The presence of the following should be noted:

Signs of hydrocephalus
Clubfeet
Flexion or extension contractures of hips, knees, and ankles
Kyphosis
Other abnormalities such as congenital heart disease; structural defects of the airway, gastrointestinal tract, ribs; developmental dysplasia of the hip; or ultrasound evidence of renal malformations such as hydronephrosis
Early complications such as CNS infection
A thorough neurologic examination should be performed. (See "Neurologic examination in children"). This should include:

Observation of spontaneous activity
Extent of muscle weakness and paralysis
Response to sensation
Deep tendon reflexes
Anocutaneous reflex (anal wink)
Surgical closure — The back lesion should be surgically closed within the first 24 to 48 hours after birth. This decreases the risk of CNS infection. Prophylaxis with broad spectrum antibiotics until the back is closed also reduces the risk of CNS infection. In a retrospective study of infants with back closure performed after 48 hours of age, ventriculitis occurred less with than without antibiotic prophylaxis (1 versus 19 percent) [39].

Hydrocephalus — Ventricular size should be evaluated soon after birth by ultrasound, CT, or MRI. Serial neuroimaging should be performed to identify the development of hydrocephalus. Progressive hydrocephalus should be treated by insertion of a ventriculoperitoneal shunt.

In some infants, simultaneous meningomyelocele repair and shunt placement may be appropriate. In a retrospective review, the frequency of CSF infection, shunt malfunction, and symptomatic Chiari malformation was similar with simultaneous and sequential repair and shunting [40]. The rate of wound leak was lower and hospital length of stay was shorter in the simultaneous group.

Orthopedic problems — Orthopedic management should be directed at correcting deformities, maintaining posture, and promoting ambulation if possible, so that patients can function at their maximum capability. Factors that predict an increased likelihood of walking ability are motor level and sitting balance [41].

Orthopedic deformities result from congenital skeletal anomalies that often involve the feet, knees, hips, and spine; unbalanced muscle action around joints; and fractures, which often affect the legs of paraplegic patients. In a review from Spain of 393 infants with myelodysplasia, hip dislocation and feet deformities occurred in 24 and 50 percent, respectively [42]. Scoliosis also is common. Management techniques that often improve function include the use of casting and corrective appliances, surgical procedures on soft tissue and bone, and the use of orthoses.

Fractures — Fractures of the lower extremities occur in approximately 30 percent of patients with meningomyelocoele [43]. They may develop without known traumatic injury or may be related to vigorous physical therapy. Factors that increase the risk of fracture include the lack of protective sensation of the leg, osteopenia, nonambulation, foot arthrodesis (fusion of the joint), and higher level of paralysis [43,44].

A fracture should be strongly suspected when a patient with myelodysplasia presents with a red, warm, and swollen limb. These clinical signs are sometimes confused with cellulitis or osteomyelitis because some children with diaphyseal and metaphyseal fractures also have fever, elevated sedimentation rate, and leukocytosis [45], The diagnosis of fracture is confirmed with a radiograph of the limb.

Urinary tract complications — Nearly all patients with spina bifida have bladder dysfunction that can lead to deterioration of the upper urinary tract. The location of the spinal lesion or the neurologic examination do not predict the type of dysfunction. However, urinary continence with intermittent catheterization can be predicted by a positive anocutaneous reflex, which indicates a competent sphincter mechanism. In one report, continence was achieved in 26 of 29 patients (90 percent) with a positive reflex compared to 41 of 82 (50 percent) with a negative reflex [46]. Fewer patients with a positive reflex needed adjunctive surgery (7 versus 28 percent).

A baseline renal ultrasound and voiding cystourethrogram should be performed to identify patients at risk for upper tract deterioration. Function of the neurogenic bladder should be evaluated in affected newborns with a cystometrogram, which measures bladder capacity, compliance, voiding pressures, and the relationship between the detrusor and the urinary sphincter [47]. Vesicoureteral reflux may result from detrusor hyperreflexia or detrusor sphincter dyssynergy. In one report, urodynamic evaluation of 36 infants with myelodysplasia showed incoordination of the detrusor and external urethral sphincter, synergic activity of the sphincter, and no sphincter activity in 18, nine, and nine patients, respectively [48]. Infants with incoordination of the detrusor-external sphincter were at high risk for urinary tract deterioration. Of that group, 13 of 18 (72 percent) developed hydroureteronephrosis, compared to two of nine with synergy and one of nine with no sphincter activity.

Urologic function can deteriorate in affected children with normal urodynamic studies after surgical repair in the neonatal period [49]. Deterioration is due to spinal cord tethering, which is most likely to occur during the first six years of life. These children require close follow-up for the early detection and correction of tethered spinal cord (show figure 2).

Patients with vesicoureteral reflux should receive antibiotic prophylaxis, anticholinergic medication to lower detrusor filling and voiding pressures, and clean intermittent catheterization to prevent urinary tract deterioration [50,51]. The efficacy of this regimen was demonstrated in a sequential nonrandomized study that compared prophylactic (clean intermittent catheterization and oxybutynin) and expectant treatment in patients with these urodynamic findings [50]. During five years of follow-up, the upper urinary tract deteriorated less often in the treated group (8 versus 48 percent).

For an anticholinergic agent, oxybutynin syrup (Ditropan, 1 mg/mL) is used in a dose of 0.1 mg/kg PO three times a day for infants <12 months of age, and 1, 2, 3, or 4 mg/kg per dose three times a day for children one, two, or three years of age, respectively. For children 5 years old, we use oxybutynin tablets (Ditropan, 5 mg PO three times a day), or the extended release preparation (Ditropan XL, beginning with 5 mg PO daily and titrated to effect, with maximum dose 20 mg daily). An alternative drug is tolterodine (Detrol) in a dose of 1 to 2 mg PO twice a day or the long-acting preparation (Detrol LA), 2 to 4 mg PO daily.

Several surgical procedures are used to manage neurogenic bladder in patients with meningomyelocele. Ureteral reimplantation is sometimes performed in patients with persistent reflux and upper tract deterioration or with recurrent urinary tract infections in spite of clean intermittent catheterization and prophylactic antibiotics [52]. A vesicostomy is performed for bladder drainage in infants with high bladder pressure who continue to worsen while receiving clean intermittent catheterization and anticholinergic medication [53]. Vesicostomy is usually used for temporary diversion, but is a long-term option in patients unlikely to achieve continence [53,54].

The most common surgical approach is augmentation of the bladder [55]. In this procedure, a detubularized segment of intestine (ileum, colon, or stomach) is added to the bladder to increase capacity and lower pressure. The procedure usually results in the achievement of urinary continence. Linear growth and bone density are comparable in children with myelomeningocele with or without the procedure, although serum bicarbonate levels are lower and chloride levels are higher in those who have ileal, but not gastric augmentation [56]. Other complications include bladder calculi, bladder rupture, and excessive mucus in the urine that may lead to catheter obstruction [52].

Patients who are unable to catheterize their own urethra may benefit from a continent catheterizable channel (such as a Mitrofanoff or Monti ileovesicostomy). The new channel is constructed from appendix or bowel with a stoma placed at the level of the umbilicus or on the lower abdomen [57,58]. This more accessible location reduces the time required for clean intermittent catheterization, especially in females with lesions at the thoracic level. The most common complication is stenosis of the stoma at the level of the skin which may require dilation or surgical revision.

A surgical technique to bypass the neurologic defect through the microanastomosis of the fifth lumbar ventral root to the third sacral ventral root has been described [59]. Among 20 children with myelomeningocele who had this procedure, 17 achieved satisfactory bladder control and continence within 8 to 12 months after the procedure. These results await confirmation by other centers.

Neurogenic bowel — The innervation for internal and external sphincter control is at the level of S2 to S5. Thus, patients with meningomyelocoele may experience varying degrees of fecal incontinence. As children become preschool or school aged, fecal incontinence leads to embarrassment and social isolation and should be avoided.