Linear Skull Fracture

Linear Skull FractureWe all know that traumatic brain injury is a significant concern when evaluating pediatric patients with head injury. Over the years, this concern has lead to significant shifts in imaging and management practices: some for the better and… others perhaps not. Fortunately, while assessing minor head injuries, the risk of medical radiation is now being better balanced with the appropriate concern for traumatic intracranial injury.  We know that not everyone benefits from a head CT.  Some events, are not minor though and some do cause injuries.  Certainly, if there is intracranial hemorrhage, we need to have heightened concern, but there is still a question as to what is beneficial to do for those who are found to have an isolated, linear skull fracture without other injuries.  Do these patients need further imaging? Do they benefit from transport to tertiary center? Do they even benefit from hospitalization? Let us look at the Isolated Linear Skull Fracture:


Head Injury: Basics

  • Physics works and kids aren’t coordinated.
    • The young child’s disproportionately larger head, decreased agility, and weaker muscles leads to the head striking surfaces in unprotected fashions more commonly than adults.
    • Gravity works (at least for the time being).
  • Most head injuries are mild, but still traumatic brain injury is a leading cause of death in children.
  • Fortunately, kids with a normal neurologic exam after head injury rarely require neurosurgical intervention! [Lyons, 2016; Schunk, 1996]


Skull Fracture

  • Skull fracture is the most common traumatic finding in kids with abnormal imaging after head injury.
    • Younger children (< 2 years of age, especially infants < 1 year of age) are particularly at risk for skull fracture.
    • Majority of skull fractures can be managed conservatively. [Bonfield, 2014; Mannix, 2013]
  • Some skull fractures are more concerning:
    • Skull fracture near major blood vessels
    • Depressed skull fracture
    • Presence of pneumocephalus
    • Presence of intracranial hemorrhage (of course)
  • Imaging
    • Plain radiographs
      • Can be useful, but, obviously, do not describe any intracranial complications.
      • In low risk patients, a normal skull x-ray may be helpful.
      • CT is generally preferred, especially for depressed fractures or diastatic skull fractures. [Kim, 2012]
    • Bedside Ultrasound
      • Has been used to define skull fracture in the ED. [Parri, 2013]
    • Rapid MRI
      • Rapid MRI protocols are useful in evaluation of hydrocephalus (see VP Shunt evaluation).
      • Rapid MRI has been shown to detect traumatic injuries with similar sensitivity and specificity as CT in followup imaging. May be what is done more commonly in the future. [Mehta, 2016]
    • CT
      • Generally considered the diagnostic method of choice, right now.
      • It is able to define the skull fracture and any associated  intracranial injuries.
      • Repeat Head CT, often performed, is not be beneficial in an asymptomatic kid with skull fracture. [Zulfigar, 2016; White, 2016; Hentzen, 2015]
      • May need to consider repeat imaging if there is neurological decline or concern for spinal fluid leakage. [White, 2016; Hentzen, 2015]
  • Dispostion:
    • Linear Skull Fracture, No Intracranial Hemorrhage on CT, and Normal Neurologic Exam;
      • Have a low risk for complications. [Blackwood, 2016; Addidoi, 2016; Arrey, 2015; Mannix, 2013]
      • These patients may be appropriate for discharge from the ED after brief period of observation in ED. [Addidoi, 2016;  Lyons, 2016; Rollins, 2010]
      • In the absence of concern for NAT, these patients also do not benefit from transfer to a level one trauma center. [White, 2016]
    • Depressed/Displaced skull fracture, fracture with altered mental status, or concern for NAT = Consult Neurosurgery and hopitalize. [Addioui, 2016]



  • Sometimes, unfortunately, the skull fracture is not due to Gravity, but rather something more sinister.
  • Non-accidental trauma should always be considered, particularly in the young who cannot communicate effectively.
  • Things that should raise your concern for NAT include:
    • Vague explanation
    • Blaming another child or sibling, particularly one that isn’t developmentally able to do the feats described.
    • Inconsistent history
    • Delays in seeking care
    • Prior history of injuries
    • Bruising over areas without bony prominences
  • Always be mindful that there may be other occult injuries.
  • Obviously, if there is concern for NAT, even a simple linear skull fracture without intracranial hemorrhage should be hospitalized.


Moral of the Morsel

  • We (especially in the USA) likely admit too many children who do not benefit from the hospitalization.
  • A period of 4-6 hours (cuz that is the magic amount of time for everything) of observation in the ED and close outpatient follow-up may be most prudent.
  • A child with a normal neurologic exam and a linear, non-depressed skull fracture without intracranial hemorrhage does not benefit from:
    • Repeat imaging
    • Hospitalization for observation
    • Transfer to trauma center
  • Try to only hospitalize patient who will benefit from it.
    • Symptom management may require admission.
    • Concern for non-accidental trauma! requires admission.
    • Consider social dynamics – ensure that the child can  return to the hospital should she/he become sicker.



Lyons TW1, Stack AM2, Monuteaux MC2, Parver SL2, Gordon CR2, Gordon CD2, Proctor MR3, Nigrovic LE2. A QI Initiative to Reduce Hospitalization for Children With Isolated Skull Fractures. Pediatrics. 2016 Jun;137(6). PMID: 27244848. [PubMed] [Read by QxMD]

Addioui A1, Saint-Vil D2, Crevier L3, Beaudin M4. Management of skull fractures in children less than 1 year of age. J Pediatr Surg. 2016 Jul;51(7):1146-50. PMID: 26891833. [PubMed] [Read by QxMD]

Mehta H1, Acharya J2, Mohan AL3, Tobias ME3, LeCompte L2, Jeevan D3. Minimizing Radiation Exposure in Evaluation of Pediatric Head Trauma: Use of Rapid MR Imaging. AJNR Am J Neuroradiol. 2016 Jan;37(1):11-8. PMID: 26381555. [PubMed] [Read by QxMD]

Blackwood BP1, Bean JF2, Sadecki-Lund C3, Helenowski IB4, Kabre R5, Hunter CJ6. Observation for isolated traumatic skull fractures in the pediatric population: unnecessary and costly. J Pediatr Surg. 2016 Apr;51(4):654-8. PMID: 26472656. [PubMed] [Read by QxMD]

Zulfiqar M1, Kim S1, Lai JP2, Zhou Y3. The role of computed tomography in following up pediatric skull fractures. Am J Surg. 2016 Aug 16. PMID: 27614418. [PubMed] [Read by QxMD]

White IK1, Pestereva E1, Shaikh KA1, Fulkerson DH2. Transfer of children with isolated linear skull fractures: is it worth the cost? J Neurosurg Pediatr. 2016 May;17(5):602-6. PMID: 26722759. [PubMed] [Read by QxMD]

Arrey EN1, Kerr ML1, Fletcher S1, Cox CS Jr1, Sandberg DI1. Linear nondisplaced skull fractures in children: who should be observed or admitted? J Neurosurg Pediatr. 2015 Dec;16(6):703-8. PMID: 26339955. [PubMed] [Read by QxMD]

Hentzen AS1, Helmer SD2, Nold RJ3, Grundmeyer RW 3rd1, Haan JM4. Necessity of repeat head computed tomography after isolated skull fracture in the pediatric population. Am J Surg. 2015 Aug;210(2):322-5. PMID: 25907850. [PubMed] [Read by QxMD]

Orman G1, Wagner MW1, Seeburg D1, Zamora CA2, Oshmyansky A1, Tekes A1, Poretti A1, Jallo GI3, Huisman TA1, Bosemani T1. Pediatric skull fracture diagnosis: should 3D CT reconstructions be added as routine imaging? J Neurosurg Pediatr. 2015 Oct;16(4):426-31. PMID: 26186360. [PubMed] [Read by QxMD]

Bonfield CM1, Naran S, Adetayo OA, Pollack IF, Losee JE. Pediatric skull fractures: the need for surgical intervention, characteristics, complications, and outcomes. J Neurosurg Pediatr. 2014 Aug;14(2):205-11. PMID: 24905840. [PubMed] [Read by QxMD]

Mannix R1, Monuteaux MC, Schutzman SA, Meehan WP 3rd, Nigrovic LE, Neuman MI. Isolated skull fractures: trends in management in US pediatric emergency departments. Ann Emerg Med. 2013 Oct;62(4):327-31. PMID: 23602429. [PubMed] [Read by QxMD]

Parri N1, Crosby BJ, Glass C, Mannelli F, Sforzi I, Schiavone R, Ban KM. Ability of emergency ultrasonography to detect pediatric skull fractures: a prospective, observational study. J Emerg Med. 2013 Jan;44(1):135-41. PMID: 22579023. [PubMed] [Read by QxMD]

Kim YI1, Cheong JW, Yoon SH. Clinical comparison of the predictive value of the simple skull x-ray and 3 dimensional computed tomography for skull fractures of children. J Korean Neurosurg Soc. 2012 Dec;52(6):528-33. PMID: 23346324. [PubMed] [Read by QxMD]

Rollins MD1, Barnhart DC, Greenberg RA, Scaife ER, Holsti M, Meyers RL, Mundorff MB, Metzger RR. Neurologically intact children with an isolated skull fracture may be safely discharged after brief observation. J Pediatr Surg. 2011 Jul;46(7):1342-6. PMID: 21763832. [PubMed] [Read by QxMD]

Schunk JE1, Rodgerson JD, Woodward GA. The utility of head computed tomographic scanning in pediatric patients with normal neurologic examination in the emergency department. Pediatr Emerg Care. 1996 Jun;12(3):160-5. PMID: 8806136. [PubMed] [Read by QxMD]

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Honey for Cough

Honey for CoughCough is a very common symptom in children. Certainly, we need to consider that an ominous cause may be lurking (Asthma, Occult Aspirated Foreign Body, Croup, Sinusitis, Pneumonia, Acute Chest Syndrome, etc.). Most often, though, the cough is due to a viral illness, but remember viral illnesses can become complicated (see, Myocarditis, Guillaine-Barre). So, take care not to be dismissive. Additionally, while you are doing your best to avoid the phrase “just a virus,” prepare yourself to address the parent’s next question: “What can be used to make the cough better?”  Unfortunately, there are not many medications that are safe to use in young children… but this may be an opportunity to “prescribe” Winnie the Pooh’s favorite: Honey for Cough.


Honey: Not for < 1 year of age

Before we get carried away by Winnie’s obsession… don’t overlook a real danger… Infantile Botulism

  • Infantile botulism is the most common form of botulism in the USA. [Brown, 2012]
  • Due to ingested Clostridium botulinum spores
    • Spores colonize the GI tract and the produce toxin.
    • Toxin prevents release of acetylcholine from the presynapse.
  • Presents with vague symptoms, but can be deadly (yup… your job is difficult).
    • Poor feeding
    • Constipation
    • Weakness (ex, weak cry, can’t hold head up) – Descending Weakness.
    • Hypotonia
    • Cranial Nerve palsies (ex, ptosis, poor suck, sluggish pupillary reflexes)
    • Respiratory Depression
      • ~50% of all infantile botulism cases require mechanical ventilation. [Brown, 2012]
    • May initially be first managed as if sepsis/serious bacterial infection is present, but child is afebrile and has negative cultures.
  • Honey consumption is the classically associated with infantile botulism, but as with many cases of “classical” medical associations, this is not the full story.
    • 85% of cases have no known honey exposure.
    • Botulinum spores are ubiquitous.
    • Honey should still be avoiding in children < 1 year of age… and don’t overlook honey being used as oral pacifier. [Benjamins, 2013]


Honey for Cough

Ok, now that we are clear that Honey should not be recommended for a child < 1 year of age, let’s get back to how it may help your older child with a cough!

  • Cough Medications from Over the Counter (OTC) [Paul, 2012]
  • Honey for Cough
    • Honey has been reported to have many health benefits (because the Bee Lobby is very active).
      • Antimicrobial activity
      • Wound Care
      • Post-Tonsillectomy Pain Management [Mohebbi, 2014; Boroumand, 2013; Ozlugedik, 2006]
    • Several small studies have shown the honey can compare favorably to the OTC cough preparations. [Cohen, 2016; Paul, 2012; Cohen 2012; Paul 2007]
    • Honey may:
      • Decrease cough frequency and severity
      • Improve child and parent sleep quality 
    • The literature may still have inadequacies [Allan, 2011], but honey is generally deemed safe with good side-effect profile.


Moral of the Morsel

  • Once again, don’t say “it’s just a virus.”
  • Be vigilant and think about ominous causes of cough… and think out loud.
  • Appreciate the persistent coughing, even if merely due to a viral infection, disturbs the entire household. Do not be dismissive of this.
  • Anticipate the question about what “medication” can be used to help.
  • Weigh the risks and benefits and know that the scales tip toward avoiding OTC cough/cold medications in the young.
  • The risk:benefit scales may not slant dramatically toward use of Honey, but as long as the child is > 1 year of age, it is safe so even some minor benefit may be worth it.  Plus… honey is delicious!



Cohen HA1,2, Hoshen M3, Gur S4,5, Bahir A4,6, Laks Y4,7, Blau H4,8. Efficacy and tolerability of a polysaccharide-resin-honey based cough syrup as compared to carbocysteine syrup for children with colds: a randomized, single-blinded, multicenter study. World J Pediatr. 2016 Jul 23. PMID: 27457790. [PubMed] [Read by QxMD]

Mohebbi S1, Nia FH2, Kelantari F2, Nejad SE2, Hamedi Y3, Abd R4. Efficacy of honey in reduction of post tonsillectomy pain, randomized clinical trial. Int J Pediatr Otorhinolaryngol. 2014 Nov;78(11):1886-9. PMID: 25193590. [PubMed] [Read by QxMD]

Benjamins LJ1, Gourishankar A, Yataco-Marquez V, Cardona EH, de Ybarrondo L. Honey pacifier use among an indigent pediatric population. Pediatrics. 2013 Jun;131(6):e1838-41. PMID: 23650307. [PubMed] [Read by QxMD]

Boroumand P1, Zamani MM, Saeedi M, Rouhbakhshfar O, Hosseini Motlagh SR, Aarabi Moghaddam F. Post tonsillectomy pain: can honey reduce the analgesic requirements? Anesth Pain Med. 2013 Summer;3(1):198-202. PMID: 24223362. [PubMed] [Read by QxMD]

Paul IM1. Therapeutic options for acute cough due to upper respiratory infections in children. Lung. 2012 Feb;190(1):41-4. PMID: 21892785. [PubMed] [Read by QxMD]

Cohen HA1, Rozen J, Kristal H, Laks Y, Berkovitch M, Uziel Y, Kozer E, Pomeranz A, Efrat H. Effect of honey on nocturnal cough and sleep quality: a double-blind, randomized, placebo-controlled study. Pediatrics. 2012 Sep;130(3):465-71. PMID: 22869830. [PubMed] [Read by QxMD]

Allan GM1, Korownyk C, Kolber M. Do cough suppressants or honey help pediatric cough? Can Fam Physician. 2011 Apr;57(4):435. PMID: 21490355. [PubMed] [Read by QxMD]
Ozlugedik S1, Genc S, Unal A, Elhan AH, Tezer M, Titiz A. Can postoperative pains following tonsillectomy be relieved by honey? A prospective, randomized, placebo controlled preliminary study. Int J Pediatr Otorhinolaryngol. 2006 Nov;70(11):1929-34. PMID: 16914210. [PubMed] [Read by QxMD]

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Traumatic HyphemaInjuries to the face are commonly encountered when caring for children. We have previously reviewed several facial injury topics (see, Mandibular FracturesDental Trauma, Tongue Lacerations, and Eyelid Lacerations).  Eye injuries can sometimes be under appreciated, especially when there are other associated injuries. One simple finding that we should pay particular attention to is the presence of a Hyphema.


Traumatic Hyphema: Basics

  • Ocular trauma is a leading cause of non-congenital, monocular blindness in children worldwide. [Yildiz, 2016; SooHoo, 2013]
  • Hyphema = blood in the anterior chamber of the eye. [Trief, 2013]
    • Deformity of the globe leads to displacement of the lens and iris, possibly tearing the ciliary body and/or iris vessels.
    • Bleeding will increase the intraocular pressure, which assists tamponading the bleeding along with clot formation.
    • Clot integrity is best 4-7 days after the injury.
  • Traumatic hyphema is more common in children than in adults. [SooHoo, 2013; Tries, 2013]
  • Most commonly occurs from blunt injury (~75%). [Trief, 2013]
    • Projectiles (like airsoft/BB guns, paintball guns) [Shazly, 2012]
    • Sports
    • Airbags deployed during MVC [Motlery, 2003]
    • Assault, Non-accidental Trauma [Calzada, 2003]
    • Miscellaneous items (hanger, towel, rubber toy snake) [SooHoo, 2013]


Traumatic Hyphema: Complications

  • Rebleed / second hemorrhage
    • Associated with a worse prognosis.
    • Typically occurs within first 4 days after injury.
  • Corneal staining (~5% of cases)
    • Can lead to amblyopia.
    • May require surgery to resolve.
  • Increased intraocular pressure
  • Synechiae
  • Glaucoma
  • Amblyopia
  • Visual Impairment [Yildiz, 2016]


Traumatic Hyphema: Evaluation

  • Don’t get distracted!
    • Evaluate for other associated traumatic injuries.
    • Evaluate for Open Globe Injuries!
      • The presence of a hyphema should heighten the concern for open globe injury.
      • History of lacerating injuries, small projectiles, or sharp objects also warrants greater concern for open globe injuries.
      • Look specifically for anisocoria and afferent pupillary defect.
      • A portable slit-lamp is a very useful tool!!  Ultrasound, used carefully, can also help evaluate globe integrity.
  • Check intraocular pressure
    • Only do this if confident that there is not open globe injury.
    • This can be challenging in children, but is very important.
  • Characterizing the hyphema can help communicate to consultants and helps to determine potential risk for complications.  [Trief, 2013]
    • Having patient sit upright will allow hyphema to settle.
    • Hyphemas can be characterized as Microscopic or Macroscopic.
    • Macroscopic hyphemas are graded by the height of the blood in the anterior chamber (AC).
      • Grade 1: Less than 1/3 of the AC; Best prognosis
      • Grade 2: 1/3 to 1/2 of the AC
      • Grade 3: 1/2 to nearly the entire AC
      • Grade 4: Fills the entire AC; Worse prognosis
  • Finish the complete eye exam.
    • Fundoscopic exam should be used to look for vitreous hemorrhage. [Trief, 2013]
    • Ultrasound can help characterize the posterior chamber, especially if there is a Grade 3 or 4 hyphema.
    • Visual acuity should also be documented.
  • History of Sickle Cell DiseaseSickle Cell Trait or other Bleeding Disorders (Hemophilia, Von Willebrands) should be considered.
    • Patients with sickle cell disease and trait are at risk for developing hyphema, even spontaneously.
    • It is important to inquire about possible sickle cell disease/trait in the family. [Trief, 2013]
    • Patients have been diagnosed with sickle cell disease/trait following traumatic hyphema. [SooHoo, 2013]


Traumatic Hyphema: Treatment

  • Outpatient care is most often successful. [SooHoo, 2013]
  • Basic care consists of:
    • Head of Bed 30-45 degrees.
    • Relative rest / limited activity
    • Avoiding Aspirin or NSAIDS.
    • Refraining from reading (or watching electronic devices up close) as accommodation can stress the injured vessels. [Trief, 2013]
    • Protective eye shield recommended by some.
    • Close Ophthalmology follow-up (sometimes daily).
  • Medication strategies include: [Trief, 2013]
    • Suppress aqueous production
      • Topical Beta Blockers
      • Carbonic anhydrase inhibitors (avoid if Sickle Cell Disease present)
    • Cycloplegics
      • Helps with comfort.
      • May reduced secondary hemorrhage risk.
      • Topical atropine, cyclopentolate, or scopolamine.
    • Steroids
      • Topical or systemic have been used.
      • Help to reduced inflammation and stabilize clot.
      • Avoid long-term use as it will increase risk of cataracts and glaucoma.
    • Antifibrinolytics
      • Decreases rates of secondary bleeding.
      • Aminocaproic acid is commonly used.
      • TXA has been found to be safe, although has less literature to show its benefits. [Albiani, 2008]
  • Inpatient care should be considered for patients with:
    • Sickle cell anemia/trait
    • Grade 3 or Grade 4 Hyphema
    • Penetrating ocular trauma
    • Secondary bleed
    • History concerning for abuse
    • Poor ability to adhere to the medical plan.
  • Surgery may be required in those who have:
    • Corneal staining
    • Uncontrolled increased intraocular pressures
    • Grade 4 hyphema that persists for >5 days
    • Large clots persisting > 10days



Yildiz M1, Kıvanç SA1, Akova-Budak B1, Ozmen AT1, Çevik SG2. An Important Cause of Blindness in Children: Open Globe Injuries. J Ophthalmol. 2016;2016:7173515. PMID: 27247799. [PubMed] [Read by QxMD]

Trief D, Adebona OT, Turalba AV, Shah AS. The pediatric traumatic hyphema. Int Ophthalmol Clin. 2013 Fall;53(4):43-57. PMID: 24088932. [PubMed] [Read by QxMD]
SooHoo JR1, Davies BW, Braverman RS, Enzenauer RW, McCourt EA. Pediatric traumatic hyphema: a review of 138 consecutive cases. J AAPOS. 2013 Dec;17(6):565-7. PMID: 24215806. [PubMed] [Read by QxMD]

Shazly TA1, Al-Hussaini AK. Pediatric ocular injuries from airsoft toy guns. J Pediatr Ophthalmol Strabismus. 2012 Jan-Feb;49(1):54-7. PMID: 21261240. [PubMed] [Read by QxMD]

Liu ML1, Chang YS, Tseng SH, Cheng HC, Huang FC, Shih MH, Hsu SM, Kuo PH. Major pediatric ocular trauma in Taiwan. J Pediatr Ophthalmol Strabismus. 2010 Mar-Apr;47(2):88-95. PMID: 20349901. [PubMed] [Read by QxMD]

Albiani DA1, Hodge WG, Pan YI, Urton TE, Clarke WN. Tranexamic acid in the treatment of pediatric traumatic hyphema. Can J Ophthalmol. 2008 Aug;43(4):428-31. PMID: 18711456. [PubMed] [Read by QxMD]

Salvin JH1. Systematic approach to pediatric ocular trauma. Curr Opin Ophthalmol. 2007 Sep;18(5):366-72. PMID: 17700228. [PubMed] [Read by QxMD]

Motley WW 3rd1, Kaufman AH, West CE. Pediatric airbag-associated ocular trauma and endothelial cell loss. J AAPOS. 2003 Dec;7(6):380-3. PMID: 14730288. [PubMed] [Read by QxMD]

Calzada JI1, Kerr NC. Traumatic hyphemas in children secondary to corporal punishment with a belt. Am J Ophthalmol. 2003 May;135(5):719-20. PMID: 12719088. [PubMed] [Read by QxMD]

The post Hyphema appeared first on Pediatric EM Morsels.


Puff of SmokeOne of the refreshing aspects of taking care of children is that, generally, they don’t have a mile long list of medications or an extensive known past medical history, unlike their adult counterparts.  Unfortunately, this generality can lead us to become complacent and be caught off guard when a child presents with concerning symptoms or has unusual medical problems. We have previously discussed several “adult” illness that occur in children (ex, Pulmonary Embolism, Cholelithiasis, Nephrolithiasis) and one of those surprising entities is Stroke (CVA). While children may have an AVM that leads to hemorrhagic stroke, one important condition that may be on their PMHx list and can lead to CVA in moyamoya.  Let’s take a moment to review moyamoya so we can remain vigilant!


Moyamoya: Basics

  • Moyamoya is a Japanese term that means “something hazy, like puff of smoke drifting in the air.” [Singhi, 2012]
    • Refers to the angiographic appearance of the abnormal collaterals that develop.
  • Cerebrovascular arteriopathy that leads to progressive stenosis and eventual occlusion.
    • It is not inflammatory and not due to atherosclerosis.
    • Stenosis is due to progressive intimal thickening and thrombosis formation.
    • Affects internal carotid, middle cerebral artery, and anterior cerebral artery [Singhi, 2012; Kim, 2010]
    • Can also affect posterior cerebral artery [Lee, 2015; Lee, 2014]
  • Rare cerebrovascular occlusive disease, but incidence varies between ethnic groups. [Smith, 2009]
  • Occurs in all ethnic groups.
  • Bimodal distribution: [Singhi, 2012]
    • One peak in childhood – usually before 10 years of age.
    • Second peak occurs during 4th decade of life.
  • Unknown cause
    • Likely combination of environmental and genetic factors
    • One large study found only 12% of cases to be familial. [Kim, 2010]


Moyamoya: What’s in a Name?

“Moyamoya,” “moyamoya syndrome,” “moyamoya disease” may be confused.

  • Moyamoya – generic term for the angiographic findings
  • Moyamoya Disease – bilateral, angiographic findings without associated clinical cause (i.e., idiopathic)
  • Moyamoya Syndrome – unilateral moyamoya or moyamoya found in association with systemic disorders that are known to be associated with moyamoya. [Singhi, 2012; Smith, 2012]
    • Genetic Disorders:
    • Hematologic Disorders:
    • Hyperthryoidism
    • Congenital Heart Disease
    • Tubercular meningitis
    • Previous cranial therapeutic radiation


Moyamoya: Presentation

  • There are 4 main types of moyamoya based on clinical manifestations:
    • Infarct Type
      • Motor deficits (in one study, ~95% of cases) [Singhi, 2012]
      • Hemiparesis (44%) [Singhi, 2012]
      • Sensory symptoms
      • Some patients will present with ischemic symptoms precipitated by crying, coughing, blowing, or hyperventilation (which leads to hypocapnia and vasoconstriction) [Smith, 2009]
    • Transient Ischemic Attacks (TIAs) Type
      • Repeated TIAs is a presentation that needs to be respected.
    • Hemorrhagic Type
      • Hemorrhage is rare in children, but does occur. More common in adults. [Singhi, 2012]
    • Epileptic Type
      • May be initial presentation or recurrent in patient with known Moyamoya
      • Seizures on presentation rates vary: ~34% in [Singhi, 2012]; 19% in [Kim, 2010]
      • Surgery shown to help reduce recurrent seizure activity (97% seizure free)  [Kim, 2010]
  • Other commonly encountered symptoms:
    • Headaches (33%) [Kim, 2010]
      • Surgery does not as greatly affect headache recurrence (26% still w/ headaches, and 16% developed new headaches) [Kim, 2010; Seol, 2005]
    • Fever
      • 30% presented with fevers [Singhi, 2012]
      • Unclear association, but fever can lead to carotid artery constriction.


Moyamoya: Evaluation

  • Gold standard for evaluation is Intra-arterial cerebral angiography. [Singhi, 2012]
    • Better able to fully image the blood vessels.
    • Helps with correlation of Stage of Disease.
      • Stages progress from Stage 1 to Stage 6
      • Staging based on progressive stenosis and eventual occlusion of internal carotid artery (ICA) along with development of moyamoya collaterals at base of brain and development of external carotid artery (ECA) collateral vessels.
  • MRI/MRA has been found reliable and often used for screening for disease and monitoring, but surgery often based on intra-arterial angiography. [Singhi, 2012; Smith, 2012]


Moyamoya: Management

  • There is no effective pharmacotherapy to treat moyamoya. [Singhi, 2012]
  • Therapies aimed at underlying syndromes, if present, for Moyamoya Syndrome. [Smith, 2012]
  • Surgical revascularization to augment collateral circulation can be helpful.
    • Various techniques debated amongst neurosurgeons.
    • Typically reserved for patients with progressive ischemic symptoms or those with evidence of inadequate cerebral perfusion. [Singhi, 2012; Smith, 2012]
  • Prognosis is highly variable.
    • May have slow progression with intermittent events.
    • May have rapid neurologic deterioration.
    • Early diagnosis and active intervention before irreversible changes occur can lead to favorable outcomes. [Bao, 2014]
    • Sadly, even in the absence of CVA, there is still often a lower quality of life observed in the patient’s with Moyamoya. [Ball, 2016]


Moral of the Morsel:

  • Not every child has “no significant past medical history.” Some children have rather complex medical histories.
  • “Adult” medical problems can occur in children.
  • A child with a history of possible TIAs should not be dismissed… consider Moyamoya!
  • A child with Moyamoya may present with progression of disease… be aware of the increased risk for further ischemic disease.



Ball AJ1, Steinberg GK2, Elbers J3. Quality of Life in Pediatric Moyamoya Disease. Pediatr Neurol. 2016 Oct;63:60-5. PMID: 27473648. [PubMed] [Read by QxMD]

Rashad S1, Fujimura M2, Niizuma K1, Endo H1, Tominaga T1. Long-term follow-up of pediatric moyamoya disease treated by combined direct-indirect revascularization surgery: single institute experience with surgical and perioperative management. Neurosurg Rev. 2016 Oct;39(4):615-23. PMID: 27180559. [PubMed] [Read by QxMD]

Bao XY1, Duan L, Yang WZ, Li DS, Sun WJ, Zhang ZS, Zong R, Han C. Clinical features, surgical treatment, and long-term outcome in pediatric patients with moyamoya disease in China. Cerebrovasc Dis. 2015;39(2):75-81. PMID: 25573764. [PubMed] [Read by QxMD]

Lee JY1, Kim SK2, Phi JH2, Wang KC2. Posterior Cerebral Artery Insufficiency in Pediatric Moyamoya Disease. J Korean Neurosurg Soc. 2015 Jun;57(6):436-9. PMID: 26180612. [PubMed] [Read by QxMD]

Baltsavias G1, Khan N, Valavanis A. The collateral circulation in pediatric moyamoya disease. Childs Nerv Syst. 2015 Mar;31(3):389-98. PMID: 25378261. [PubMed] [Read by QxMD]

Lee JY1, Choi YH, Cheon JE, Paeng JC, Ryu HW, Kim KJ, Phi JH, Wang KC, Cho BK, Chae JH, Kim SK. Delayed posterior circulation insufficiency in pediatric moyamoya disease. J Neurol. 2014 Dec;261(12):2305-13. PMID: 25212285. [PubMed] [Read by QxMD]

Singhi P1, Choudhary A, Khandelwal N. Pediatric moyamoya disease: clinical profile, literature review and sixteen year experience from a tertiary care teaching institute. Indian J Pediatr. 2013 Dec;80(12):1015-20. PMID: 23525975. [PubMed] [Read by QxMD]

Smith ER1, Scott RM. Spontaneous occlusion of the circle of Willis in children: pediatric moyamoya summary with proposed evidence-based practice guidelines. A review. J Neurosurg Pediatr. 2012 Apr;9(4):353-60. PMID: 22462697. [PubMed] [Read by QxMD]

Kim SK1, Cho BK, Phi JH, Lee JY, Chae JH, Kim KJ, Hwang YS, Kim IO, Lee DS, Lee J, Wang KC. Pediatric moyamoya disease: An analysis of 410 consecutive cases. Ann Neurol. 2010 Jul;68(1):92-101. PMID: 20582955. [PubMed] [Read by QxMD]

Amlie-Lefond C1, Bernard TJ, Sébire G, Friedman NR, Heyer GL, Lerner NB, DeVeber G, Fullerton HJ; International Pediatric Stroke Study Group. Predictors of cerebral arteriopathy in children with arterial ischemic stroke: results of the International Pediatric Stroke Study. Circulation. 2009 Mar 17;119(10):1417-23. PMID: 19255344. [PubMed] [Read by QxMD]

Smith JL1. Understanding and treating moyamoya disease in children. Neurosurg Focus. 2009 Apr;26(4):E4. PMID: 19335128. [PubMed] [Read by QxMD]

Seol HJ1, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK. Headache in pediatric moyamoya disease: review of 204 consecutive cases. J Neurosurg. 2005 Nov;103(5 Suppl):439-42. PMID: 16302616. [PubMed] [Read by QxMD]

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Patellofemoral Pain Syndrome and Anterior Knee Pain

Patellofemoral Pain SyndromeWith childhood obesity becoming ever more prominent leading to potential medical issues (ex, Cholelithiasis, Eating Disorders) it is wonderful to encourage all of our kids to be more active, but activity and sports have their own potential issues as well.  Previously, we have discussed sport-related, overuse injuries like Little League Shoulder and Osgood Schlatter’s Disease as well as common orthopaedic injuries like Supracondylar Fractures and Shoulder Dislocations.  Let us take a moment to digest a tasty Morsel on another common extremity complaint: Patellofemoral Pain Syndrome.


Patellofemoral Pain Syndrome: Basics

  • The knee is bears a significant load during all activities.
  • The patella facilitates force of knee extension. [Houghton, 2006]
  • The patella also protects the patellar tendon from friction.
  • Patellofemoral Pain Syndrome is one of the most common causes of anterior knee pain in the primary care arena. [Yin, 2016; Pengel, 2014; Houghton, 2006]
  • Cause is multifactorial and varies between individuals. [Finlayson, 2014; Houghton, 2006]
    • Patellar malaignment
      • Larger Q angles associated with increased static patellofemoral joint stress.
      • Quadricep weakness (especially vestus medialis) also contributes to improper tracking of patella.
    • Mechanical overload of joint
      • Excessively jumping, running, etc.
      • Additionally, hip muscle weakness can increase load on the knee (the hip muscles can absorb up to 25% of the load that occurs during landing).
    • Decreased flexibility.
      • Inflexible muscles/tendons are less able to absorb eccentric loads .
      • Quadriceps, hamstings, hip flexors, gastrocnemius, and other soft tissues of the lower extremities all influence the patellofemoral joint.
    • Regional inflammatory changes.
  • Epidemiology:
    • Affects females > males [Stracciolini, 2014; Houghton, 2006]
    • More commonly seen in adolescents. [Pengel, 2014]
    • May be related to activity, but also seen without specific activity.
    • Can be unilateral or bilateral.


Patellofemoral Pain Syndrome: Presentation

  • Typically have poorly localized complaint of knee pain (“Knee Grab Sign“). [Pengel, 2014]
    • Dull and achy pain during and after activity. [Houghton, 2006]
    • Worse with weight bearing sports, squatting, or climbing stairs.
    • Also exacerbated upon standing after sitting for awhile.
  • Patient may report a sensation of “giving way,” but there is no joint instability.
    • Occurs with ascending or descending stairs or an incline.
    • Meniscal / ligamentous injury leads to instability and apprehension with pivoting or twisting movements.
  • On exam may have tenderness over:
    • Medial and/or lateral patellar facets
    • Superior and inferior poles of the patella
  • Pain over the physes or joint line is NOT normal for patellofemoral pain. [Houghton, 2006]


Patellofemoral Pain Syndrome: DDx

  • Always consider the following:
  • The history and exam may be encouraging, but persistent pain warrants consideration for more ominous issues and, thus, images may be warranted.


Patellofemoral Pain Syndrome: Management

  • Educate and Reassure
    • Do not dismiss as “growing pains.”
    • Generally, gradual improvement and resolution of symptoms can be achieved. [Houghton, 2006]
  • Activity Modification
    • Reduce impact exercises
      • Avoid running and jumping sports.
    • Reduce activities that load the knee
      • Avoid squatting exercises and sports (not good to be a catcher in baseball).
  • Increase Flexibility
    • Particularly hamstrings, quadriceps, iliotibial band, and gastrocnemius.
    • We all could stand to be more flexible (in all aspects of life).
  • Increase Strength
    • Paying attention to appropriate technique to not do more harm!
    • Straight leg raises can strengthen the Quads without placing excessive force on patella. [Ganley, 2006]
    • Water therapy and elliptical trainers can be helpful.
  • Cryotherapy (just like Michael Jordan would do)
  • NSAIDs
  • Corrective orthotics may be helpful in some cases.


Moral of the Morsel

  • Appreciate that your words have weight.
    • Even though your exam is reassuring, don’t merely call it a “knee sprain.”
    • Similar to not saying “it’s just a virus.”
  • Consider the potential Badness and look for clues… and don’t freak about about getting a few Xrays.  We are talking about major medical radiation.
  • If you choose not to image, encourage follow-up… and don’t label it as anything…
    • “Knee Pain not otherwise specified” is better than a misdiagnosis of patellofemoral pain syndrome in a kid who eventual gets diagnosed with osteosarcoma.



Yin AX1, Sugimoto D2, Martin DJ3, Stracciolini A4. Pediatric Dance Injuries: A Cross-Sectional Epidemiological Study. PM R. 2016 Apr;8(4):348-55. PMID: 26318766. [PubMed] [Read by QxMD]

Pengel KB. Common overuse injuries in the young athlete. Pediatr Ann. 2014 Dec;43(12):e297-308. PMID: 25486038. [PubMed] [Read by QxMD]

Finlayson C. Knee injuries in the young athlete. Pediatr Ann. 2014 Dec;43(12):e282-90. PMID: 25486036. [PubMed] [Read by QxMD]

Stracciolini A1, Casciano R, Levey Friedman H, Stein CJ, Meehan WP 3rd, Micheli LJ. Pediatric sports injuries: a comparison of males versus females. Am J Sports Med. 2014 Apr;42(4):965-72. PMID: 24567251. [PubMed] [Read by QxMD]

Houghton KM1. Review for the generalist: evaluation of anterior knee pain. Pediatr Rheumatol Online J. 2007 May 4;5:8. PMID: 17550634. [PubMed] [Read by QxMD]

Ganley TJ1, Gaugles RL, Moroz LA. Consultation with the specialist: patellofemoral conditions in childhood. Pediatr Rev. 2006 Jul;27(7):264-9; quiz 270. PMID: 16815995. [PubMed] [Read by QxMD]

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