Fussy infant/Hemophilia

Question 1

DDx Fussy infant

Answer 1

• Nonorganic causes: offer parental support and reassurance.

- Organic causes.

Question 2

Fussy infant, DDx, nonorganic causes?

Answer 2

Hunger, sleep, dirty diaper
Overbundled infant, inadequate clothing
Teething, postvaccination
Infantile colic, need for attention

Question 3

Fussy infant, DDx, organic causes?

Answer 3

Dermatologic, musculoskeletal, GI, Infections, Ocular, Oral, GU, Neurologic, Behavioral, Hematologic

Question 4

Fussy infant, DDx, Dermatologic causes?

Answer 4

Eczema, impetigo, diaper rash, insect bites, allergic/pruritic rash, urticaria, scabies, burns

Question 5

Fussy infant, DDx, musculoskeletal causes?

Answer 5

Fracture, contusion, tourniquet syndrome, nursemaid’s elbow, nonaccidental or accidental trauma

Question 6

Fussy infant, DDx, GI causes?

Answer 6

GERD, constipation, food allergy/intolerance, ingestions/poisoning, intussusception

Question 7

Fussy infant, DDx, Infectious causes?

Answer 7

URI, pharyngitis, gastroenteritis, OM, viral illness, cellulitis, herpangina, HFMD, UTI, sepsis, pneumonia, meningitis

Question 8

Fussy infant, DDx, ocular causes?

Answer 8

corneal abrasion, glaucoma

Question 9

Fussy infant, DDx, oral causes?

Answer 9

Thrush

Question 10

Fussy infant, DDx, GU causes?

Answer 10

Obstructed inguinal or ovarian hernia, testicular torsion

Question 11

Fussy infant, DDx, neurological causes?

Answer 11

Pseudotumor cerebri, developmental delay

Question 12

Fussy infant, DDx, behavioral causes?

Answer 12

Postpartum depression, family stress, disorders of temperament regulation

Question 13

Fussy infant, DDx, hematologic causes?

Answer 13

Hemarthrosis, CNS hemorrhage or thrombosis, intra-abdominal hemorrhage, sickle cell pain crisis

Question 14

Hemophilia, common sites of involvement

Answer 14

Joint, muscles, iliopsoas with risk of neurovascular compromise, head, spine, GI, renal

Question 15

Hemophilia in joint

Answer 15

S: warmth and discomfort with movement followed by swelling
Sg: Tenderness, effusion, reduced ROM
Wu: XR to confirm Fx, no test needed to confirm bleed

Question 16

Hemophilia in muscles

Answer 16

S: muscle pain worse on stretching or contracting it
Sg: Tension and tenderness on palpation, swelling
Wu: Assess NV bundle, esp w/ deep muscles, Hgb as needed to check for blood loss

Question 17

Hemophilia affecting iliopsoas w/ risk of NV compromise

Answer 17

S: Pain in lower abdomen, groin, lower back
Sg: Pain on extension but not on rotation of hip joint
Wu: US/CT/MRI to Dx and monitor extent of bleed, Hgb

Question 18

Hemophilia affecting head

Answer 18

S: Sudden severe HA
Sg: Focal neurologic deficits
Wu: CT/MRI

Question 19

Hemophilia affecting spine

Answer 19

S: Sudden severe back pain
Sg: Focal neurologic deficits
Wu: CT/MRI

Question 20

Hemophilia in GI system

Answer 20

S: Hematochezia, Melena, Hematemesis
Sg: Sudden unexplained pallor, tense and distended abdomen, shock
Wu: US/CT/MRI

Question 21

Hemophilia in renal system

Answer 21

S: Painless hematuria often triggered by NSAIDs, trauma or exertion, pain once clots form
Wu: r/o other causes of hematuria such as infection, stones, neoplasm

Question 22

Epidemiology of Hemophilia

Answer 22

MC hereditary bleeding disorder in USA, incidence: 1/5000 live male births. CNS bleeding is rare with an incidence btw 2-8%, <10% of this bleeding happens within the spinal canal

Question 23

What is Hemophilia?

Answer 23

X linked bleeding disorder

Question 24

Hemophilia A, what is the defect?

Answer 24

Deficiency in Factor VIII

Question 25

SEH?

Answer 25

Spinal Epidural Hematoma

Question 26

Why are infants w/ hemophilia at high risk for SEH?

Answer 26

Cervical musculature starts to develop btw 4-6 m, increased cervical motion in this area (due to acceleration and deceleration of the head in babies with poor head control) –> cervical spine injury and potential for hemorrhage

Question 27

Another reason for higher risk for SEH in infants w/ hemophilia?

Answer 27

They typically do not start prophylactic factor treatment until > 1 y or after their 1st joint or muscle bleed once ambulatory

Question 28

Types of spontaneous SEH?

Answer 28

venous - arterial

Question 29

What happens in venous SEH?

Answer 29

Absence of valves in the venous plexus allows for venous backflow and sudden increases in pressure (crying, straining, coughing, sneezing) lead to spontaneous rupture of small veins

Question 30

What happens in arterial SEH?

Answer 30

It is thought that the higher pressure of arterial bleeding could result in cord compression with a rapid onset in patients who previously performed strenuous activities

Question 31

SEH in infants? Clinical manifestations?

Answer 31

Nonspecific symptoms: unexplained irritability, inconsolable crying, poor feeding or refusal to bear weight. Some infants can develop Torticollis and Horner syndrome, although this is rare
Classic clinical triad may be difficult to assess
Specific neurological signs: decreased neck ROM, diminished arm reflexes, decreased strength, tone and sensation of upper extremities

Question 32

What is the classic clinical triad of SEH?

Answer 32

1. Radicular pain, 2. Severe localized spinal pain, 3. Sensorimotor deficits
   (hard to assess in infants)

Question 33

Imaging for SEH?

Answer 33

Modality of choice: MRI

If MRI not available –> Spinal US

Question 34

Management of suspected SEH in Hemophilia

Answer 34

Initial Tx: empirical factor replacement even < imaging to achieve factor levels of 100%
Cons surgical decompression with or without laminectomy, but may be deferred if there is a stable neurologic exam + successful correction of the underlying coagulopathy

Question 35

Multidisciplinary approach for SEH and Hemophilia

Answer 35

Pediatric Hematologist, Neurosurgeon and a Pediatric Intensivist

Question 36

What is Horner syndrome?

Answer 36

aka Oculosympathetic paresis: Classic neurologic syndrome with ptosis, miosis and anhydrosis due to a lesion along the sympathetic pathway that supplies the head, eye and neck.
Evaluate for an underlying disorder and strongly consider paraspinal masses as an etiology