GI, Ortho, Rashes and Infections Flashcards

1
Q

diagnostic landmarks in adult vs child bone

A
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2
Q

What is the system used for grading fx through growth plate?

A

Salter Harris

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3
Q

Why do we care for injuries through growth plate???

A

Injuries to physis can result in premature closure

Partial closure may cause angular deformity

Complete closure may cause limb shortening

Most common locations: distal femur and distal/proximal tibia

Growth plate most susceptible to torsional and angular force

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4
Q

define type I-V Salter Harris grading system

A
  • Type I – fracture through growth plate only
  • Type II – fracture through metaphysis and growth plate
  • Type III – fracture through epiphysis and growth plate
  • Type IV – fracture through metaphysis and epiphysis
  • Type V – Crushed through growth plate
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5
Q

Common sites of grwoth plate fx

A
  • Distal radius
  • Distal tibia
  • Distal fibula
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6
Q

most common salter type fx?

A

Salter II

Fracture through portion of physis and metaphysis

Fracture passes across most of growth plate and up through metaphysis

Good prognosis

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7
Q

Grade fx ?

A

Salter I

Transverse fracture through the physis

Cannot occur if the growth plate is fused

Good prognosis, growth disturbance is unusual

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8
Q

Grade Fx

A

Salter II (most common)

Fracture through portion of physis and metaphysis

Fracture passes across most of growth plate and up through metaphysis

Good prognosis

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9
Q

grade fx

A

Salter III

Fracture through portion of physis and epiphysis into joint

Poorer prognosis because of intra- articular component and because of disruption of growing or hypertrophic zone of the physis

7-10%

Fracture plane passes through epiphysis and growth plate

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10
Q

Grade Fx

A

Salter IV

Fracture through metaphysis, physis, and epiphysis

high risk of complication

Poor prognosis

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11
Q

Grade Fx

A

Salter V(crush)

Crush injury of the physis

Crushing type injury does not displace growth plate but damages it by direct compression

Worst prognosis –>Subsequent growth arrest of this area confirms presence of SalterHarris type V injury

Complete obliteration or diminished physeal distance of the affected extremity confirms the diagnosis

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12
Q

More likely to require surgical fixation if

A
  • Displaced epiphyseal fractures
  • Displaced intra-articular fractures
  • Fractures in child with multiple injuries
  • Open fractures
  • Unstable fractures
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13
Q

complciations of Fx

A

•Overgrowth

  • In long bones, result of increased blood flow associated with fracture healing
  • Femoral fractures in children <10 can overgrow 1-3 cm
    • So end-to-end alignment for femur and long-bone fractures may not be indicated
  • After 10 y/o age, overgrowth less of a problem, end-to-end alignment is recommended

Neurovascular Injury

  • Common locations: distal humerus and knee

Compartment Syndrome

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14
Q

Fracture remodeling Process

A
  • Periosteal resorption
  • New bone formation
  • No need perfect anatomic alignment
  • Younger patients have greater potential for fracture remodeling
  • Rotated fractures, and fracture deformity not in ‘plane of motion’ don’t remodel as well
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15
Q

Incomplete fracture of long bone produced on convex cortex, while concave cortex bends

Dx? MOI?

A

Greenstick Fx

Results from bending force applied perpendicular to shaft

•FOOSH

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16
Q

Type of incomplete fracture

occurs at metaphyseal-diaphyseal junction –>FOOSH

A

Buckle or Torus Fx

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17
Q

How do Toddlers Fx present

A

Presents limping and pain with WB, but minimal swelling and pain

*Often no trauma recalled

*Patients usu. 1-3 y/o

Minimally/nondisplaced oblique fx of- Tibia without fibula fx

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18
Q

define Supracondylar Elbow Fx & MOI

A

Extra-articular fracture of distal humerus at elbow

  • occurs in children between 5-9 y/o
  • 50% to 70% of all peds elbow fracture

Almost always due to accidental trauma

  • FOOSH from a moderate height (bed/monkey-bars)
  • Typically (>90%) onto extended elbow

Result in an extra-articular fracture line

Posterior displacement of the distal component

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19
Q

tx of Supracondylar Elbow Fx

consrvtaive

surgucal

A

Conservative

  • Long arm cast after initial splint
  • Analgesics
  • Serial radiographs (q 1-2 wks.)

Reduction with Pin Fixation

  • Two lateral pin technique for stable fixation with a medial pin
  • Correct medial pin placement is critical
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20
Q

comapre contrast

Galeazzi Fracture Dislocation

Monteggia Fracture Dislocation

A

Galeazzi Fracture Dislocation: FOOSH w/ flexed elbow

Fracture of distal radius + Dislocation of distal radioulnar joint (DRUJ)

  • I_ntact ulna_
  • 9-12 years of age

Monteggia Fracture Dislocation: secondary to FOOSH

  • Fracture of ulna shaft+ Dislocation of radial head
  • Displaced and overlapped _fracture of the ulnar shaf_t
  • Radial head is dislocated anteriorly
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21
Q

define MUGR & GRIMUS

A

Galeazzi involves fracture of the radius (MUGR)

Monteggia involves fracture of the ulna (MUGR)

GRIMUS

G: Galeazzi

R: radius (distal radioulnar joint (DRUJ)

I: inferior

M: Monteggia

U: ulna (dislocation of radial head anterioirly)

S: superior

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22
Q

Tx of Monteggia Fracture Dislocation

A

ORIF

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23
Q

Nursemaids Elbow MOI

Tx

A

pulled/lifted by the hand NO FALL!!- yanked by arm

  • Radial head subluxes under Annular Ligament

Tx by pressure on radial head and gentle supination while flexing the elbow

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24
Q

How is DDH dx?

A

Common pediatric orthopedic condition - Focus of newborn evaluation (Barlow THEN Ortolani)

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25
DDH si/sx in infant/child
* Toe walking- can be unilateral * Limb length inequality * Waddling Gait * Hyperlordosis (Swayback)
26
DDH Diagnosed with spectrum of anatomic abnormalities including:
* Hip that is dislocated and irreducible * Unstable (dislocatable and reducible) * Dysplastic, but within acetabulum
27
Tx of DDH
_Pavlik Harness_ - Double-diaper Abduction orthosis (if Pavlik harness fails) If all conservative measures fail or \>6months of age * Closed reduction 1st option * Open reduction if closed reduction fails * •pica cast to hold hip/hips in reduced position \*US post application- verify position of harness
28
Legg Calve Perthes- LCP stages of Dz?
1. Necrosis: Initial period of ischemia/loss of blood supply to femoral head 2. Fragmentation: Re-absorption of bone with femoral head collapse 3. Re-ossification: New bone regrows to reshape the femoral head 4. Remodeling: Femoral head reshapes itself into spherical shape
29
define LCP
Idiopathic osteonecrosis of capital femoral epiphysis Vascular interruption to subchondral bone Peri-articular cartilage not affected Epiphyseal changes due to subchondral Fx * Ages 2-14 (mostly 5-8 years of age) * Boys 5x \> Girls
30
si/sx of LCP
**male** - Small for age Very active or hyperactive **Pain** may be non-specific * Anterior hip, thigh or knee * Insidious onset (maybe weeksmonths) **Mild limp** Usually no history of trauma **Limited motion: abduction + internal rotation** Guarding with leg rolling Atrophy of quad muscle secondary to disuse **Leg length inequality** -collapse of femoral head
31
imaging of LCP
**AP Pelvis and Frog Lateral** - Compare to contralateral side * Early changes: smaller epiphysis, radiodense (sclerosis) * _Crescent sign or mild flattening_ * Metaphyseal radiolucency
32
Tx of LCP
**Self-healing in 2-4 years** _Problem_: Not all end up with a spherical head * Can produce permanent femoral head deformity and early arthritis in adulthood _Poorer outcome_ in older patients \>8 years of age
33
si/sx of Slipped Capital Femoral Epiphysis- “SCFE”
Obese boy (+) limping Intermittent groin/knee pain (weeks –months) Sudden onset of pain in groin/hip - Maybe after fall or trauma Inability to walk/bear weight on affected leg Shortened, externally rotated leg with significant slip/grade
34
tx of Slipped Capital Femoral Epiphysis- “SCFE”
Operative stabilization of the fracture/ ‘slip’ * Percutaneous Screw Fixation Goal: to stabilize the physis and prevent any further slippage…ultimately further complication
35
Most common cause of hip pain in children
Transient Synovitis of Hip
36
si/sx of Transient Synovitis of Hip
follows URI. +/- low grade fever : Rapid onset of limping and subsequent refusal to walk/bear weight ROM of hip limited by pain and spasm, hip held in flexion
37
dx of Transient Synovitis of Hip
_\*Diagnosis of exclusion_ ## Footnote Labs: +/- mild elevation of WBC, ESR, CRP X-ray: AP Pelvis/frog-leg lateral. Usu. normal, may show slight joint space widening US: Evaluate effusion (negative
38
tx of transient hip synovitis
**Bed rest** until symptoms and signs improve Gradual increase of activity _NWB generally lasts 1-2 days_ May have limp and decrease ROM up to two weeks _NSAIDs_ – wt. based _No Abx_ due to not infectious etiology
39
define Apophysitis & 2 types
Apophysitis= painful inflammation of a bony outgrowth and in an area of active Growth at the end of a bone Osgood-Schlatter’s Sever’s “Disease”
40
Traction at insertion of patella tendon into tibial tuberosity Pain over tibial tuberosity relieved with rest Can be bilateral Prominent tibial tubercle
Osgood-Schlatter’s Think ‘4-sport’ per year During a growth spurt
41
tx of Osgood-Schlatter’s
Rest , Avoidance Out of sports Ice & NSAIDs Knee immobilizer for a few days to quiet symptoms Reassurance
42
tx of Severs Dz
RICE& NSAIDS, DC sports moderate/severe _Gel heel pads if mild_; ½” heel inserts if moderate or worse (wear in both shoes for symmetry) _Stretching of Achilles’;_
43
Common cause of heel pain in children dx & MOI
Sever’s “Disease” Repetitive stress (Running, jumping, etc.) on growth plate as foot strikes ground results in inflammation/pain •Often seen beginning of new season of sports
44
si/sx of severs dz
heel pain bad enough to cause a limp Noticed initially after sports Then during and after activity ends As it progresses, pain without running/jumping Pt. will often report “new cleats” or footwear
45
compare and contrast physiologic vs pathologic varus/valgus
46
Physiologic Genu Varum AKA ??
Bowlegs ## Footnote Symmetric Varus Age: 0-2 years Normal growth plate on x-ray
47
Physiologic Genu Varum (bowlegs) Worrisome clinical features
* Lateral thrust during gait * Short stature * Ligament laxity * Abnormal location of the deformity
48
XR for physiology genu varum IF???
* Asymmetry * Atypical age * Worsening deformity
49
Name causes of Pathologic Genu Varum what is diagnostic?
Osteochondral dystrophy Rickets Tibia varum/Blount's DX; X-rays
50
# define Pathologic Genu Varum blounts rickets
**Blounts Dz** - Progressive deformity unilateral * Early walking, obesity, * Family history of Blount’s * _Lateral Thrust during gait_ * If a child \>2 y/o still has Genu Varum → refer **Rickets** * Short Stature * Enlargement of elbow, wrists, knee, and ankles
51
Physiologic Genu Valgus- AKA???
“Knock Knees” ## Footnote Normal growth plate on x-rays
52
Presents when toddler begins to walk +/-Limits ankle dorsiflexion +/-Reduction of ankle dorsiflexion Normal neurologic exam
Toe Walking
53
toe walking is dx of exclusion , must r/o???
Neuromuscular disorder * Cerebral Palsy * Autism * Due to increase in vestibular stimulation
54
tx tow walking
PT: Heel-cord stretching Serial casting Surgical: Heel-cord lengthening
55
Differentiate from metatarsus varus by seeing the _____ in club foot
Differentiate from metatarsus varus by seeing the **Equinus** in club foot * _Equinus_ = the inability of the ankle joint to dorsiflex
56
Pathology of club foot
Pathology •Equinus, Adductus, Varus, and medial rotation
57
tx of club foot
_surgery (heel cord lengthening) and/or serial casting_ Serial casting - Casting 3-6 weeks, heel cord lengthening, shoes with brace full time for thee months, then nights and naps till 4 years old. PT guided daily stretching, taping **Goal of Treatment** * Flexible, “shoe-able” foot * Foot, leg never looks entirely normal in true congenital clubfoot deformity
58
# define scoliosis 3 types
Lateral spinal curvature with a Cobb angle of \>10° 1. Idiopathic- 90% F, +/- fam. hx, adolescent growth spurt 2. Congenital- vertebral abnormality 3. Neuromuscular- underlying d/o
59
explain scoliosis exam and what we observe for
Pt should stand erect with feet slightly apart, knees straight, and arms hanging loosely at sides _Observe for_ * •One shoulder higher than the other * •Larger space from arm to side of body(compare both sides) * •Uneven waist creases * •Uneven iliac crest levels * •Forward Bend --\> Rib prominence
60
Dx / imaging of scoliosis
Full length Scoli X-ray series
61
tx of scoliosis ## Footnote **_nfant and Juvenile (3-10 yo)_** **_Adolescent_**
**3 - O’s** Observe, Orthosis , Operation **_Infant and Juvenile (3-10 yo)_** * Observe to 25 degrees * Orthosis \>25 degrees * Operate \>40 degrees **_Adolescent_** * Observe to 30 degrees * Orthosis 30 to 40 degrees * Surgery \> 40 degrees or rapidly progressing curves
62
the normal bony outgrowths that arise from separate ossification centers and eventually fuse with the bone in time
Iliac Apophysis
63
how is Iliac Apophysis classified??
Risser classification: •Grades skeletal maturity based on level of ossification of iliac crest apophyses
64
describe 5 stages of Risser classification of ilkiac apophysiss
_stage 0_: no ossification _stage 1:_ apophysis 25-50% of iliac crest _stage 3:_ apophysis \>50-75% of iliac crest _stage 4_: apophysis \>75% of iliac crest _stage 5_: complete ossification and fusion of iliac crest apophysis
65
•Most common bacterial infection in children
Impetigo
66
name pathogens fo Impetigeo
Both - Type 1 Non-bullous (most common) S. aureus - Type 2: Bullous Β-hemolytic strep group A - Type 3: Ecthyma
67
si/sx of type 1 , 2 and 3 impetigo
**Type 1 Non-bullous (most common)** * Papules on the face ® vesicles & erythema ® pustules ® golden _”honey colored”_ crusts * May have regional lymphadenitis * NO systemic symptoms **Type 2: Bullous** * Vesicles ® flaccid bullae with clear fluid ® rupture leaving _thin brown crust_ * Fewer lesions * _Affects trunk_ * Related to pemphigus * Due to Staph Aureus that produce exfoliative toxin A à loss of cell adhesion in superficial dermis **Type 3: Ecthyma** * _“Punched-out”_ ulcers --\> covered w/ yellow crust that is surrounded by violaceous raised margins * Affect dermis and epidermis * ulcers
68
tx of impetigo topical oral
Tx for BOTH = Staph Aureus and Strep A **Topical therapy (limited dz)** * Mupirocin (Bactroban) TID * Retapamulin (Altabax) BID **Oral therapy (extensive dz) -** bullae, multiple lesions in multiple locations * Dicloxacillin 250 mg QID * Cephalexin (Keflex) 250 mg QID * Clindamycin * Bactrim (MRSA)
69
cause of Pinworms tx?
_Enterobius vermicularis_ **Albendazole** (Albenza) 400 mg * (tx & repeat- 2 wks to kill eggs) **Pyrantel pamoate (OTC)** T_x family members & Good hygiene_
70
tx of Enuresis
DDAVP--\> ↑ ADH --\> ↓ in urine output Other treatments… * •Moisture alarms * •Scheduled voiding * •Decrease bladder stimulants like caffeine * •Limit fluid intake before bed
71
Anatomical abnormalities: Vesicoureteric reflux •If family history of GU reflux…watch for unexplained fevers in new sibs responsible for???
pedi UTI
72
si/sx of UTI in infancy dx??
URI SXS WITH FEVER AND \<3MO Dx: catheterized specimen
73
si/sx of UTI in children teens
**CHILDREN**: * Toilet training & poor wiping * Constipation / withholding * ABDOMINAL PAIN, maybe dysuria and frequency * CLEAN CATCH SPECIMEN WHEN TOILET TRAINED * Male – get workup !! * Female – workup second UTI **TEENS:** * STI / sexually related until proven otherwise * E COLI * PROTEUS (does not show on cx) * Pee after se F
74
pathogens responsible for?? ## Footnote Measles (Rubeola) Rubella (German measles) Fifth disease (Erythema infectiosum) Infectious Mono Roseola Hand, foot, & mouth
Measles (Rubeola) - Paramyxovrius Rubella (German measles) - Toga virus Fifth disease (Erythema infectiosum) - Parvovirus 19 Infectious Mono - EBV) Roseola =HHV-6 & 7 Hand, foot, & mouth - Coxsackie virus
75
Prodrome (ex. cough, coryza, conjunctivitis, fever) •Macular, morbilliform rash starting on head, spreading to extremities (cephalocaudal) Erythematous papules (palms/soles affected) _Koplick spots_ - white spots on mouth (“stuck on appearance”
Measles (Rubeola)
76
dx Measles (Rubeola)
Measles IgM abs Measles RNA by PCR Serum and NP swab Can also collect urine
77
tx measles
**MMR vaccine ppx** - within 72 hours of exposure **Immunoglobulin** – 6 days of exposure **Vitamin A** - administered immediately on diagnosis and repeated the next day. * Vitamin A deficiency --\> delayed recovery and high rate of complications. * measles infection =acute vitamin A deficiency & xerophthalmia (severe dry eye). * measles --\> preventable childhood blindness,
78
si/sx of Rubella cpmplications
* No prodrome +/- low-grade fever * +/- lymphadenopathy , arthralgia – older kids & adults * Pale pink morbilliform macules complications: birth defects in pregnant women
79
si/ sx of Fifth disease (Erythema infectiosum) ## Footnote Day 1-2 Day 2-5:
**Day 1-2** * Fever * HA * N/V/D **Day 2-5: RASH (no transmission after rash)** * Acute onset asymptomatic diffuse, macular erythema (slapped cheek) * Lacey, reticular pattern formation * Circumferential pallor
80
si/sx of mono
* Fever, fatigue * Pharyngitis/tonsillitis (“kissing tonsils”) - pharyngeal erythema; exudates; petechiae * Posterior cervical lymphadenopathy * Splenomegaly * Non-specific rash- maculopapular~ urticarial~ petechial (following use of PCN/Ampicillin or azithromycin, levoquin, cephalexin
81
dx mono
**Atypical lymphocytosis** – early **Heterophil Ab monospot** - horse RBC agglutination **EBV panel** – si/sx of IM , heterophile (-)after retesting 1 week later -\> EBV panel * IgM and IgG VCA and EBNA (nuclear antigen –only in latency stage) * If EBV panel (-)--\> CMV, HIV, Toxo, HHV6 or 7, Hep B
82
mono Splenic Rupture Precautions
3 weeks mild activity min 4 weeks for contact/high risk sports and activities Or longer if spleen enlarged by palpation or ultrasound REST during periods of fatigue but bedrest not necessary
83
* Sudden onset high fever (102-104)- fver subsides and THEN ---\> * Asymptomatic SUDDEN rash (trunk & extremities) pathogen & Dx?
Roseola HHV-6 & 7
84
si/sx of hand, foot and mouth
* Aphthous stomatitis * Rash on hands & feet * Fever * Poor-feeding * Irritability
85
tx of hand foot and mouth
* Supportive (self-limiting) * Antipyretics (ex. Tylenol, Motrin) _•Magic mouthwash_- Benadryl and maalox to coat the oral lesions •Anticipatory guidance
86
•Faint macules = vesicular eruptions (dew on a rose petal) dx?
Varicella dx Tzank smear (multinucleated giant cells)
87
# define straabismus eso vs exo latent manifest
Nasal Deviation (eso-)vs. Temporal Deviation (exo-) Latent = Phoria * present when fixation interrupted (Esophoria vs exophoria) Manifest = Tropia * present without interruption of gaze (Esotropia vs exotropia)
88
dx strabismus
* Hirshberg * Cover * Cover/uncover * Bruckner red reflex
89
tx of strabismus
**Medical** * Glasses * Miotic drops * Patching * Visual training exercises **Surgical**: Reposition/shortening
90
when to refer strabismus
+constant esotropia at any age + persistent esodeviations after 4 mo +deviation with corneal light reflection test or cover test + Asymmetry of appearance on Bruckner test + deviations that change with position of gaze (incomitant) + inexplicable torticollis with strabismus Any parental or provider concerns
91
define kawasaki dz
Widespread inflammation of medium sized blood vessels throughout the body VASCULITIS - _Most common childhood vasculitis_ * Most importantly affects the vessels of the heart * Long term affects seem to be only on ARTERIES * Most visibly affects mucous membranes
92
si/sx of kawasaki
**CRASH" and BURN** Non- exudative _Conjunctivitis_ (bilat bulbar conjunctival injection)/cheilitis _Rash_ (polymorphous, desquamations involving perineum) _Adenopathy_ (cervical) - sinuglar and large _Strawberry tongue_ _Hand & feet_ (desquamation, swelling, erythema) _Fever (\>5 days)_ Redness and induration at the site of a previous vaccination with Bacillus Calmette-Guérin (BCG
93
dx studies for kawasaki
Baseline echocardiogram INC ESR & PLTS Sterile pyuria Each patient with diagnosis will have cardiac echo to detect CA aneurysms * repeat in 4-6 weeks to document resolution
94
dx criteria for kawasaki
95
tx of kawaskai
Hospital admission for CV monitoring * Must have cardiology consult at DX **IVIG 2g/kg** - ↓ RISK OF CA ANEURYSM (day5-7) **Aspirin** **Steroids**- if IVIG fails twice (methylprednisolone) **TNF**-I (infliximab)- consider only if IVIG fails twice
96
complciation of kawasaki
•Coronary artery aneurysm
97
Fetal hydrops in utero - hydrops-fetal demise is a complication of??
Fifth disease (Erythema infectiosum
98
dz characterized by ## Footnote post prandial non bilious PROJECTILE VOMITING --\> feel better then want to eat again Hungry after vomiting
Pyloric Stenosis P - Peristalsis (Visible peristalsis in abdomen) Y - Yuck! So the kid vomits everything he eats (non bile stained vomiting) L - Lump in abdomen which is on the Left side O - Olive mass, Doughnut sign on USG R - Ramstedt's pyloromyotomy, mass moves on Respiration I - Imbalance of electrolytes C - Circular muscle hypertrophy
99
* Palpable OLIVE RUQ (felt best after vomiting) * Peristaltic Waves may be visualized pre-emesis
pyloric stenosis
100
dx pyloric stensosi
ULTRASOUND (or ugi) Endocscopy (only if above inconclusive) Check electrolytes to measure (de)hydration status (BMP)
101
tx pyloric stenosis
Correct hydration status FIRST SURGICAL : PYLOROMYOTOMY Ramstedt pyloromyotomy * •Minimally invasive * •Low complication Rate * •Longitudinal incision over pylorus with blunt dissection * •To level of submucosa, * •relieves the constriction
102
TRIAD of pain-palpable mass- current jelly stool
Intussusception
103
pathogens responsible for Intussusception
Virus: Rotavirus, Uri, OM, flu
104
Intussusception Lead point from underlying pathology
_Meckel diverticulum_ * Polyp * Lymphoma * Cysts * Vascular malformations parasites _•HSP- Henoch Schonlein Purpura_
105
define Intussusception
Invagination (telescoping) of the intestine into itself •Most common cause of obstruction 6-36 mo of life
106
dx imaging for Intussusception
**Xray to r/o obstruction** * Lack of colonic gas with massively distended loops of bowel * _Target sign_ over right kidney (peritoneal fat surrounding intuss) * _Crescent sign_ is soft tissue density procting into gas of large bowel **Ultrasound** * Sensitivity/specificity close to 100% if skilled rads * Classic appearance is “_bull’s eye” or “coiled spring”_ * Doppler may show poor/absent perfusion
107
•Classic appearance is “bull’s eye” or “coiled spring” on US id Dx for??
Intussusception
108
tx Intussusception
**Non-operative:** Tx of choice in _stable pts w/o__perforation_ * _Enema with hydrostatic (barium) or pneumatic (air) pressure_ * flouroscopic or u/s guidance * 80-95% successful (best if idiopathic etiology) * PPX antibiotics considered but not necessarily used * 10% recurrence * Requires surgeon in the suit in case of perforation **SURGICAL**: Indicated if failed non operative approach * Suspected or proven perforation or bowel necrosis * Risk approx 1 % after surgical rx.
109
etiology of PKU
Deficiency of Phenylalanine Hydroxylase (PAH) * PAH catalyzes conversion of phenylalanine to tyrosine No PAH = increase serum and urine phenylalanine * INCREASE phenylalanine--\>intellectual disability * ↑ phenylalanine may interfere with brain growth, myelination and neurotransmitter synthesis
110
si/sx of PKU
Mental disability and impaired IQ Epilepsy Abnl gait _Blood and urine may smell “mousy”_ Pigmentation issues Eczema
111
dx PKU tx
Method of testing is tandem mass spectrometry avoidance of PKU foods
112
si/sx of Appendicitis ## Footnote **\<1mo** **\<5 yo** ​**5-12 yo** :
**\<1mo** – RARE **\<5 yo** * Listless * Low grade fever (100.2-101) * w/ diffuse pain, anorexia, vomiting * rebound, guarding … perf!! **5-12 yo** : much more common * Present with abd pain, anorexia and vomiting. * migratory pain to RLQ * Kids may limp or bend over * May complain of hip pain
113
dx apendicitis
CBC/diff- elevated wbc and polys UA- r/o uti- sterile pyuria in kids, do NOT let it fool you UHCG- r/o pregnancy (nl and ectopic) **US** - young, thin, female or male that are at no more than moderate risk after pe and labs Consider CT without rectal contrast if : * Ultrasound inconclusive * Not a good u/s candidate •The surgeon requests the CT
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tx apendicitis non perf perf
**Non perforated :** * •Fluids * •Pain control- anti pyretics& emetics * •NPO * •Pre op antibiotics (cefoxitin) * •Surgery **Perforated** * •Fluids * •Pain control + anti pyretics/ emetics * •NPO * •Triples (amp/gent/flagyl) _SURGERY:_ * within 6-8 hours of dx if non perforated * If perforation, may use IV antibiotics for 24-48 hours and then surgery
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tx of constipation infants older children
Infants * Glycerine suppository * Lubricated thermometer Older children * Glycerine suppository * MIRALAX * Enema ? * Laxative ?
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causes of constipation organic functional
Organic \<5% Anatomic: Anal Stenosis, imperforate anus, pelvic mass Metabolic: hypothyroid, cf, dm, gluten Neuropathic: spinal cord, neurofibromatosis, tethered cord Intestinal nerve or muscle disorders: eg Hirschsprung Abd musculature disorder: Prune belly, Down Syndrome Food intolerance heavy metal ingestion, meds Functional * Painful defecation * Toilet training issues * Dietary issues * Trauma
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etiology of Hirschsprung Disease
Primarily mutations of the RET proto-oncogene * Incomplete migration of neural cells in the myenteric and submucosal plexus PART OF THE COLON LACKS GANGLION CELLS * This results in the affected segment constricting, thus the normal PROXIMAL segment becomes distended with feces 75% of the time the _aganglionic region is rectosigmoid_ 8% of the time it is the entire colon
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si/sx of Hirschsprung dz newborn child
**Newborn**: Failure to have complete stooling or stool **Child:** * Swollen belly, vomiting, constipation, diarrhea * Failure to thrive, fatigue
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dx & Tx of Hirschsprung Disease
**dx:** _Biopsy- gold standard_ * Manometry of anorectum * Barium enema * Xray **tx:** Surgical excision of the affected area with anastomosis of the healthy ends.
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explai rule of 2s and where meckles dievrticulum is located
Rule of 2’s: * •2 % of population * •2:1 males to female ratio * •2 years old is the most common time of presentation Where is it ? * •2 feet proximal to the ileocecal valve * •2 inches in length
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Hirschsprung dz 75% of the time the aganglionic region is \_\_\_\_\_\_\_.
Hirschsprung dz 75% of the time the aganglionic region is **rectosigmoid**
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dx and tx or meckles
dx (“incidentaloma”) ## Footnote treated with excision
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define malrotation
Congenital anomaly of the mid gut leading to: * Small intestine found on R side abdomen * Cecum displaced into epigastric region * Ligament of Treitz displaced * Fibrous bands form leading to obstruction * Narrow base of small intestine --\>è volvulus volvulus - Twisting that can --\> ischemia --\> perforation etc
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si/sx of malrotation
Infants present with symptoms of volvulus or obstruction * BILIOUS vomiting * Abdominal pain= fussy, crying, not eating/drinking * Abdominal distention * Melena and or mucousy stool
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dx of malrotation
Abd xray: obstruction UGI series if not emergent * _corkscrew appearance of the distal duodenum_ Contrast enema if very doubtful
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Most common cause of Encopresis is \_\_\_\_?
Most common cause of Encopresis is constipation
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dx criteria for encopresis
1. Voluntary / involuntary passage of stool outside bathroom or diaper 2. One event a month for at least 3 mo 3. \>4 yrs old 4. Stooling not result of laxatives or illness involving colon such as colitis
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tx of encopresis
Miralax – LOTS Stool softeners Scheduled stooling