Roy D. Strand

Neurological Complications of Hemolytic-Uremic Syndrome

Of 78 children identified with hemolytic-uremic syndrome at the Childrens Hospital, Boston, from 1976 to 1986, 16 patients (20.5%) had neurological manifestations during their hospitalization. The most common manifestations were significant alterations in consciousness (coma, stupor) in 12 patients, and either generalized or partial seizures in ten patients. Others included hemiplegia (4 patients), decerebrate posturing (3), cortical blindness (2), hallucinations (1), and dystonic posturing (1). Cranial computed tomographic scans were abnormal in eight of 11 patients scanned. The abnormalities included diffuse cerebral edema (4 patients), large vessel infarctions (3), diffuse multiple small infarcts (4), and multiple hemorrhages (1). Five patients died as a result of their central nervous system complications, and six had neurological sequelae at discharge. Five patients recovered and at discharge had no evidence of neurological dysfunction. (J Child Neurol 1989;4:108-113).

Computed tomography of Sturge-Weber syndrome in infants.

Computed tomographic findings in two infants with Sturge-Weber syndrome include calcification of the brain not visible on plain radiographs. In both cases, the choroid plexus was very prominent, and, in one, a hemangioma of the choroid plexus was found at surgery. In the other, especially dense deposits of mineral were identified at surgery to be calcified branches of the middle cerebral artery.

TRAUMATIC PARTURITIONAL INTRACRANIAL HEMORRHAGE

Twenty‐five cases of intracranial hemorrhage due to injury at birth, encountered over a seven‐year period, are presented. CT scans showed many different pathways of spread of tentorial hemorrhage; the likelihood of an arterial source is emphasized. Recurrent or delayed bleeding was documented twice. Surgical evacuation of hematomas was necessary in 10 cases, seven involving hemorrhage into the posterior fossa. There were three deaths, one due to pulmonary hemorrhage complicating subtentorial subdural hematoma, another due to severe central parenchymal hemorrhage and a third because of postoperative cerebellar hemorrhage due to disseminated intravascular coagulation. Three survivors are dependent on shunts, two because of the birth injury. The outcome for neurological and intellectual function depends more on associated asphyxia than on the trauma.

Surgical division of craniopagi

The worlds surgical experience on the division of craniopagi is reviewed, and a new case is reported. The problems, including ethical considerations, that must be addressed in selecting patients for operation and in preparing for the procedure are discussed in detail. Hemodynamic stability and the problem of intraoperative gravitational distortion of the brain are addressed. The importance of careful planning is stressed. Survival of the 31 craniopagi after operation is discussed in relation to types of junction.

Metrizamide ventriculography and computed tomography in lesions about the third ventricle.

Metrizamide ventriculography was used in 24 patients with suspected lesions in and near the third ventricle. The diagnoses were aqueduct stenosis (6), upper brain stem expansions (5), third ventricular tumors (6), craniopharyngiomas (2), subarachnoid cyst (1) and normal (1). Computed tomography was used as the guide to predict the likely causes of obstruction and to determine if ventricular shunting was appropriate before or after ventriculography. Several cases required pneumoencephalography in combination with ventriculography in order to determine whether an upper brain stem lesion was intrinsic or extrinsic.

Evaluation of craniofacial surgery in the treatment of facial deformities.

Surgical access to the cranial. orbital, and facial areas, as developed by Tessier, has produced not only definitive repair of previously uncorrectable congenital deformities such as orbital hypertelorism and facial stenosis (e.g., Crouzons, Aperts syndromes) but also has improved markedly the treatment of traumatic and neoplastic defects. The surgical approach allows complete dissection of facial soft tissues including the orbits from the underlying bones followed by corrective osteotomies and fixation. Mobilization of the frontal lobes through a frontal bone flap exposure may be required. The ramifications of this latest intrusion by surgeons into a previously inviolate anatomic area have involved neurosurgeons, ophthalmologists, anesthesiologists, and dental and psycho-social disciplines. The disciplines of genetics and embryology are being influenced by this new field of surgery, much as the study of immunology was influenced by transplantation surgery two decades ago. This report analyzes a 10 year experience with over 100 patients with emphasis on patient selection by disease, age, intellectual status, morbidity, complications, and the psycho-social reactions of patient and family. Procedures initially planned to correct dental and aesthetic defects are proving beneficial for other functions including hearing, taste and smell, articulation and tongue movement, respiratory function, vision, and possibly bone growth. The development of self image, a normal process always in operation, is also strikingly altered. These operations may last as long as 14 to 16 hours. We have had no deaths or postoperative blindness. One postoperative cerebrospinal fluid leak was successfully repaired. Three partial losses of bone grafts and four instances of late cellulitis have occurred. Prevention of infection seems related to avoidance of dead spaces and primary closure of all mucosal, dural, conjunctival and skin surfaces.

Reconstructive Surgery for Major Craniofacial Deformities

Repair of the congenital deformity does not necessarily solve the child’s or the parent’s multiple problems since associated anomalies and side effects may remain and cloud the patient’s future. The study of congenital abnormalities may provide clues to etiology and prevention.

Cerebrospinal fluid rhinorrhea via the fossa of rosenmuller

Traumatic cerebrospinal fluid fistulas in children are not rare as described by Harwood‐Nash1 and Caldicott, North, and Simpson.2 Those involving the sphenoid bone apparently are rare, particularly in early childhood.

Evaluation of cerebral ventricles by computed tomography in the first year of life.

Ninety-seven normal cranial computed tomography studies in the first year of life have been reviewed. The lateral, third, and fourth ventricles were measured, and their size and variations are discussed.

Arteriography in Children Using an Isocentric Parallelogram

A modified U-am with parallelographic motion is readily usuable for serialographic neurological, abdominal, and peripheral angiographic studies in infants and children. Clinical experience with the apparatus has demonstrated dependable and mechanically simple operation, and confirmed that undistorted rotated and sagittally angled views can be obtained without the need for repositioning sedated pediatric patients.