Disclosures None. Common Neurosurgical Problems Seen in Office Encounters. Macrocephaly Low Back Pain Sacral Dimple Concussion Chiari Malformation

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Common Neurosurgical Problems Seen in Office Encounters When to Manage, When to Refer Andrew Jea MD FAAP Professor and Chief of Pediatric Neurosurgery Riley Hospital for Children Indiana University School of Medicine Goodman Campbell Brain and Spine Disclosures None Topics Macrocephaly Low Back Pain Sacral Dimple Concussion Chiari Malformation

Macrocephaly Head circumference for age percentiles: Girls, birth to 36 months Macrocephaly Cc: 6 month old baby girl presents to your office with a large head Birth 12 24 36 Age (months) Head circumference for age percentiles: Girls, birth to 36 months Macrocephaly Cc: 6 month old baby girl presents to your office with a large head History: No inconsolable irritability, no persistent nausea/vomiting, no lethargy, no h/o seizures. Meeting developmental milestones. Father with a large head also. Birth 12 24 36 Age (months)

Head circumference for age percentiles: Girls, birth to 36 months Macrocephaly Cc: 6 month old baby girl presents to your office with a large head History: No inconsolable irritability, no persistent nausea/vomiting, no lethargy, no h/o seizures. Meeting developmental milestones. Father with a large head also. Examination: Awake and alert; enlarged pulsatile anterior fontanelle; no splaying of sutures; no sunsetting of eyes. Otherwise, neurologically normal. Birth 12 24 36 Age (months) Head circumference for age percentiles: Girls, birth to 36 months Macrocephaly Baby continues to do well neurologically, meeting developmental milestones with no signs or symptoms of increased intracranial pressure. Birth 12 24 36 Age (months) Head circumference for age percentiles: Girls, birth to 36 months Macrocephaly Baby continues to do well neurologically, meeting developmental milestones with no signs or symptoms of increased intracranial pressure. At 12 and 18 months, head circumference begins to plateau. Birth 12 24 36 Age (months)

Head circumference for age percentiles: Girls, birth to 36 months Macrocephaly Baby continues to do well neurologically, meeting developmental milestones with no signs or symptoms of increased intracranial pressure. At 12 and 18 months, head circumference begins to plateau. And at 24 months, head circumference follows an appropriate growth curve above the 95 th %ile. Birth 12 24 36 Age (months) Macrocephaly When to Refer Hydrocephalus Rapidly increasing head circumference plus: Bulging anterior fontanelle Splayed sutures Sunsetting or upward gaze palsy Bradycardic or apneic spells Developmental delay Papilledema CN VI palsy or palsies When ok to Manage Familial macrocephaly Benign extra axial fluid collections of infancy Ex vacuo enlargement of CSF spaces (secondary to atrophy) Macrocephaly Familial macrocephaly Only abnormality is a large head without any other underlying conditions Usually there is a family history of large heads (a family trait) Often associated with benign extra axial fluid collections of infancy

Macrocephaly Benign extra axial fluid collections of infancy Rapid head growth in first few months of life Rate of growth then normalizes by 15 18 months of age Neurological examination and development usually normal Considered a benign, self limited condition between 3 12 months and resolves spontaneously by 2 3 years of age Low Back Pain Low Back Pain Cc: 15 yo boy presents to your office with a two week history of low back pain

Low Back Pain Cc: 15 yo boy presents to your office with a two week history of low back pain History: Onset associated with football injury; no pain in legs; no weakness in legs. No urinary or bowel movement accidents. No fever. No h/o malignancy. Low Back Pain Cc: 15 yo boy presents to your office with a two week history of low back pain History: Onset associated with football injury; no pain in legs; no weakness in legs. No urinary or bowel movement accidents. No fever. No h/o malignancy. Examination: motor examination limited by pain; normal reflexes; no long tract signs; exquisite tenderness with palpation of paraspinous muscles Low Back Pain When to Refer Unremitting pain after 6 8 weeks of conservative treatment h/o malignancy/arthritis/collagen vascular disorder Radicular leg pain Association with fever Pain at night/rest Bowel and bladder dysfunction When ok to Manage No red flags Improving pain

Low Back Pain Estimated prevalence of 1.1 66% in children Etiologies Infectious Neoplastic Traumatic Inflammatory Musculoskeletal Congenital Degenerative Low Back Pain Initial management extrapolated from adult management Most episodes self resolve Conservative therapy should be first line treatment Physical therapy Avoidance of strenuous physical activities NSAIDs and muscle relaxants (avoid narcotics) Followup in 6 8 weeks Improvement > continuation of conservative treatment Failure to improve > referral Sacral Dimple

Sacral Dimple Cc: 7 week old baby girl come to your office with a sacral dimple noted by parents since birth Sacral Dimple Cc: 7 week old baby girl come to your office with a sacral dimple noted by parents since birth History: No drainage; no UTIs; soils diapers regularly; no h/o meningitis Sacral Dimple Cc: 7 week old baby girl come to your office with a sacral dimple noted by parents since birth History: No drainage; no UTIs; soils diapers regularly; no h/o meningitis Examination: Moves legs symmetrically and strongly; intergluteal sacral dimple, tracking caudally. No other cutaneous stigmata of spinal dysraphism. No scoliosis. No leg length discrepancy or clubfoot deformity.

Sacral Dimple When to Refer Located above the gluteal fold Deviated gluteal cleft Rostral tract Association with other cutaneous markers Lipomatous mass Hemangioma Tuft of hair Scoliosis Orthopedic anomalies Leg length discrepancy Clubfoot deformity Sacral Dimple When to Refer Located above the gluteal fold Rostral tract Association with other cutaneous markers Lipomatous mass Hemangioma Tuft of hair Scoliosis Orthopedic anomalies Leg length discrepancy Clubfoot deformity Older child: leg muscle atrophy; leg weakness; abnormal gait (toe walking) When ok to Manage Intergluteal sacral dimple No other stigmata worrisome for spinal dysraphism Neurologically normal Sacral Dimple Intergluteal sacral dimples usually result in a caudally directed fibrovascular stalk that attaches to the tip of the coccyx These benign sacral dimples must be differentiated from the ostium of a dermal sinus tract, which are usually midline above the gluteal cleft Missed dermal sinus tract could lead to repeated bouts of meningitis and attendant morbidities

Concussion Concussion Cc: 16 yo boy presents to your office 2 days after suffering a concussion while playing football Concussion Cc: 16 yo boy presents to your office 2 days after suffering a concussion while playing football History: No LOC at time of injury; remembers events preceding and following injury; still with headaches, dizziness, and difficulty with concentration. No prior concussions.

Concussion Cc: 16 yo boy presents to your office 2 days after suffering a concussion while playing football History: No LOC at time of injury; remembers events preceding and following injury; still with headaches, dizziness, and difficulty with concentration. No prior concussions. Examination: No cranial nerve deficits; normal strength and reflexes; normal gait. Moderate sway with Romberg testing. Concussion Cc: 16 yo boy presents to your office 2 days after suffering a concussion while playing football History: No LOC at time of injury; remembers events preceding and following injury; still with headaches, dizziness, and difficulty with concentration. No prior concussions. Examination: No cranial nerve deficits; normal strength and reflexes; normal gait. Moderate sway with Romberg testing. CT: no skull fracture; no intracranial hemorrhage Concussion When to Refer Almost always The Zackery Lystedt Youth Sports Concussion Law When in doubt, sit them out... Then refer! Altered mental status Worsening headaches Vomiting Seizures Deficits in motor or sensory function Neck pain LOC > 30 seconds

The Zackery Lystedt Story Concussion http://www.cdc.gov/headsup/providers/training/index.html When ok to Manage Completion of the online concussion training for health care providers provided by the CDC Understanding the 5 step Return to Play progression Concussion Goal of managing child with concussion is to speed recovery and prevent secondary injury?long term effects: cognitive and psychological Second impact syndrome: malignant (and fatal) cerebral edema Cognitive rest Temporary absence from school Shortening school day Reduction of school workload Allowing more time to complete tasks and tests

Concussion Physical rest Restriction from physical exertion until asymptomatic (could take up to 7 10 days, and in some cases, even longer) Return to Play (asymptomatic for > 24 hours prior to starting pathway) Step 0: No same day play Step 1: light aerobic activity exercise bike, walk, light jog; no heavy lifting, jumping, or hard running Step 2: moderate activity moderate jog; more intensive exercise bike; and ok for weightlifting at light weights Step 3: heavy, non contact activity high intensity running and biking; weightlifting at usual weights Step 4: practice and full contact Step 5: competition Concussion Return to play (personal biases) Second concussion > end season Third concussion > end participation in that particular sport.. period Chiari Malformation

Chiari Malformation Cc: (Same) 16 yo boy presents to your office 2 days after suffering a concussion while playing football History: No LOC at time of injury; remembers events preceding and following injury; still with headaches, dizziness, and difficulty with concentration. No prior concussions. Examination: No cranial nerve deficits; normal strength and reflexes; normal gait. Moderate sway with Romberg testing. CT reading: no skull fracture; no intracranial hemorrhage; INCIDENTAL CHIARI MALFORMATION Chiari Malformation MRI done for further workup confirms Chiari malformation type I; no syrinx; effacement of subarachnoid space at the craniocervical junction Chiari Malformation MRI done for further workup confirms Chiari malformation type I; no syrinx; effacement of subarachnoid space at the craniocervical junction Further questioning: no h/o headaches (specifically occipital headaches that worsen with Valsalva maneuvers); no neck pain; no arm pain; no difficulty with swallowing; no difficulty with breathing; no new hoarseness of the voice; no arm pain or weakness; no difficulty with gait or balance

Chiari Malformation Further examination: no downbeat nystagmus; no Lhermitte s sign Chiari Malformation When to Refer Occipital headaches that worsens with coughing, laughing, sneezing, and bearing down Cerebellar signs and symptoms: difficulty with coordination; difficulty with balance and gait Lower brainstem signs and symptoms: dyspnea and dysphagia; changes in voice Upper spinal cord signs and symptoms: neck and arm pain; arm and hand weakness Presence of a syrinx on neuroimaging When ok to Manage Asymptomatic Chiari Malformation Chiari I malformation most common of form Chiari I malformation should be differentiated between cerebellar tonsillar ectopia (a normal variant) and Chiari II malformation Chiari I malformation has peg like tonsillar tips, while cerebellar tonsillar ectopia maintains tonsil with rounded tips Chiari II malformation exclusively seen in cases of spina bifida

Chiari Malformation Occipital headaches exacerbated by Valsalva maneuvers represent the most frequent (and earliest) clinical presentation Currently, the natural history of Chiari I malformation is unknown; likewise, ideal treatment for Chiari I malformation is unknown Bony decompression alone vs. bony decompression plus intradural exploration More References International Society for Pediatric Neurosurgery. The ISPN Guide to Pediatric Neurosurgery. http://ispn.guide. Email: ajea@goodmancampbell.com Office No.: 317 396 1401 Cell No.: 317 478 6903 Thank you!