Object The goal of this paper is to elucidate the clinical presentation of tethered cord syndrome (TCS) following repair of a myelomeningocele. Methods Approximately 10 to 30% of children will develop TCS following repair of a myelomeningocele. Because essentially all children with repaired myelomeningocele will have a tethered spinal cord, as demonstrated on MR imaging, the diagnosis of TCS is made based on clinical criteria. The six common clinical presentations of TCS are increased weakness (55%), worsening gait (54%), scoliosis (51%), pain (32%), orthopedic deformity (11%), and urological dysfunction (6%). The primary goal of surgery is to detach the spinal cord where it is adherent to the thecal sac, relieving the stretch on the terminal portion of the cord. Conclusions Early diagnosis and surgical release of the tethered cord results in stabilization or improvement in most cases. Because TCS may present with orthopedic and/or urological signs or symptoms, children with myelomeningocele should by followed, ideally in a multidisciplinary clinic, by neurosurgeons, orthopedic surgeons, and urologists who are aware of this condition.

Intraventricular hemorrhage (IVH) and subsequent posthemorrhagic hydrocephalus (PHH) commonly complicate the course of extremely preterm infants. Many methods for treating the hydrocephalus have been used, none of which are ideal. We present the largest series of infants with PHH treated with one modality, the ventricular access device (VAD). One hundred and forty-nine preterm infants with PHH were treated by placement of a VAD and serial taps to control intracranial pressure and ventricular size. Variables recorded include gender, race, gestational age, weight at birth, IVH grade, incidence of VAD infection, malfunction or local wound problems and indwelling time to either shunt placement or VAD removal. Of the 149 preterm infants, 91 were males and 58 females. The average birth weight was 994 g and the average gestational age at birth was 26.3 weeks. Three infants were IVH grade 1, 8 were grade 2, 62 were grade 3 and 76 were grade 4. VAD occlusion occurred in 15 infants (10%). Nine infants required contralateral VAD placement for a trapped ventricle. VAD infection occurred in 12 infants (8%), 5 of whom were treated successfully with a combination of systemic and intra-VAD antibiotics without removal of the VAD. The total rate of revision was thus 20% (15 for occlusion, 9 for trapped ventricle, 7 for infection). Wound problems were minimal and consisted of 4 cerebrospinal fluid leaks and 14 subgaleal fluid collections. For the 133 survivors, the rate of shunt placement was 88%. The VAD, while not ideal, is an excellent treatment at this time for PHH. It can be utilized for several months with acceptable rates of infection, blockage and wound complications. The VAD tap is simple to perform, not disruptive to minimal stimulation protocols, and can be done by physician extenders. In addition, medications can be administered via the access device, thus allowing treatment of some infections without VAD removal as well as instillation of thrombolytic agents such as urokinase.

Focal cortical dysplasia (FCD) is a congenital disorder of neuronal migration that is increasingly recognized as a common cause of seizures in children, occurring in 20–30% of all surgically treated cases of epilepsy in the pediatric population. Advances in neuroimaging have contributed to recognition of FCD. We report 15 children (9 female, 6 male) with FCD and surgically treated intractable epilepsy. In 9 cases, a surgical strategy of anatomic (frameless stereotactic) grid placement and physiologic (electrocorticography) resection was employed. Postoperative MRI scans were obtained, the pathologic specimen was graded according to the Brannstrom system, and seizure outcome was defined using the Engel classification. There were no deaths and no permanent morbidity. After, on average, 4 years since treatment, 10 children are seizure free, 2 are 2A, 2 are 2B and 1 is 3A. Predictors of good outcome are an MRI-defined lesion and increased cortical disorganization (higher Brannstrom grade). Subtotal resection did not preclude a seizure-free outcome.

Despite improvements in the care of preterm infants, intraventricular hemorrhage (IVH) and posthemorrhagic hydrocephalus (PHH) continue to be frequent occurrences in this patient population. Shunt procedures in these children are frequently complicated by obstruction and/or infection. As the hydrocephalus is usually caused by an obliterative arachnoiditis due to contact of the blood with the basilar meninges, it was postulated that infusion of urokinase into the ventricles of infants who have sustained an IVH would clear the blood, mitigate the arachnoiditis, and prevent the progression of PHH. Accordingly, 18 preterm infants who had sustained IVH and subsequently developed PHH were treated with intraventricular urokinase instilled via a surgically implanted subcutaneous reservoir. There were no complications associated with the urokinase. Infants were divided into two dosage groups: low dose (110,000–140,000 IU total) and high dose (280,000 IU total). One infant in the low-dose group died at 1 month of life of respiratory complications. In the low-dose group, 3 of 8 (37%) infants required shunt placement; in the high-dose group, all 9 required shunt placement. For the total group, the shunt rate was 71 %. This compares to a historical control group shunt rate of 92%. While the difference between the treatment group as a whole and control group approaches, but does not reach, statistical significance (p = 0.068), there was a significant reduction in the shunt rate when the low-dose group was considered separately (p < 0.002). For those infants that required shunt placement, there were fewer shunt revisions performed in the treatment group than in the control group during the first 24 months following shunt placement: 0.67 versus 1.5 shunt revisions/shunted child. Initial experience with intraventricular urokinase following IVH and PHH in preterm infants suggests a beneficial effect in reducing the shunt revision rate in both high- and low-dose groups. Reduction in shunt placement rate is seen only in the low-dose group.

Thrombus is a frequent cause of shunt malfunction both of the proximal end following intraventricular hemorrhage and of the distal catheter of a vascular shunt. Continued blockages may result in numerous shunt revisions until the blood has been cleared. We have treated 3 children with shunt malfunctions secondary to thrombus with urokinase, a thrombolytic agent. Two children had intraventricular hemorrhage following a shunt revision and were treated with intrashunt urokinase, and 1 with occlusion of an atrial catheter was treated with both intrashunt and systemic urokinase. All were symptomatic at the time of treatment (headaches, vomiting, full fontanel, somnolence) and all had ventriculomegaly demonstrated on computed tomography. Various dosage regimens were used with total intrashunt doses of 20,000, 50,000, and 70,000 IU. All improved clinically, computed tomography scans demonstrated improvement, and all were discharged from the hospital. There were no complications of the urokinase administration. The 2 children with proximal occlusion have not required further shunt revisions at 12 and 27 months following treatment. The infant with atrial end occlusion subsequently underwent two proximal revisions with eventual removal of the atrial catheter because of infection. We conclude the intrashunt urokinase can be of value in the treatment of shunts by blood and blood products.