Peds Mnemonic Flashcards
NF1 criteria
Cafe an lait spots Axillary freckling Lisch nodules Bony deformity Optic glioma Relative affected Neurofibromas
a first-degree relative with NF1
6 or more cafe-au-lait spots
2 or more neurofibromas or 1 plexiform neurofibroma
axillary/inguinal/or other intertriginous freckling
2 or more Lisch nodules
a distinctive osseous lesion (e.g. sphenoid bone dysplasia)
optic nerve glioma
A “pilocytic astrocytoma” of the optic nerve is equivalent to an optic glioma. On neuro-imaging, optic gliomas often appear as “fusiform” or “sausage-shaped” or “kinked” enlargements of the optic nerve.
NF1 may be assoc/w/synchial angle closure (and rarely NVI/NVA).
U6B10
The “fake” chemistry formula “U6B10” is a way to remember that unilateral cataracts should be taken out by 6 weeks, and bilateral cataracts by 10 weeks
Also, babies don’t start to make eye contact until 6 wks of age, and don’t develop vertical eye movements until 6 mo.
Congenital nystagmus
Congenital nystagmus
Convergence and eye closure dampen the nystagmus;
Oscillopsia is usually absent;
Null zone is present;
Gaze position does not change the direction of nystagmus;
Equal amplitude and frequency of nystagmus in each eye;
Near acuity is good because convergence dampens the nystagmus;
Inversion of optokinetic nystagmus occurs;
Turning of head or abnormal head posture to allow eyes to enter a null zone leads to better visual acuity;
Absent nystagmus during sleep;
Latent nystagmus occurs.
Phakomatoses and chromosome numbers
chromosome 9 – tuberous sclerosis (also chromosome 16)
chromosome 22 – NF-2
Chromosome 17– NF1
Ch3 = VHL
Scheie
“she (Sheie) has the cloudiest corneas!”
Scheie syndrome is an inherited disease of metabolism in which the body cannot properly break down long chains of sugar molecules called glycosaminoglycans. The syndrome belongs to a group of diseases called mucopolysaccharidoses (MPS). The enzyme defect in Scheie syndrome is of lysosomal alpha-L-iduronidase. Scheie syndrome is actually the mildest form of MPS type 1 which also includes the entities Hurler syndrome and Hurler-Scheie syndrome. MPS type 2 is equivalent to Hunter syndrome whose major ophthalmic sign is retinal degeneration. All of the syndromes listed, besides Scheie syndrome, do not feature corneal clouding as part of their stigmata.
Lens position
Marfan’s syndrome – imagine a tall marfinoid basketball player reaching out and dunking –> superotemporal subluxation
Homocystinuria – “uria” –> goes down toward the bladder –> inferonasal subluxation.
Pediatric glaucoma - law of 2/3
peds glaucoma - law of 2/3
affects 2/3 of males, and affects OU 2/3 of the time
Ectopia Lentis DDx
WATCH HIM SEE Weil Marchesani Aniridia Trauma Congenital glaucoma Homocystinuria
Hereditary ectopia lentis
Iris coloboma
Marfans
Sulfite oxidase deficiency
Ehler Danlos
Ectopia lentis pupillae
Rhabdo
Everyone gets Embryonal (most common 80%, superonasal quadrant) Alveolar is Awful (most lethal, lower orbit, survival <10%) Pleomorphic Please (best survival rate, 97%, so if someone gets rhabdo please let it be this type)
Genetic association with Axenfeld Reiger
Axe the Foxy (FOXC) Pix(pics) (PITX): Axe for Axenfeld, FOXC1 for first word, PITX2 for second word
Cloudy cornea at birth
Peter's anomaly is on the list of causes of cloudy cornea at birth. These can be remembered by the mnemonic STUMPED:Sclerocornea, Trauma (forceps), Ulcer, Mucopolysaccharidoses, Peters, Endothelial (CHED) Dermoid.
ROP screening
For ROP screening, the first dilated fundus exam should take place between 4 and 6 weeks of postnatal age (or within the 31st to 33rd week of postconceptional or postmenstrual age, whichever is later). Since this child is currently 2 weeks old, he should be screened 2 to 4 weeks from now.
Mnemonic: 3+1= 4, 3+3= 6
A and V pattern
If a resect-recess surgery is decided upon, then displacement of the rectus muscles should be in opposite directions according to the normal rules in managing “A” or “V” patterns. In this case of an A-pattern exotropia, the medial rectus should be tightened and displaced toward the “apex” (i.e. superiorly in this case) while the lateral rectus should be loosened and displaced toward the “open area” (i.e. inferiorly for an “A” pattern).
A good mnemonic to remember these rules is: “MALE” – Medials to the Apex; Laterals to the Empty space.
10 for A pattern and 15 for V pattern
Myotonic dystrophy
for myotonic dystrophy – imagine Frankenstein! He would have a hard time releasing a handshake, isnt too bright, cant see very far (xmas tree cataracts) definitely has ptosis, his eyes dont move around much (CPEO), and certainly has cardiac conduction issues from those bolts on his neck they electrocute him with.
Answer:Ptosis, orbicularis weakness, progressive external ophthalmoplegia, and pigmentary retinopathy similar to that of Kearn’s Sayre syndrome. Aside FROM Christmas tree cataracts which contain multicolored iridescent crystals, patients with myotonic dystrophy may also have spokelike cortical opacities along the suture line.
Kearns Sayre
Eyes can’t track, retina is black, heart attack.
Lids are slack.
This patient demonstrates the classic clinical findings of Kearns-Sayre syndrome: chronic progressive external ophthalmoplegia (CPEO), retinal pigmentary changes, and cardiomyopathy. An EKG is necessary to diagnose the presence of possible heart block.
