William R. Cheek

Two Siblings with the Chiari I Malformation

Two siblings with symptoms characteristic of the Chiari I malformation are described. The malformations were confirmed by magnetic resonance imaging scans and at surgery. Upon review of the world literature, no instance of the Chiari I malformation in a single family has been reported.

Successful conservative management of cerebellar hematomas in term neonates

appeared in the program of The Child Neurology Society, October, 1980, Savannah, Georgia. *Reprint address: Department of Pediatrics, Baylor College of Medicine, 1200 Moursund Ave., Houston, TX 77030. tomas who were treated surgically and survived. RaveneF reviewed the literature and added two additional cases, both of which were treated successfully by surgical drainage o f the hematoma. In the 21 cases diagnosed during Abbreviations used CT: computerized tomography TCH: Texas Childrens Hospital life, only one patient did not undergo operative procedure, and that infant died; the survival rate was 85% in the surgically treated patients. Thus, early surgical intervention was suggested as the most efficacious therapy for this problem. This paper documents the spontaneous resoluVoL 98, No. 3, pp. 466-468 0022-3476/81/030466+03500.30/0 9 1981 The C. V. Mosby Co. Volume 98 Brief clinical and laboratory observations 4 6 7 Number 3 Fig, 1. A, Patient 1. CT scan performed on second day of life demonstrating hemorrhage in the cerebellum with blood extending into the third and lateral ventricles and modest hydrocephalus. B, CT performed the third day of life demonstrating a decrease in ventricular size. t ion o f in t race rebe l l a r h e m a t o m a s and assoc ia ted hydrocepha lus in two t e rm infants w h o s e s u b s e q u e n t deve lopm e n t has b e e n sat isfactory. C A S E R E P O R T S Patient 1. A 2.8 kg male infant was born to a gravida 6, para 4, abortus 1 mother after an uncomplicated 38-week gestation. The baby was delivered from a frank breech presentation and the amniotic fluid was meconium stained. The Apgar scores were 5 and 8 at one and five minutes, respectively. Resuscitation via facemask and bag was utilized. Pallor was noted approximately 35 minutes after birth when the baby had a seizure characterized by general stiffening of the trunk and extremities with the eyes staring straight ahead. Thereafter, he developed intermittent opisthotonic posturing, nystagmus, and bradycardia. The cerebrospinal fluid was bloody and the infant was transferred to Texas Childrens Hospital at 16 hours of age. Upon arrival head circumference was 33.5 cm, the anterior fontanelle was full, the baby moved all extremities spontaneously and withdrew appropriately to painful stimuli. He cried only after a prolonged stimulLls. Eye movements were full but vertical nystagmus was noted. The sucking reflex was poor, as was the Moro reflex. The deep tendon reflexes were increased and bilateral Babinski signs were present. A CT scan done on the second day of life demonstrated an intracerebellar hematoma with extension into tl~e third and lateral ventricles (Fig. 1, A); hydrocephalus was present. Intermittent posturing, bradycardia, apnea, and bicycling movements of the lower extremities were noted. His condition was stabilized and he was given a blood transfusion (because of a hematocrit of 24) in preparation for surgery. Because his neurologic function did not deteriorate and his fontanelle seemed less tense, a repeat CT scan was done on the third day of life (Fig. 1, B). This demonstrated improvement in the degree of hydrocephalus, and conservative management was continued. The course was one of steady improvement and the infant was discharged on the tenth day of life. A CT scan done prior to discharge revealed further resolution of the hematoma and continuing decrease in ventricular size. When examined at 2 months of age his head circumference was continuing to grow along the fiftieth percentile. His development was progressing at a normal rate and his neurologic examination disclosed only slight increased tone and hyperreflexia. At 8 months of age his development continued to progress satisfactorily and a repeat CT scan revealed minimal enlargement of the fourth ventricle and subarachnoid spaces, and an area of low density within the cerebellum. Patient 2. A 3 kg male infant was admitted to TCH at 36 hours of age because of apnea, bradycardia, and opisthotonic posturing beginning at 24 hours of age. He was born to a gravida 2, para 1 mother after an uncomplicated 37-week gestation. The delivery was somewhat difficult because of a footling breech presentation. The Apgar scores were 8 and 10 at one and five minutes, respectively. On admission he was noted to move all extremities spontaneously; he had brisk deep tendon reflexes but poor sucking and grasp reflexes. The anterior fontanelle was soft. The hemoglobin was 17 gm/dl and the cerebrospinal fluid was grossly bloody. A C T scan done at 2 days of age showed an intracerebellar hematoma with a slight increase in ventricular size (Fig. 2). He was treated conservatively, and the intermittent episodes of bradycardia with or without apnea became less frequent and ceased by 7 days of age. Serial CT examinations showed resolution of the hematoma and a decrease in ventricular size. A repeat examination at 7 weeks of age demonstrated complete resolution of the hematoma and a normal ventricular system. When evaluated at 5 months of age his development was thought 4 6 8 Brief clinical and laboratory observations The Journal of Pediatrics March 1981 Fig. 2. CT scan pe~rformed on the second day of life demonstrating a cerebellar hematoma and mild ventricular dilatation. to be minimally delayed. His examination disclosed the head circumference to be in the fiftieth percentile, and his neurologic examination was normal except for slight generalized hypotonia, strabismus, and titubation of the trunk and head in the sitting position.

Cerebellar hemorrhage in the term neonate: Developmental and neurologic outcome

To elucidate the effects of cerebellar hemorrhage on the term neonate, neurodevelopmental assessments were conducted at a mean age of 32 months on six children. In addition to cerebellar hemorrhage, ventriculomegaly was present on each subjects initial computed tomographic scan. All were managed without surgical evacuation. Two patients required shunts for progressive ventriculomegaly. Five patients had follow-up computed tomography indicating mild atrophy of the superior anterior vermis of the cerebellum; however, none had abnormal ventricular size or abnormalities of the cerebrum. On detailed examination conducted between the ages of 18 and 48 months, five had hypotonia, truncal ataxia, and intention tremor; two had nystagmus. Only one patient walked independently. Intellectual performance of four patients was within the retarded range and two had mildly delayed development. Two patients had markedly disordered expressive language. These data suggest that term neonates surviving cerebellar hemorrhage have neurologic deficits related to the site of hemorrhage, and cognitive deficits related to more generalized cerebral insult.

Tethered-cord syndrome after repair of meningomyelocele

The occurrence of tethered-cord syndrome is one of the delayed consequences of the repair of meningomyclocele. The existing neurological deficit worsens, or a new deficit is superimposed on the existing one. In addition, urological and orthopedic symptoms are also frequently encountered. Although radiological studies may be suggestive of tethering of the cord, not all children are symptomatic. Magnetic resonance imaging is the best radiologic study available. Ultrasonography, although economical and easy to perform, does not yield an optimal image. It appears that a careful periodic clinical evaluation is the best way to evaluate the patients for surgery.

Effects of Ventricular Drainage and Dural Closure on Cerebrospinal Fluid Leaks after Posterior Fossa Tumor Surgery

In a retrospective study of 50 consecutive children with posterior fossa tumors treated at Texas Childrens Hospital, Houston, Tex., in 1989-1992, we evaluated perioperative factors which might influence the development of postoperative cerebrospinal fluid leaks. Factors analyzed included the presence of preoperative hydrocephalus, the institution of cerebrospinal fluid diversion, and the method of dural closure. No statistically significant impact on subsequent cerebrospinal fluid leakage was demonstrated.

Neurogenic bladder and the lumbar spine

Abstract From a large number of patients with urologic symptoms, 8 were selected in six years as having “true neurogenic bladder” with chronic and mild symptoms in the lumbar spine. Symptoms and neurologic and radiologic findings in the preoperative period are described. The type of urologic evaluation is briefly presented with characteristic examples. The postoperative results of these and other series are presented and questioned because of the lack of improvement on urodynamic studies in patients deemed better clinically. The need for a complete evaluation with more sophisticated urologic tests before and after surgery is stressed before a final decision regarding the relation between neurogenic bladder and “asymptomatic” cauda equina compression can be reached.

Ventriculoatrial shunting via the azygos vein

The treatment of hydrocephalus has evolved through many stages but the “cure” is still elusive. It is not unusual for the neurosurgeon to find that the commonly used routes for catheter placement or sites for drainage of cerebrospinal fluid (CSF) cannot be employed. The azygos vein was used to gain access to the right atrium when the CSF could not be drained into the peritoneal cavity, nor could the neck veins be used to place the catheter into the right atrium. The azygos vein is a convenient and safe route to reach the right atrium in selected patients.

A new staging method versus TNM staging in children with posterior fossa primitive neuroectodermal tumor (medulloblastoma)

Prognostic factors were evaluated in 30 children with primitive neuroectodermal tumors of the posterior fossa (classical medulloblastoma or differentiated medulloblastoma). The preoperative TNM (Tumor, Node, Metastasis) staging system has been applied to estimate prognosis of patients with central nervous system tumors, but this system was not applicable in assessing the post-operative status of the tumor. To fill this need, a post-operative staging system was developed known as MAPS (Metastasis, Age, Pathology, Surgery), and in this report its efficacy is compared with that of the TNM system. The predictive values of MAPS staging in these 30 children were better than those obtained using the TNM method (MAPS r2=−0.90; TNM r2=−0.73; P<0.05).

Device for extradural monitoring of intracranial pressure: technical note.

An ICP-monitoring device utilizing an extradural method is described. Clinical and laboratory use has shown it to be easily installed, safe, accurate, and reliable. It can be used with currently available transducers and monitoring equipment. It is hoped that this simple device will encourage the increased use of ICP monitoring.

Survival of split calvarial bone grafts in a dog model

To gain a better under-standing of the strength and long term viability of split calvarial bone graft, nine mongrel dogs each received four trephine craniotomies (1.5 mm diameter). The harvested bone plugs were split through the diploë and a partial-thickness plug was returned to each craniotomy site. The unused partial-thickness plugs underwent strength testing. After 90–150 days the dogs were sacrificed, bone scans and X-rays of the graft recipient sites performed, and the grafted plugs harvested for strength testing and histologic examination. Bone scanning showed increased osteoblastic activity within the grafts, strength testing showed a progressive loss in strength over the 150 days, and histologic examination demonstrated a creeping substitution. These results imply a similar evolution in strength and remodeling to that seen in long-bone grafts, whose remodeling typically spans 1 year.