We evaluate many common conditions every day in the Peds ED. We become very comfortable with them. As we have discussed several times previously, part of our job is to remain every vigilant for those rare, yet dangerous, entities that present in a similar fashion to the common, benign conditions. Hyperbilirubinemia is often encountered. What might make you concerned for something more ominous than breastfeeding jaundice? When should you consider Biliary Atresia?
Biliary Atresia: Basics
- Inflammatory Destruction of the intrahepatic and extra hepatic bile ducts!
- Exact etiology is debated and being researched heavily.
- Biliary atresia is rare, but is the leading cause of liver transplantation in children.
- 5-7 / 100,000
- More common in Asia and Pacific regions – 10-32 / 100,o00
- Most frequent surgically correctable liver disorder in infancy.
- Early diagnosis is important! [Mieli-Vergani, 2009; Wadhwani, 2008]
- Median age at diagnosis for those with good outcomes = 52 days
- Median age at diagnosis for those with poor outcomes = 72 days
- Those diagnosed at >100 days had universally poor outcomes
- Surgical outcomes are better when performed at <60 – 90 days and worse after 90 days.
- Unfortunately, some children may not have even had their 1st visit to the Primary Care Physician by the time their condition has become critical.
Biliary Atresia: Two Forms
- Perinatal or Postnatal
- More common (90%)
- Possible infectious related
- Fetal or Embryonic
- Less common (10%)
- High frequency of associated malformations
- Cardiovascular defects
- Polysplenia or Asplenia
- Abdominal situs inversus
- Intestinal Malrotation
- Portal Vein and Hepatic Artery abnormalities
Biliary Atresia: Presentation
- Unfortunately, the initial presentation can be subtle.
- Issues that should raise your concern:
- Prolonged neonatal jaundice
- Physiologic jaundice in healthy, full-term newborns typically resolves by the 5th or 6th day.
- Jaundice appearing outside the 1st week of life should raise your concern.
- Conjugated Hyperbilirubinemia
- There are other causes (ex, neonatal hepatitis), but Biliary Atresia needs to be high on your list of concerns!!
- Liver disease manifesting as conjugated hyperbilirubinemia accounts for ~1 in 500 cases of jaundice in kids 2-4 weeks of age.
- Unconjugated hyperbilirubinemia can be associated with other bad conditions (ex, Sepsis, Gilbert’s Syndrome), so don’t get complacent if it is not conjugated.
- Get in the habit of always feeling for (and documenting) the liver edge!
- It can be helpful in so many critical childhood conditions (ex, Subtle Signs of Heart Failure).
- Acholic Stools
- Ask specifically about this. [Mieli-Vergani, 2009]
- Can be difficult for discern when mixed with urine.
- Stool cards have been successful in helping to alert to this diagnosis.
- Dark Urine
- May be difficult to discern also.
- Consider obtaining a urine when concerned.
- Failure to thrive, splenomegaly and ascites appear later, after the time when intervention is unlikely to be helpful. [Mieli-Vergani, 2009]
Biliary Atresia: Treatment
- Coagulopathy, if present, is responsive to Vitamin K
- Kasai Procedure
- Best predictor of success is age at surgery, emphasizing importance of early diagnosis [Mieli-Vergani, 2009]
- Good bile flow seen in 80% if operated on by 60 days of age.
- If operation is later, only 20-30% had good bile flow.
- 33-50% of those who undergo a Kasai will still require a liver transplant.
- Liver Transplant
- Used for those who fail Kasai procedure.
- Disease does not recur after liver allograft.
- Prior to the surgical strategies, there was a 100% mortality by 2 years of age… now 90% survive into adulthood.
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The Emergency Department is appealing to many of us because of the speed at which it occurs. We evaluate rapidly. We get results quickly (relatively anyway… often we get cranky because they aren’t rapid enough). We make decisions expeditiously. Our environment is loud and fast. The chaos and cacophony can be appealing to many, but can also be terrifying to others. Children with Autism, particularly, may not respond well to this environment and it is our job to adjust to them, otherwise everyone may end up having a bad day.
- Autism is a “neurobehavioral disorder”
- Affects children’s Social Communications and has Restricted Behaviors
- Large spectrum of disordered behaviors
- Social skills and behaviors can vary greatly between individuals.
- Many prefer well defined daily routines.
- Can lead to some being very anxious when placed in unfamiliar situations (like the ED)
- Cognitive ability can range from severe delay to gifted.
- Most prevalent childhood neurodevelopmental disorder.
- Increasing over the past several decades
- Possibly due to awareness and altered definitions
- Estimates range from 1 in 50 to 1 in 500
Autism in the ED
- Often have concomitant medical conditions:
- 79% of patients presenting to ED in one study had more multiple chronic medical conditions.
- Abdominal Complaints
- Seizure Disorders (prevalence of ~30% in one study)
- Behavioral Problems
- Children with autism have demonstrated an increased risk for head, face, and neck injury. [McDermott, 2008]
- There is evidence that children with Autism do not come to the ED frivolously. [Cohen-Silver, 2014]
- 71% of visits were deemed to have higher triage acuity levels.
- 15% presented for Neurologic Concerns and Seizures
- 15-25% presented for Gastrointestinal issues
- Dental issues are also another common reason for ED visits.
- Time measures: [Cohen-Silver, 2014]
- Patients with autism spent an average of 6 hours in the ED.
- They were placed in rooms in 1 hour.
- It took the provider almost 2 hours to do initial assessment.
- These may be typical numbers for your ED… maybe even better… but for a child who does not do well with new environments and alterations to schedule, this can cause significant distress.
Autism: Some Basic Tips
- Despite your desire to be expedient and quick, SLOW DOWN!
- Your typical pace of evaluation may be, in fact, counterproductive.
- Do not rush in and expect to examine the child.
- Enter slowly. Approach only after understanding the dynamic well.
- Taking your time early will potentially save time later.
- Parents know the chid best! Ask them!!
- The parents’ input in how to approach, speak with, and examine the patient is extremely valuable!
- Often they appreciate your understanding of this and will be very helpful to you!
- Asking about what has worked well previously (from simple examinations to full sedations) will save everyone time and frustration.
I once cared for a child who was vigorously resisting examination of her laceration. I asked the mother what we should do to try to calm the child. Simply placing a band-aid on the child’s knee (which was not injured) was her signal that all was safe. We would have never of thought of this and it calmed her immediately. ALWAYS ASK FIRST!
- Ask about stressors and triggers. [Shellenbarger, 2004]
- Some items and issues that you may not perceive as being alarming, may be to the patient with autism.
- Ask the family about this and try to eliminate and avoid them if possible.
- If unable to eliminate or avoid, ask the family how they typically deal with them.
- Ask what calms the child. [Shellenbarger, 2004]
- Obviously, this can be very valuable.
- Speak clearly and concisely.
- Should do this more for all patients… and family and friends too.
- Simple words work best.
- Do not assume that poor eye contact indicates poor attention. [Shellenbarger, 2004]
- The child may use different social cues and interactions.
Autism & Sedation
- Again, first ask the parents what has worked best for their child!
- Many will, unfortunately, already have had negative experiences with medicines.
- No need repeating the mistakes of the past.
- Know that children with neurodevelopmental disorders are at increased risk for airway compromise during sedations.
- Some advocate for the use of:
- Clonidine [Mehta, 2004]
- Mean time to achieve sedation = 58 min; Recover = 105 min
- May decrease BP and HR, but no instability seen.
- Dexmedetomidine [Lubisch, 2009]
- 7-fold greater affinity for alpha 2 vs alpha 1 receptors than clonidine.
- Elimination 1/2-life of 2-3 hours.
- Highly efficacious with good safety
- It is costly.
- No perfect solution…
- Tailor the therapy to the individual and the specific situation.
- Maybe a band-aid on the knee is all you will need.
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We all know that when you “hear hoofbeats, think of horses” and when you “see stripes, think of zebras.” Unfortunately, the horses and zebras often look similar. While tachypnea is most often due to a viral process like Bronchiolitis, or a Pneumonia, we must stay vigilant for it being one of those Subtle Signs of Heart Failure. Keeping a keen eye and ear on alert for these zebras is part of what our job demands. A child presenting with Back Pain should catch your attention just like a the child complaining of persistent leg pain. While it is most likely innocuous, it could also be a zebra getting ready to stampede: Osteosarcoma.
- In the USA, cancer is the second leading cause of death in children.
- Leukemia leads all other cancers – ~50 per 1,000,000 (CDC Stats)
- Osteosarcoma incidence – ~5 per 1,000,000 (CDC Stats)
- Osteosarcoma is the most common primary bone tumor in patients < 40 years of age. (Haddox, 2015)
- Other important primary bone tumors = Ewing and Chondrosarcomas
- High rates of metastasis.
- Vast majority of osteosarcomas in children are High Grade. (Gorlick, 2010)
- Survival has improved with advances in surgical, chemotherapeutic, and radiation care.
- Before 1970’s, 5-year survival was < 20%.
- Now it approaches 70%.
- Generally, children have a better prognosis compared to patients 18-40 years of age. (Haddox, 2015)
- Peak frequency = onset of puberty
- Most arise in the intramedullary space of the metaphysis. (Gorlick, 2010)
- Most often in the long bones of the lower extremities.
- ~50% involve the knee (Distal Femur and Proximal Tibia)
- Proximal Humerus is next most common site.
- Possible relationship with Growth Plates.
- In older pts, it is more common in the axial skeleton.
- Spine involvement only in 4-5% of cases.
- Primary complaint = Pain.
- Often insidious
- Pain with activity is most common complaint.
- Just like everything else that hurts… it hurts more when you use it.
- “Growing Pains” is often the original misdiagnosis.
- May present with limp due to pain.
- Larger lesions may present with palpable mass.
- Uncommon to have night sweats, fever, or other systemic symptoms initially.
- Pathologic Fracture (Lee, 2013)
- Can cause the initial presentation (5 – 12%)
- Can also develop during treatment
- Associated with poorer prognosis.
- Appearance on Radiographs
- Lytic and blastic bone lesion
- “Sunburst” appearance
- Periosteal elevation related to soft tissue mass producing “Codman’s Triangle.” (Gorlick, 2010)
- Metastasizes to the Lungs and other Bones primarily.
So the next time you see the pre-teen who is complaining of knee pain after running in gym and you really want to blame “growing pains” or a minor strain… just pause and consider that those hoofbeats you hear are actually those of the stampeding Osteosarcoma Zebra.
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Musculoskeletal complaints are common in the pediatric Emergency Department. As I sit here watching March Madness Basketball (for those not in the US, that is our college basketball tournament, during which my wife consistently humiliates me by picking the winner), I was inspired to write this Morsel. We have covered several Orthopaedic topics in the past (ex, Toddler’s Fracture, Plantar Puncture Wounds, Supracondylar Fracture, Clavicular Head Dislocation), but one of the most commonly encountered is Osgood Schlatter’s Disease.
Osgood Schlatter’s Disease: Basics
- Common cause of knee pain in children
- 20% of children who participate in sports are affected
- Believed to be an overuse injury
- Tension at the insertion site of the patellar tendon on the anterior tibial tubercle
- Repetitive use before the closure of the epiphysis can lead to traction injury and microavulsion of the tubercle
- Occurs most often during rapid growth phase
- Boys: 13-15 years of age
- Boys more commonly affected (Hanada, 2012)
- Girls: 11-13 years of age
- Amongst basketball players, females had more overall knee injuries than males, but had lower rates of Osgood Schlatter’s Disease. (Ito, 2015)
- Patellofemoral Dysfunction is more common than Osgood Schlatter’s Disease in females. (Foss, 2014)
- Body Mass found to be associated with severity
- Lower weight and lower BMI had lower severity on radiographs (Hanada, 2012)
- Self-limited condition
- On rare occasions can lead to persistent pain
- Extremely rare to require surgical intervention
- Can develop associated bursitis
Osgood Schlatter’s Disease: Presentation
- Pain at the anterior tibial tubercle
- Pain worse with running, jumping, climbing stairs
- Often associated with basketball, soccer, football, ballet, figure skating, gymnastics
- Pain improves with rest
- Has normal range of motion and gait
- Tenderness over tibial tubercle
- Ocassional warmth and erythema of this area as well (it is an inflammatory process)
Osgood Shlatter’s Disease: Evaluation
- The diagnosis does not require any specific testing
- Radiographs are often obtained to rule-out other conditions (ex, malignancy)
- Lateral x-ray may show:
- Thickening of the patellar tendon
- Thin, anterior ossicles / fragment separating from the tibial tubercle
Osgood Schlatter’s Disease: Treatment
- Basic management is conservative.
- Conservative management does not mean that you “do nothing.”
- Decreased Activity
- Not, NO ACTIVITY!
- Do not recommend just sitting on couch, watching TV (unless it’s during March Madness and there are multiple games to watch).
- Recommend that they decrease their activities to the point where pain is improved and then begin adding back in activity as it is tolerated.
- Cross training may help (ex, swimming)
- I usually tell them to pretend like they are Michael Jordan and need to ice down their knees.
- Good trick is to fill small paper cups with water and freeze them. Then they can peel the paper and then use that to apply directly over the tibial tubercle.
- Physical Therapy
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Simple answers are always better than complicated ones. Often we get caught focusing on the complex and can forget that Basics are the Best (ex, Pediatric Shock Index, Optimize Chest Compressions, and Penicillin for Pneumonia)! Often, I have found myself knee deep in complicated decisions only to be recued by a straightforward answer. One such scenario is the “Hair Tourniquet Syndrome” case… is there a simple answer or do I have to perform surgery?
Hair Tourniquet Syndrome
- Caused by more than just Hair.
- We have discussed the unique situation of Metal Band Tourniquet.
- Hair – one study did show that 95% of cases were due to hair. [Claudet, 2010]
- Fine thread
- Thin rubber bands
- Many advocate for this condition to be termed “constricting tourniquet” or “hair-thread tourniquet.” [Plesa, 2015]
- Circumferential constriction can lead to substantial injury.
- Lymphatic drainage is initial obstructed, leading to edema.
- Increasing edema eventually impedes venous flow.
- Venous obstruction further increases swelling and can eventually obstruct arterial blood flow… that leads to badness.
- Tissue necrosis
- Non-healing ulcer
- Process can take hours, days, weeks, or even months.
- May go undetected, especially in the very young (who’s discomfort is often difficult to decipher – consider this in the fussy/crying infant).
- The swollen tissue can obscure the etiology!
- As the process continues, the hair/thread cuts into the skin.
- The skin can scar, cover over the thread, and obscure the etiology.
- Often involves distal appendages in the young. [Barton, 1988]
- Toes – 43%
- External Genitalia – 33%
- Fingers – 24%
Hair Tourniquet Syndrome – Management Options
- Need to remove the constricting band as soon as possible.
- Unwind the Thread
- Certainly, if caught early enough, you may be able to simple unwind the thread.
- Occasionally, using a curved needle to get under the thread can assist in this.
- Need to ensure that the entire amount is removed and that no residual constrictive portion remains.
- Depilatory Cream [O’Gorman, 2011; Plesa, 2015]
- Cream with thioglycolate and calcium hydroxide or sodium hydroxide (Brand Name = NAIR)
- The thioglycolate will break down the disulfide bonds of keratin, dissolving the hair.
- The alkaline component assists with penetration into the hair.
- Found to dissolve hair within 2.5 minutes and 8 minutes (depending on hair thickness). [Plesa, 2015]
- The manufacturer does not recommend application on open skin… so a deep laceration may not be the best place to apply it.
- Can cause skin irritation and even minor burns (it is an alkali).
- DOES NOT dissolve cotton, polyester, or rayon threads. [Plesa, 2015].
- Incise the Thread
- A DORSAL incision, along the long axis, down to the bone of the digit is recommended. [Barton, 1988; Serour, 2003]
- Lateral incisions may injury nerve or blood vessel.
- Incision along long axis may incise extensor tendon, but should not affect the tendon’s function.
- Incision down to the bone should ensure transection of the constrictive thread.
- Low threshold for surgical exploration
- Incision needs to avoid the urethra in the corpus spongiosum.
- Incise on one of the lateral, inferior aspects between the corpus cavernosum and the corpus spongiosum. [Barton, 1988]
Hair Tourniquet Syndrome – Management Plan
This is submitted as a reasonable option…
- Treat pain! (remember even neonates feel pain!)
- Topical anesthetic may help initially.
- This will also help if an incision is needed.
- Could also consider a digital block.
- Can you grasp the thread?
- If able, carefully remove it and ensure no residual material remains.
- This can be challenging with small digits and swollen tissues.
- Is there a deep laceration?
- Applying topical depilatory cream may cause more pain, so may want to proceed directly to incision.
- Unable to grasp/unwind, but no deep laceration, then apply Depilatory Cream.
- Since the majority of the threads will be hair, application of depilatory cream is reasonable.
- The cream should work within 10 minutes.
- After 10 minutes, rub the area and rinse with water. If no improvement, proceed to incision.
- Consider surrounding structures!
- Consultation for surgical exploration is required if there is any concern that there is continued constriction.
- Topical antibiotic cream if there is evidence of skin breakdown.
- Close Follow-up is appropriate.
- If evidence of vascular compromise, consider emergent Consultation.
- If good cap refill and improvement after management, close follow-up as outpatient is reasonable.
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Fortunately, the critically ill child is not as common in the Emergency Department as the critically ill adult. Unfortunately, when the critically ill child does arrive, it can be challenging to recognize him/her initially. This can lead to delays in resuscitation care. Even at the extreme point of being pulseless, children can be tricky (See Palpation of Pulse). Then there is always the challenge that having to account for the effect that age has on normal vital signs (See Blood Pressures). Often, though, by focusing on the Basics, we can met the challenge of detecting Pediatric Shock and act aggressively to treat it!
- Broadly speaking, shock is the state in which there is a failure to meet the metabolic demands of the body leading to anaerobic metabolism. (Mtaweh, 2013)
- Often categorized as:
- Toxin mediated – Septic
- Hypersensitivity reaction – Anaphylaxis
- Loss of sympathetic tone – Neurogenic
Pediatric Shock: A Challenge
- The diagnosis is initially suspected based upon clinical exam.
- There is no lab value or “test” that defines shock. (See Lactate)
- Clinical Findings:
- Must account for age-adjusted values!
- Often children present with elevated heart rates without overt illness.
- Poor Capillary Refill
- Normal capillary refill can vary with age and is influenced by the environment. (Schriger, 1988)
- The initial cap refill in the ED, may artificially affected by the pre-hospital environment.
- Peripheral Pulse Quality
- Altered Mental Status
- Cold/Mottled Extremities
- Poor Urine Output
- Not likely useful in the initial assessment in the ED.
- If the patient is “hanging out” in your ED for some time, monitor this!
- Of these clinical findings, only Altered Mental Status and Poor Peripheral Pulse Quality was associated with development of Organ Dysfunction. (Scott, 2014)
- No single finding defines shock, but the absence of all of them is reassuring.
Pediatric Shock: The Shock Index
- The Shock Index (Heart Rate / Systolic BP) has been shown to be useful in detecting adult patients with shock.
- There is evidence that the Shock Index can be useful in pediatric patients also. (Yasaka, 2013; Rousseaux, 2013)
- Since, pediatric vital signs alter with age, it would make sense to have a “adjusted” tool. (Acker, 2015)
- Using standard heart rate and systolic BP values for age ranges, Maximum Normal Shock Index values were calculated.
- Shock Index, Pediatric Adjusted (SIPA)
- 4-6 years = 1.2
- 6-12 years = 1
- > 12 years = 0.9
- Comparing the patient’s actual HR / Systolic BP to the SIPA was shown to perform better and identify those most severely injured following blunt trauma. (Acker, 2015)
- Obviously, this may not apply to all pediatric patients presenting with shock, but I do like the concept of utilizing Basic information that is age adjusted.
- Consider utilizing this tool as another method to help find those subtle presentations of shock. Remain Vigilant!
Dellinger RP1, Levy MM, Rhodes A, Annane D, Gerlach H, Opal SM, Sevransky JE, Sprung CL, Douglas IS, Jaeschke R, Osborn TM, Nunnally ME, Townsend SR, Reinhart K, Kleinpell RM, Angus DC, Deutschman CS, Machado FR, Rubenfeld GD, Webb S, Beale RJ, Vincent JL, Moreno R; Surviving Sepsis Campaign Guidelines Committee including The Pediatric Subgroup. Surviving Sepsis Campaign: international guidelines for management of severe sepsis and septic shock, 2012.
Intensive Care Med. 2013 Feb;39(2):165-228. PMID: 23361625
. [PubMed] [Read by QxMD]
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