FAR EASTERN UNIVERSITY DR. NICANOR REYES MEDICAL FOUNDATION Regalado Avenue.

, corner Dahlia Street, Fairview, Quezon City Department of Child Health

SPLIT CORD MALFORMATION

A case of a newborn, female with Diastematomyelia

Sarmiento, Angela D., MD

First Year Resident

Introduction

This is a case of C.A., a newborn female delivered via spontaneous vaginal delivery with an APGAR score of 9 and 10, birthweight of 2,930g, appropriate for gestational age who was born with a pedunculated mass at the lumbosacral area. Upon further examination and with diagnostic imaging using ultrasound, was diagnosed with Diastematomyelia.

She stayed in the neonatal intensive care unit for 13 days and during hospitalization, she underwent surgical repair of myelomeningocele and excision of bony spur with release of the tethered cord, which she tolerated well.

Split cord malformation or Diastematomyelia is a rare and complex condition. Patients may remain asymptomatic but in 50% of cases, it tends to be associated with abnormalities of the vertebral bodies like hemivertebra, hypoplasia, kyphoscoliosis, spina bifida, and myelomeningocele, including fusion defects, unilateral foot abnormalities, talipes equinovarus, claw toes, atrophy of the gastrocnemius, and intestinal malrotation just to name a few. Due to the rarity of this case, pathogenesis and management of these complex anomalies still remains controversial but the diagnosis has become easy with the advent of neuroimaging techniques. Early recognition of diastematomyelia is critical in order to recognize all the associated anomalies and to plan the management strategies.

Her condition was marked by a cleft in the spinal cord, which was divided longitudinally by a septum of bone emanating from the posterior vertebral arch. The cord or cauda equina was impaled by the bony spur and differential growth between vertebral column and spinal cord results in the stretching of the cord above its point of fixation resulting in a tethered cord.

Objectives: y To discuss a case of a newborn female diagnosed with diastematomyelia, its diagnosis, management and prognosis.

Neonatal History

C.A., born to a 23 year old, Gravida 1 Para 1 (1001), blood type A positive, HBsAg non-reactive mother at 37 weeks age of gestation by LMP after a pregnancy complicated with urinary tract infection on the 28th and 37th weeks age of gestation, treated with Cephalexin 500mg/tablet, 1 tablet thrice a day for 7 days. Urinalysis was done on both occasions revealed normal results. Congenital scan done on the 28th week of pregnancy revealed normal results. On her 38th week of pregnancy, patient had episodes of non-productive, non-distressing cough which resolved spontaneously after three days without any medical intervention.

Maternal History

The mother had regular pre-natal check up as well as regular intake of multivitamins and ferrous sulfate. Folic acid supplementation was started during the second month of her pregnancy. She is a nonsmoker, non-alcoholic drinker, non-diabetic, non-hypertensive and non-asthmatic. She has no known food or drug allergy. She has no history of exposure to radiation, trauma and toxic chemicals.

Physical Examination:

General Survey: awake, active with good cry and reflexes with the following vital signs HC: 32cm (p25) CR: 140s/min CC: 32cm RR: 40s/min AC: 29cm Temp: 37.10C BL: 48cm (p50) BW: 2,930g (p50)

HEENT: flat, soft, open anterior and posterior fontanel measuring 3x2cm and 2x1cm respectively. Molding and a 2x2cm caput succedaneum in the parietal area with gaping suture lines were noted. Pink palpebral conjunctivae, white sclerae, ear with instant recoil, patent ear canals, patent nostrils, intact lips and palate. Neck: Supple, with no mass or crepitations Chest: Symmetrical chest expansion, no retractions clear breath sounds, good air entry Heart: Adynamic precordium, normal rate and regular rhythm, distinct heart tones, no murmur. Abdomen: slightly globular, soft, no mass, no organomegaly, with bowel sounds Genitalia: Normal looking external female genitalia, with labia majora covering the labia minora and clitoris.

Rectal exam: no skin tag, no fissure, good sphincter tone, empty rectal vault, with greenish material upon withdrawal of examining finger and with anal wink. Trunk and Spine: straight spine, no tufts of hair, with 5x6 cm soft, skin covered, pedunculated lumbosacral mass, and a pit 2 cm from the mass were noted. Skin: Pink all over with no cyanosis, CRT < 2 seconds.

Neurologic Examination: Cerebrum: alert and active Cerebellum: no nystagmus Cranial Nerves: I: not assessed II: pupils are round, and reactive to light, follows brightly colored objects. (+) Red orange reflex III, IV & VI: with spontaneous eye movements V: (+) bicorneal reflex VII: No facial asymmetry, with good suck VIII: intact gross hearing IX & X: can swallow, with good cry XI: can turn head from side to side XII: no fasciculations or deviation

Sensory: With anal wink Motor: with active movement on all extremities Reflexes: (+) moro, (+) sucking, (+) rooting, (+) palmar grasp, (+) plantar, (+) stepping, (+) placing

Course in the ward:

The patient stayed in the ward for 13 days.

C.A., was born term, via spontaneous vaginal delivery with an APGAR score of 9 and 10, birth weight of 2,930 grams, appropriate for gestational. Upon physical examination, the patient was awake, active, with good cry and reflexes, with urine output and one episode of meconium, with stable vital signs and normal anthropometric measurements. Pertinent physical examination revealed a straight spine, with no tufts of hair on the sacral area, with a 5x6cm, soft, non-erythematous, skin covered, pedunculated mass on the sacrococcygeal area and a pit 2cm from the mass was noted. Rectal examination was done and revealed no skin tag, no fissure, good sphincter tone, empty rectal vault, with greenish material upon withdrawal of examining finger and with anal wink. She was mixed feed with NAN HW 1:1 dilution and breastfeeding every 3-4 hours. Initial assessment was Meningocoele. She was referred to a neonatologist who suggested for a Lumbosacral CT-Scan with contrast. The CT-Scan revealed a dorsal lumbar meningocoele and midline bony sagittal spur at L3 level.

On the second hospital day, the patient was awake, active, with good cry and reflexes, with adequate urine output and with bowel movement. A referral to Neurology and Neuro-surgeon was done. Diagnosis at the time of examination was Meningocoele with probable dermal sinus tract. Spinal ultrasound done revealed myelomeningocoele. Along the level of myelomeningocoele, doubling (diastemyelia) of the spinal cord and tethered cord was also noted. There was no evident communication of the skin pit to the myelomengocoele. A tethered cord was also noted. The patient was scheduled for elective operation. Blood typing was done which revealed O positive.

On the 5th hospital day, squinting of the left eye was noted hence she was seen by the ophthalmologist. Upon physical examination, matting of the eyelashes over the right eye, with weak lateral rectus muscle. Fundoscopic findings on both eyes revealed cup disc ratio of 0.3, well defined disc boarders with slightly pale optic disc. Impression was Conjunctivitis, OD and probable cranial nerve palsy, OS. Erythromycin eye ointment was started.

On the 7th hospital day, Prothrombin time, Activated thromboplastin time, BUN, Creatinine, Sodium, Potassium, KUB ultrasoind and Chest X-ray (AP) revealed normal results. Ruling out associated

congenital anomalies, a nephrology service consult was done. She was also cleared by a Cardiologist since there is a rare association between meningocoele with conotruncal defects, usually transposition of great artery, and tetralogy of fallot and babies are also usually cyanotic.

On the 8th hospital day, the patient was awake, active, with good cry and reflexes, with adequate urine output and with bowel movement. Head circumference was 34cm. Repair of

myelomeningocoele with excision of bony spur was tolerated well. She had febrile episodes postoperatively, hence given Paracetamol. She was transfused with 40cc of packed red blood cells for 6 hours. Post blood transfusion Complete blood count with platelet count revealed normal results. Feeding was started at 15cc per feeding every 3 hours with strict aspiration precautions. She was noted to have no urine output for 10 hours (last urine output was before surgical operation). Fluid challenge with 30cc plain LR IV push was given at 10cc/kg. The patient had adequate urine output afterwards.

On the 9th hospital day, feeding was maintained at ad libitum per feeding every 3 hours with strict aspiration precautions. Complete blood count with platelet count revealed normal results. No discharge, redness or swelling was noted. Whitish plaques all over the tongue was noted, hence Nystatin swab to affected area thrice a day for 14 days was started.

On the 11th hospital day, the patient was awake, active, with good cry and reflexes, with adequate urine output and with bowel movement. Head circumference was 35cm. Urinalysis was done and revealed leukocytosis. Feeding was maintained at ad libitum per feeding every 3 hours with strict aspiration precautions. Urine culture and sensitivity done revealed no growth after 48 hours of incubation period. Complete blood count with platelet count revealed normal results. Cefazolin was discontinued. Ceftriaxone 165mg through slow intravenous infusion by syringe pump over 30 minutes, once a day was started (TD: 50.8m/kg/day). Dexamethasone was discontinued.

On the 12th hospital day, the patient was awake, active, with good cry and reflexes, with adequate urine output and with bowel movement. Head circumference was 35.5cm. Post-operative site was dry with no signs of inflammation.

On the 13th hospital day, the patient was awake, active, with good cry and reflexes, with adequate urine output and with bowel movement. Head circumference was 36cm. Feeding was

maintained at ad libitum per feeding every 3 hours. The patient may go home anytime with improved health. Cranial ultrasound as out-patient basis was requested. Ceftriaxone was shifted to oral Cefixime to complete 7 days (TD: 1.8mg/kg/dose) and Nystatin oral suspension, swab to oral cavity thrice a day for 10 more days were given as home medications. Final diagnosis was: Live, born term, single, female, delivered via normal spontaneous delivery with an APGAR score of 9 and 10, birth weight of 2,930g, appropriate for gestational age. Urinary tract infection. Oral Candidiasis. S/P Repair of Myelomeningocoele, T/C Cranial Nerve Palsy.

The patient came in for follow up. She was awake, active, with good cry and reflexes, afebrile and not in cardiorespiratory distress. Vital signs and antrophometric measurements: Cardiac rate of 121/min, respiratory rate of 41/min, and temperature of 36.3. Head circumference of 36cm, chest circumference of 32cm, abdominal circumference of 32cm, length of 49cm and weight of 3.3kg. Physical examination revealed open, flat, soft, anterior and posterior fontanels with gaping sutures, pink palpebral conjunctivae, with white sclera, with inward deviation of left eye, patent ear canals, patent nostrils, moist lips and buccal mucosa. Incision with intact sutures at midline sacral area with no redness, swelling or discharge was noted. Cranial ultrasound done revealed: Minimal communicating hydrocephalus. Chiari II. She was referred back to a neurologist and the plan was for repeat of cranial ultrasound after one month, monthly follow-up and close monitoring of head circumference. If hydrocephalus progresses, ventriculoperitoneal shunting might be warranted. She was referred to Ophthalmology services wherein pertinent physical examination revealed bilateral inward primary gaze was noted. Assessment was T/C bilateral rectus palsy, probably secondary to Hydrocephalus or increased intracranial pressure, or maldevelopment or non-development of Cranial nerve VI. She was sent home with final diagnosis of: Live, born term, single, female, delivered via normal spontaneous delivery with an APGAR score of 9 and 10, birth weight of 2,930g, appropriate for gestational age. Oral Candidiasis. S/P Repair of Myelomeningocoele, Bilateral Lateral Rectus Palsy.

Laboratory Results: CT Scan of the Lumbo-sacral Spine (Januay 25, 2011) y There is a wide dorsal dyraphic spinal defect in the lumbar and sacral area. y A CSF filled sac measuring 4.6 x 5.4 x 1.4 cm is seen herniating through a dorsal dural sac defect at L3-L4. No abnormal enhancement is noted within this cystic lesion on post contrast study. y A midline bony sagittal spur is seen at L3 level separating the spinal canal into two compartments. y Impression: Dorsal lumbar meningocele, Midline bony sagittal spur L3 Level

Spinal Ultrasound (January 26, 2011) y Along the level of myelomeningocele there is doubling (diastemyelia) of the spinal cord. A focal anterior spinal column opening is seen with the widest diameter of 1.1cm representing myelomeningocele measuring 2.7 x 2.5 x 2.7cm (volume 10cc). Doppler study shows vascularity. There is no evident communication between the skin pit to the myelomeningocele. Note of tethered cord with the conus medullaris seen along the level of L5-S1 vertebrae y Impression: Mylomeningocele

KUB Ultrasound (January 31, 2011) y Normal kidneys and urinary bladder sonographically

Blood Chemistry (January 31, 2011) Normal Values BUN Creatinine Sodium Potassium Chloride Ionized Calcium 1.1 4.3 27-88 135-148 3.5-5.30 96-106 1.12-1.32 Test 2.0umol/L 36.5umol/L 136.30mmol/L 5.11 mmol/L 107.2 mmol/L 1.33 mmol/L

January 31, 2011 PT Test Control Activity INR PTT Test Control Difference Ratio 11.7 11.9 106% 0.98 39.0 33.3 5.7 1.1

CBG (February 1, 2011) y 71mg/dL

Blood Type (February 1, 2011) y O POSITIVE

Urinalysis Normal Values Color Character pH Specific Gravity Protein Glucose Pus Cells RBC Yellow Clear to slightly hazy 6-7 1.010-1.030 Negative Negative 0-2 1-3 February 2, 2011 Yellow Turbid 6.5 1.010 Negative Negative 16-18 1-2

Cranial Ultrasound (February 5, 2011) y CBC Hemoglobin Hematocrit WBC Platelet Count Segmenters Lymphocytes Monocytes Eosinophils Stabs Impression: Unremarkabe brain sonogram Normal Values 14.5 22.5 0.44 0.72 9.4 34.0 150 400 0.54 0.62 0.25 0.33 0.03 0.07 0.01 0.03 0.03 0.05 Jan-31 14.3 0.41 11.6 281 0.31 0.48 0.19 0.01 0.01 Feb-2 15.5 0.45 19.42 344 0.69 0.17 0.10 0.02 0.02 Feb-4 14.50 0.42 16.96 436 0.41 0.55 0.04

Cranial Ultrasound (February 7, 2011) y y The lateral ventricles are minimally dilated with the following measurements: On mid-sagittal section, the fourth ventricle and the posterior fossa structures are in place and normal looking. There is beaking of the tectum of the midbrain. The cerebellum is pushed downward and posteriorly. The cerebral hemispheres are symmetrical and there is no shifting of midline structures. y Impression: Minimal communicating hydrocephalus. Chiari II.

Discussion:

The nervous system begins on the dorsal aspect of the embryo as a plate of tissue which eventually will invaginate and close dorsally to form the neural tube. Closure generally proceeds rostrally and caudally. The anterior end of the neural tube closes at approximately 24 days and the posterior ends at approximately 26 days. The essential defect in myelomeningocele is restricted failure of posterior neural tube closure. Approximately 80% of lesions occur in the lumbar area presumably because this is the last area of the neural tube. The large majority of the lesions are associated with dorsal displacement of the neural tissue, such that a sac is created on the back or in this case, a pedunculated, skin covered mass. The onset of myelomeningocile is probably no later than 26 days of gestation. Recognized causes of such defects include multi-factorial inheritance, single mutant genes, chromosomal abnormalities, specific drug teratogens and specific phenotypes of unknown causes. In C.A. s case, a congenital scan performed at the 28th week of pregnancy revealed normal results. There is also no familial history of neural tube defects in the family and there was no history of any exposure to certain teratogenic medications that may warrant the occurrence of the anomaly. Maternal history was complicated only by urinary tract infection on the 28th and 37th week age of gestation. She was given Cephalexin which is known to be a safe drug that can be used to treat urinary tract infection in pregnant women. C.A. s mother also had regular intake of folic acid (Folium OB) 10mg once a day which she started on the second month of gestation. Folate is intricately involved in the prevention and etiology of neural tube defects. To be effective, folic acid supplementation should be initiated before conception and continued until at least the 12th wk of gestation when neurulation is complete. All women of childbearing age and who are capable of becoming pregnant are advised to take 0.4 mg of folic acid daily. Myelomeningocele represents the most severe form of dysraphism involving the vertebral column and occurs with an incidence of approximately 1/4,000 live births. A myelomeningocele may be located anywhere along the neuraxis, but the lumbosacral region accounts for at least 75% of the cases. In this case, C.A. s was located on the lumbosacral area with a pit 2 cm from the pedunculated mass. The extent and degree of the neurologic deficit depend on the location of the myelomeningocele. A lesion in the low sacral region causes bowel and bladder incontinence associated with anesthesia in the perineal area but with no impairment of motor function. Often, examination of the infant shows a flaccid paralysis of the lower extremities, an absence of deep tendon reflexes, a lack of response to touch and

pain, and a high incidence of lower extremity deformities which are absent in this patient. She had good muscle tone with active spontaneous movement, adequate urine output and regular bowel movement during her stay in the nursery. During fetal development, the spinal cord occupies the entire length of the vertebral column, the conus medullaris in a child ultimately assumes a position at the level of L1. Spinal ultrasound done on her 2nd day of life revealed conus medullaris along the level of L5 to S1 vertebrae, hence, she was diagnosed with a tethered cord. This results when a thickened ropelike filum terminale persists and anchors the conus at or below the L2 level. Neurologic signs may develop as a result of abnormal tension on the spinal cord, compromising blood supply, particularly during flexion and extension movements. In a study done by Pang and co-workers, the only other positive predictor of ventral tethering is the association of dermal sinus tract which was ruled out in this case through ultrasound of the spinal cord.

Since caudal neural tube formation by the processes of canalization results in the conus medullaris and filum terminale. The conus is usually prolonged and the filum terminale is thickened. Moreover, these structures frequently are tethered or fixed at their caudal end. This fixation is thought to impair the normal mobility of the lower spinal cord and as a consequence, movements of the lower spinal cord and as a consequence, movements of the trunk such as flexion and extension transmit tension through the prolonged conus to the spinal cord and cause injury. About 80% of cases exhibit a dermal lesion in the lumboscral area, consisting of abnormal collections of hair, or cutaneous dimples or tracts which was proven through spinal ultrasound that is absent in this case t. Surgical release of the tethered cord tends to halt progression of neurologic signs and prevents the development of dysfunction in asymptomatic patients. Diastematomyelia is division of the spinal cord into two halves by projection of a fibrocartilaginous or bony septum originating from the posterior vertebral body and extending posteriorly. It represents a disorder of neural tube fusion with the persistence of mesodermal tissue from the primitive neurenteric canal acting as the septum. Pang and coworkers proposed that the basic error of all spinal cord malformation occurs very early in the development of the embryo. During the transformation of the bilaminar to the trilaminar embryo in early grastrulation, which is one of the cardinal events in the formation of the notochord. Spratt demonstrated that the notochord becomes elongated by the addition of new cells to the caudal end of a short head process that points toward the cephalic end of the embryo. The notochord therefore grows rearward, as it were, along with backward elongation of the notochord is also concomitant with

shrinking of the primitive streak, as the stem cells from the latter structure are being exhausted to form the new germ layers. These cells must in some way achieve midline integration as they line up at the caudal end of the elongating notochord, to become a solid cord. The basic error in all spinal cord malformation is very possibly a failure of midline integration of the newly forming notochord cells. It may be due to a defective adhesion molecule or mistiming or even misplacement of the normal molecule. If this failure of midline integration occurs only for a brief moment in time, in a square pulse fashion, the result will be a small midline area of persistent adhesion between the ectoderm and the endoderm, flanked by two heminotochords. Whether primitive meninx cells, with their bivalent ability to form bone and parenchyma, are spatiotemporarily available to be incorporated into the edomesenchmal tract will determine if the hemicords are bisected by a bone spur in between two separate dural sacs. Given the pluripotential nature of mesenchymal cells, both dorsal and ventral fibrous septa are frequently associated or even embedded with large blood vessels, fat and muscle. If neural crests cells are also entrapped by the endomesenchyal tract, paramedian dorsal nerve roots (being central processes of dorsal ganglion cells,) and ganglia cells will form between the dorsal medial aspect of the hemicords and the dorsal dura along side the fibrous bands and vessels, giving rise to myelomeningocoele. The ectodermal elements may form a dermal sinus opening and tract continuous with the median septum. An important non-invasive initial evaluation is ultrasonography, a procedure made possible in newborn in part because of the poor ossification of posterior spinal elements. CT is useful in demonstrating anomalous bony structures and diastematomyelia spurs like in this case. A full anterior fontanel and split cranial sutures are helpful signs for the diagnosis of hydrocephalus, if myelomeningocele is not leaking CSF. Increasing head size and gaping suture lines present in our patient provides valuable information in the evaluation of hydrocephalus. In the case of C.A., head circumference taken initially at birth was 32cm, 33cm on the 3rd day of life, 34cm on the 4th day of life, 35.5cm on the 12th day of life and 36cm upon follow-up on the 13th day of life. The site of the lesion is also helpful in predicting the presence or imminent development of hydrocephalus. With thoracolumbar or lumbosacral lesions, the incidence of hydrocephalus is approximately 90%. Over 80% of infants who have hydrocephalus with myelomeningocele may have rapid ventricular dilatation leading to Arnold-Chiari hindbrain malformation. This is a major cause of neurological morbidity among infants in this case. Effective control correlates with ultimate neurological function.

Some 80-90% of patients with diastematomyelia are female. A variety of skin lesions mark the site of the defect. Most commonly these are tufts of hair or dimples but vascular malformations or dermal sinuses can also be present. Progressive sensorimotor deficits represent the most common clinical manifestation for diastematomyelia. The subject may experience weakness and spasticity of the lower extremities with awkward gait, incontinence of bladder and rectum and less commonly, posterior root pain. With suspicion aroused by cutaneous anomalies and neurologic dysfunction of the lower limbs and sphincters, diagnosis can be best made by means of MRI of the spinal cord. Surgical removal of the bony spur allows the cord to become freely movable. Although this does not alter the nonprogressive hypoplastic syndrome, it prevents the onset or arrests and even improve the progression of the syndrome. The more recent the neurologic deficit, the more likely it is to be reversible; hence, prophylactic surgery for this particular infant without neurologic deficit is indicated. The disturbances of neurological function depend on the level of the lesion. Particular attention should be paid to examination of motor sensory and sphincter function. In this case, the lesion is approximately at the level of L5 S1. Expected predicaments would involve knee flexion, ankle

dorsiflexion, ankle plantar flexion, cutaneous sensation on the lateral leg and medial foot (L5) and sole of foot (S1) but these problems were not encountered in this particular case. Assessment of the functional level of the lesion allows reasonable estimates of potential future capacities. The neurologic signs are thought to result from flexion and extension movements of the cord, which produce traction and additional trauma by the impaling septum. The prevalent notion is that early closure of the back lesion is optimal. The rationale for this approach has been the prevention of infection and the loss of motor function that may occur. Surgery is performed primarily to prevent development of neurologic deficits. The optimal timing of surgery in the infant with few or even no neurologic signs. It is controversial, but the combination of excellent preoperative imaging, microsurgical techniques and intraoperative monitoring of cord has decreased morbidity that treatment in the neonatal period before the onset of symptoms has been recommended. Surgical release of the tethered cord combined with the removal of the bony spur prevents deterioration and may partially reverse deficits acquired. Surgery is often done within a day or so of birth but can be delayed for several days. For a child who is born with a myelomeningocele and who is treated aggressively, the mortality rate is approximately 10 15%, and most deaths occur before age 4 yr, although life-threatening complications occur at all ages. At least 70% of survivors have normal intelligence, but learning problems and seizure disorders are more common than in the general population.

A full anterior fontanel and split cranial sutures are helpful signs for the diagnosis of hydrocephalus, if myelomeningocele is not leaking CSF. Increasing head size and gaping suture lines present in our patient provides valuable information in the evaluation of hydrocephalus. In the case of C.A., head circumference taken initially at birth was 32cm, 33cm on the 3rd day of life, 34cm on the 4th day of life, 35.5cm on the 12th day of life and 36cm upon follow-up on the 13th day of life. Upon follow up, her head circumference was 37cm with gaping suture lines. Her repeat cranial ultrasound done as out-patient revealed minimal communicating hydrocephalus, Chiari II malformation. Hydrocephalus is secondary to the Arnold-Chiari Malformaion which is part of myelomeningocele. With thoracolumbar or lumbosacral lesions, the incidence of hydrocephalus is approximately 90%. This is a major cause of neurological morbidity among infants in this case. About 15% of infants of this case will develop symptoms of hindbrain dysfunction like difficulty feeding, choking, stridor, apnea, vocal cord paralysis, pooling of secretions, and spasticity of the upper extremities, which, if untreated, can lead to death. In C.A., lateral rectus weakness or squinting of the left eye was seen during her 4th day of life. This Chiari crisis is due to downward herniation of the medulla and cerebellar tonsils through the foramen magnum leading to an increase in intracranial pressure. Approximately 10% Chiari malformations produce symptoms during infancy consisting of stridor, weak cry, and apnea. She was referred back to a neurologist and the plan was for repeat cranial ultrasound after 3 months, monthly follow-up and close monitoring of head circumference to note for the progression of hydrocephalus wherein surgery by shunting or by posterior fossa decompression may be warranted. Recommendation: Diastematomyelia is a complex condition usually associated with a number of other neurologic condition such as tethered cord and hydrocephalus. Magnetic resonance Imaging or MRI should be done in all patients with spinal cod malformation. Surgery must be performed as early as possible, before the onset of any neurological deficit. Close follow up is recommended to patients with diastematomyelia for early recognition of neurologic deficit.

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