Primary Spinal Developmental Anomalies

Question 1

Surgical closure of myelomeningocele should be done within
what time?
● A. 24 hours
● B. 48 hours
● C. 56 hours
● D. 3 days
● E. 7 days

Answer 1

A. 24 hours

Question 2

What is the most common complicating condition associated with myelomeningocele?
● A. Neural injury
● B. Aseptic meningitis
● C. Hydrocephalus
● D. CSF leak
● E. Bladder injury

Answer 2

C. Hydrocephalus

Question 3

Myelomeningocele most commonly involves which region of spine?
● A. Thoracic
● B. Lumbosacral
● C. Thoracolumbar
● D. Lumbar
● E. Sacral

Answer 3

B. Lumbosacral

Question 4

If a myelomeningocele patient has increasing scoliosis, increasing spasticity, worsening gait, or deteriorating urodynamics, what should be the first workup for?
● A. Syrinx
● B. Brain stem compression
● C. Dermal sinus
● D. Lipoma
● E. Shunt malfunction

Answer 4

E. Shunt malfunction

Question 5

It is difficult to differentiate a tethered cord from congenitally low-lying conus. In congenitally low-lying conus, the filum diameter is generally normal, and the conus should not be
below which level?
● A. L3
● B. L4
● C. L5
● D. S1
● E. S2

Answer 5

A. L3

Question 6

A patient with SCM type 1 is has which of the following?
● A. Two hemicords in one dural sac
● B. Two hemicords in separate dural sac
● C. Anterior sacral meningocele
● D. Open myelomeningocele
● E. Thoracic dermal sinus tract

Answer 6

B. Two hemicords in separate dural sac

Question 7

Which of the following is true about spinal neuroenteric cyst?
● A. Anterior to the spinal cord and extradural
● B. Posterior to the cord and extradural
● C. Anterior to the cord and intradural
● D. Anterior and posterior to the cord
● E. Intradural and intramedullary

Answer 7

A. Anterior to the spinal cord and extradural

Question 8

Which of the following is the clinical triad of Klippel-Feil syndrome?
● A. Low anterior hairline, bervicollis, and limitation of movement
● B. Low posterior hairline, bervicollis, and limitation of movement
● C. Low posterior hairline, bervicollis, and normal range of movement
● D. Low posterior hairline, bervicollis, and fusion of two vertebrae
● E. Torticollis, bervicollis, and fusion of two vertebrae

Answer 8

B. Low posterior hairline, bervicollis, and limitation of movement

Question 9

What is the most common presenting sign and symptom in tethered cord?
● A. Hypertrichosis
● B. Posterior spina bifida
● C. Gait difficulty
● D. Subcutaneous lipoma
● E. Sensory deficit

Answer 9

B. Posterior spina bifida

Question 10

What is the congenital anomaly in lumbosacral nerve root type 3?
● A. Conjoined nerve root with common dural sheath
● B. Two nerve roots exit through one foramen
● C. Adjacent nerve roots are connected by anastomosis
● D. Two nerve roots exit through separate foramen
● E. None of the above

Answer 10

C. Adjacent nerve roots are connected by
anastomosis

Question 11

Spinal arachnoid cysts are almost always dorsal and most common in thoracic spine. Following statements regarding arachnoid cysts are correct except?
● A. Etiologies can be congenital, post-traumatic, or post-infectious
● B. Symptoms of myelopathy are strong indication of surgery
● C. Pain is a strong indication of surgery
● D. It can be treated with percutaneous procedures like needle aspiration or needle fenestration
● E. Open procedures for its treatment are surgical resection, cyst fenestration, or shunting

Answer 11

C. Pain is a strong indication of surgery

Question 12

Myelomeningocele (MM) is a spinal defect called spina bifida aperta or spina bifida cystica in which there is congenital defect in vertebral arches, with cystic dilatation of meninges and structural or functional abnormality of spinal cord. Its incidence can be lowered down by using prenatal folate. What percentage of MM patients develop hydrocephalus?
● A. 45 to 65%
● B. 65 to 85%
● C. 35 to 45%
● D. 85 to 90%
● E. 25 to 35%

Answer 12

B. 65 to 85%

Question 13

Surgical closure of meningomyelocele defect should be done within 36 hours after birth. Following are steps of management of myelomeningocele before surgical closure of the defect except?
● A. If the lesion is ruptured, antibiotics are started and the defect is covered with Telfa soaked in N/S or R/L
● B. Lowest level of neurological function is identified for correlation after surgical repair
● C. Orbitofrontal circumference is taken and head US is also done to rule out hydrocephalus
● D. No need for orthopedic consultation for kyphotic or scoliotic deformities
● E. The patient is started on regular urinary catheterization and urological consultation is taken

Answer 13

D. No need for orthopedic consultation for kyphotic or scoliotic deformities

Question 14

Lipomyeloschisis is dorsal spinal dysraphism with lipoma which includes dural lipoma, lipomyelomeningocele, or fibrolipoma of filum terminale. What is lipomyelomeningocele?
● A. A visible defect on back with protrusion of fat and meninges
● B. A lipoma on back with protrusion of meninges and neural tissue
● C. A tuft of hair on skin with a subcutaneous lipoma that passes through the defect in lumbosacral fascia, vertebral neural arch, and dura
● D. A subcutaneous lipoma that does not merges with low-lying tethered cord
● E. Lipoma and meninges with protruding neural tissue separately visible on skin surface

Answer 14

C. A tuft of hair on skin with a subcutaneous lipoma that passes through the defect in lumbosacral fascia, vertebral neural arch, and dura

Question 15

What is the suggested treatment of filum terminale lipoma?
● A. Prophylactic division of fatty filum terminale without symptoms
● B. Removal of only fat with no division of filum terminale
● C. Division of fatty filum terminale if symptomatic
● D. Fatty filum sectioning for asymptomatic lesion if surgery is being performed for some other reason
● E. Both C and D

Answer 15

E. Both C and D

Question 15

All of the following statements are true regarding lipomyeloschisis except?
● A. It consists of removing abnormal fatty tissue
● B. Untethering of cord is done during surgery
● C. Surgery is essentially performed before 2 months of age
● D. Plain lumbosacral spine X-ray will show spinal bifida in most cases
● E. Most common presentations are back mass and bladder problems

Answer 15

C. Surgery is essentially performed be-
fore 2 months of age

Question 16

Following statements about spinal dermal sinus are correct except?
● A. Failure to separate neural ectoderm from dermal ectoderm at the time of closure results in myelomeningoceledefect
● B. It is recommended that surgical excision should only be done if symptomatic causing neurologic or infection symptoms
● C. There can be epidermoid cyst or dermoid cyst along the path of sinus
● D. These are potential pathways for intradural infection with chances of meningitis
● E. If present at birth, ultrasound is the best means to evaluate for spina bifida or mass inside the canal

Answer 16

B. It is recommended that surgical exci-
sion should only be done if symptomatic causing neurologic
or infection symptoms

Question 17

Congenital fusion of two or more cervical vertebrae is a defect that results from failure of normal segmentation of cervical somites between 3 and 8 weeks of gestation. This condition presents with classic clinical triad of low posterior hairline, shortened neck, and limitation of neck movements. What is this condition is called?
● A. Fused cervical deformity
● B. Cervical myelopathy with fused vertebrae
● C. Klippel-Feil syndrome
● D. Platybasia
● E. Basilar invagination

Answer 17

C. Klippel-Feil syndrome

Question 18

Which of the following is true regarding tethered cord syndrome?
● A. It is low-lying conus medullaris with thickened filum terminale and intradural lipoma
● B. The most common presenting symptom of tethered cord syndrome in adults is pain
● C. Filum terminale is distinguished from nerve root by the presence of characteristic squiggly vessel on the surface and whiter appearance under microscope
● D. Diagnosis must be made clinically in patients with myelomeningocele as all of these patients will have tethering radiographically
● E. All of the above

Answer 18

E. All of the above

Question 19

All of the following are true regarding split cord malformation (SCM) except?
● A. Type 1 SCM is two hemicords each with central canal and surrounding pia in a separate dural tube
● B. Type 1 is also called diastematomyelia
● C. There is no rule of untethering of cord in these patients
● D. Type 2 is two hemicords within a single tube
● E. Type 2 is also called diplomyelia

Answer 19

C. There is no rule of untethering of cord
in these patients