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Here are some of the comments that were posted back then:
"Furthermore, her agitation was quickly labeled hysteria when it quite clearly could also have been due to pain or delerium or both."
"However, even the best approach does not guarantee that the diagnosis of SS disease would have been made."
"I would probably have missed the diagnosis for two reasons -- I may not have noted the clitoral prominence and I may not have thought of clitoral prominence as being equivalent to a state of priaprism, and I therefore may not even have considered the diagnosis of SS disease as a diagnostic possibility."
"A low hb would probably have raised some eyebrows."
"A very interesting part of this is also the absolutely LOUSY job done in teaching people to do mental status exams. I see this all the time, where an agitated individual is sluffed off as having emotional problems."
"Short cuts like the so-called mini mental status exam (50% error rate) are taught instead of teaching the mse, and what its component parts mean. A true mse is as sensitive as any other part of the physical exam."
"If you read the book by Stub and Black on the MSE, what you see is not magic, but a very systematic way of approaching the exam with full understanding of what each part of the exam actully measures."
"I am aware that I may seem foolish in venturing any opinion since I am not an ER doc, and also this unfortunate particular case is really a rather scary and emotional eye-opener for any doc regardless of specialty, but for teaching purposes: a little tiny girl in the ER is screaming non-stop apparently and cannot be comforted; moreover there is high suspicion for a sexual assault, and this in itself can engender strong feelings from the examining doc maybe to the point that he doesn't want to be more invasive toward the child by attempting to do a more "forcible" lung auscultation or abdomen palpation or order blood tests or even order a temp to be taken (even if only auricular and not rectal). Nevertheless, as described, the child seems that she cannot be comforted and continues to cry and squirm or scream, and this is somehow assumed to be all "psychological" (due to the possible rape) rather than physiological response to unbearable pain (like in a sickle crisis or other potential horrible abdominal complication or co-occuring disease). At the same time, the genitalia externally only appear mildly tumefied and erythematous but not obviously traumatized. At this point, some alarm bell regarding another possible etiology for the pain should maybe have rung. And obviously, if any alrm bell would have rung, one would have become somewhat more invasive/aggressive re. observation and description of demeanor and mental status and other points of a full physical exam and maybe blood tests. But, just think, a tiny tot screaming, described as "hysterical" (I somehow hope that this was not the term actually recorded in the doc's notes), moreover mom undoubtedly distraught and everyone horrified re. sexual assault suspicion. One can understand how everyone in that ER just wanted to get the little girl out of there, somewhere where (one assumes) the issue of the sexual assault will be taken care of, and no one in that ER will have to continue to bear either her distress or her screams or the horrible repugnance and distress anyone feels when confronted with a potential sexual assault on such a helpless child.
I guess, in a roundabout way, what I was trying to get at is that for teaching purposes, it may be helpful to explore the so-called "counter-transferential" feelings that an ER doc may have felt and acted upon during his decision-making process, and, by extension, how such strong feelings may have a bearing towards any assessment of any potentially difficult or distressing patient presenting to the ER (yes, drug seekers included). In any case, a good rule of thumb that I have given some of my medical students that I've taught during their psych. rotation, is that when they get a strong "vibe" (either positive or negative or distressing) about a particular patient, then it is really time to think about it, explore a little bit more re. potential causes for the strong feeling, document and assess well, both medically and psych. mental status wise-demeanor, etc. What struck me in the beginning re. this case was the "unconsollable" nature of the crying, and I though about abdominal pathology (just like one suspects an infant with intestinal intussusception or obstruction). (I didn't think of sickle cell at all, either)."
"I do not think the lesson from this case should be: The MSE requres lab tests on all patients so as not to miss some rare and atypically presenting condition.
What lessons do you think are reasonable in this case?"
"Well, the fact alone that this has generated a pretty lively discussion in this forum, with opinions ventured forth from physicians from all specialties (including psych)-made me think that this is a potential good teaching case for an ER resident. Not strictly regarding the medical zebra-type presentation (sickle cell crisis presenting with female clitoral enlargement), since indeed this would definitely be a very rare occurence that would be unlikely to come across their next ED encounters. But more from the perspective of the "narrow diagnostic focus" as you have mentioned, and ways to guard oneself against superficial judgments and potential misses-especially in today's ED context- where one does tend to unfortunately see a lot of patients with major social problems which may sometimes cloud the issue or lead to premature "labels" and "jumps to a potentially erroneous conclusion". The most common cases that I'm sure the residents have already heard about, would involve, for example acutely drunk agitated patients with some mental status alterations and co-morbid subdural hematomas or pneumonias, or even fractures, especially if the patient is already "known" to the ER as an old alcoholic frequently coming in to "sleep it off", or for "a hot meal", etc.
Also, since this case involved a little girl in pain and distress and the horrific suspicion for sexual assault- how does one cope in general with doing a good medical exam and history taking for ED clearance while coping with the feelings of outrage and distress engendered by this case and while waiting at the same time for input from social services (which may have completely different agendas and priorities than the treating doc)."
"I absolutely agree that the inclusion of agitated delirium with organic cause is critical to avoid missing organic pathology that is frequently present in patients with altered mental status. I also agree that the typical mental status exam we perform in the ED setting is generally not much more than a glorified GCS determination which will NOT detect delirium unless a more focused exam is performed that includes a full set of vitals.
In the May 2000 issue of Emerg Med Clinics of North America, Williams cites a 1994 study (small numbers & I can't recall the specific citation) which found that greater than 60% of patients presenting with new psychiatric complaints had an organic cause for their condition.
Had the diagnostic category of altered mental status/agitated delirium been included in this case, a more vigorous attempt to at least obtain a full set of vitals would have probably resulted in the elevated temp being identified and that in turn might have lead to more diagnostic studies as Paul as suggested that would have cinched the diagnosis.
I agree with Paul that a good Hx/PMH/FH might have clued the doc into possible sickle cell as a working diagnosis before any diagnostic studies.
Although anecdotal, I find that my personal practice follows Paul's comment regarding the inverse relationship of ease of examination and diagnostic studies. I tend to order more diagnostic studies when barriers exist (language and exam issues such as intoxication, beligerence, etc) to a reasonable H&P.
You have all made good points, and I think by looking at this case as an example of how an agitated delirium can present, there are lessons to be learned beyond the rare condition of sickle-cell induced clitoral priapism."
"You know what I think the greatest take home message is from all of this?
We are human. We have been given intensive training to recognize disease states. Do your very best to NOT be politically correct and TRUST YOUR GUT. I teach that to my residents and invariably get the typical novice response: but the book says or the ddx SHOULD be...... . I don't fault them for it. When I was a rookie I thought far more rigidly b/c I did not have the experience under my belt to think outside the lines.
IMO the teaching pearl here [and this is a real Mikimoto] is to always ask why and not just jump on the bandwagon with what seems convenient and easy to dx/treat. A screaming child is no one's favorite, but all the more reason why a newbie should focus even more energy in the proper dx. And you know what you said -or was it Paul- about the inverse relationship of testing and difficulty struck a nerve. I find that I tend to spend more time and energy on the head-scratchers than the gimmes. Maybe OUR take home message is that we need to address the gimmes again and make sure they really are.
This one is going to my residents too. Especially in their forensic rotation with me!"