose and frequency of administration should be titrated to treatment response. PEG appears to be more effective than lactulose, magnesium hydroxide, stimulants, and mineral oil in treating functional constipation in children. Stimulants may be considered for treating slow-transit functional constipation. More resistant cases may require a stimulating agent such as senna syrup or bisacodyl preparations on a short-term basis.
         The third element of constipation management is dietary education. Adequate fluid and fiber intake (age of child plus 5-10 g per day) is essential. You can increase carbohydrates (corn syrup or brown sugar) and prune juice in the diets of infants. Older children should have increased intake of fluids, prune, apple, or pear juice, bran cereal, and fruits and vegetables high in fiber.
          Watch a video of Dr. Mellick discussing constipation in children with pediatric gastroenterologist Osman Altun, MD​.

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Wednesday, November 30, 2022

Intraosseous (IO) vascular access use in children began in the 1940s, but the practice was abandoned when intravenous catheters were invented. A resurgence of interest in IO access began decades later, and the procedure was first added to the Pediatric Advanced Life Support guidelines in 1986. Intraosseous access has now become the preferred access method over central line placement and for peripheral IV access that takes longer than 30 seconds.

Initially, manual intraosseous needles were used for neonatal resuscitations because they were the only tools available. Once we learned the skill, manual IO needles served their purpose well. Cup the needle top in your palm and insert the needle with a rotational pressure perpendicular into the bone until you feel a trapdoor effect as the needle passes through the cortex into the medullary space. Later, when the EZ-IO drill was invented for IO needle insertions, we immediately fell in love and our affections for the manual IO needles waned. Now, however, evidence suggests that our first love, the manual needles, deserves to be reconsidered for attaining intravenous access in neonates.

Umbilical venous access is often recommended for emergency venous access in newborn resuscitations. That procedure, however, is rarely performed for newborns, and maintaining the equipment and skills are challenging. In fact, it is reportedly done in only 0.12 percent of all births. (Arch Pediatr Adolesc Med. 1995;149[1]:20.)

Consequently, emergency physicians are significantly less experienced with this technique. Umbilical venous catheterization is an important skill, but studies show that it is significantly faster to establish intraosseous access than an umbilical venous catheter. (Pediatrics. 2011;128[4]:e954; Pediatr Crit Care Med. 2018;19[5]:468.) My ED doesn't place many intraosseous needles because our nurses are skilled at placing intravenous catheters in even the smallest neonates. Nevertheless, the smallest neonates presenting in cardiac arrest are the ones who may require IO needle placement for intravenous access.

Evidence suggests that manual and EZ-IO access are associated with a significant number of failures in neonates because of small bone size and the difficulty in judging the depth and when the needle passes into the medullary space. The trapdoor sensation as the needle drops through the cortex is much easier to detect with manual IO needles than drill-inserted ones. The shortest EZ-IO needle used for neonates is vulnerable to adipose tissue back pressure in chubby neonates even when you maintain the 5-mm-of-needle-above-the-skin rule. I have seen perfectly placed needles dislodged moments later by the mechanics of adipose pushing back on the needle hub.

The question is whether the EZ-IO drill is the most appropriate tool for this procedure. A relatively large study survey suggested the drill can be highly successful. (Front Pediatr. 2022;10:952632; http://bit.ly/3XH6ea8.) A total of 206 IO access attempts were made in 161 neonates (145 term and 16 preterm infants), and IO access was successfully established in 146 (91%) and on the first attempt in 109 (75%). A semiautomatic battery-driven device (EZ-IO) was used in 162 attempts (88%). This seems to be a strong affirmation for using the EZ-IO, but this was a mandated two-year anonymous report to a nationwide surveillance unit. I am not confident that there wasn't some potential for reporting bias.

There is other hard evidence that our success rates with intraosseous needle placement in infants is nowhere near that pristine. Several smaller, post-mortem studies suggest our failure rate for neonates is much higher. A review based on postmortem CT scans found that tibial IO needle insertion had a 53 percent failure rate (31 cases with 42 devices) in infants 6 months and younger. (Prehosp Emerg Care. 2020;24[5]:665.) A study using pediatric cadavers reported that 16 of the 34 ION devices (47%) were malpositioned. (Resuscitation. 2019;145:1.)

Another study observed poor success rates with manual and EZ-IO devices in children under 8 kg compared with those over 8 kg. Manual IO had success rate of 55 percent (17/31) vs. 47 percent (8/17) for the EZ-IO (p=0.61) in patients 8 kg or less. (J Paediatr Child Health. 2018;54[5]:546; http://bit.ly/3VxIHqg.) Manual and EZ-IO driver placement success rates in neonates were nothing to brag about. On the other hand, manual IO did have a shorter time to placement: 4.5 min versus 12.8 for EZ-IO (p=0.02). (J Paediatr Child Health. 2018;54[5]:546; http://bit.ly/3VxIHqg.) There is another smaller, albeit low-quality study that suggests that manual insertion techniques with butterfly needles may have higher success rates. One study reported manual success rates of 61 percent for butterfly needles, 43 percent (95% CI:23.4%-65.0%) for hand-twisted EZ-IO screwing, and 39.7 percent for the semi-automatic drill (Arrow EZ-IO). (Resuscitation. 2018 Jun;127:79.)

Be aware of the challenges of using the drill. This video documents my own failure with the EZ-IO driver during a neonatal resuscitation​​​. Manual insertion of IO needles may be the faster tool for vascular access, but it is not clear whether this is clinically significant. Nevertheless, I believe we should add manual IO needles back to our intravenous resources for newborns and neonates.

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We learned recently that one brand of manual IO needles commonly used for neonates had been recalled, but we learned that the needles sold with the EZ-IO drill are also approved for manual application. Problem solved!