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Biomedical subjects

L Schut

Publications and source records attributed to L Schut.

At least 145 records · Page 8Linked to original sources
PubMed1977

Early surgery for isolated craniofacial dysostosis. Improvement and possible prevention of increasing deformity.

We describe our experiences with craniofacial surgery at less than 9 months of age in 8 patients with isolated craniofacial dysostosis. We believe that the early release (preferably before the age of 6 months) of the constricting forces in bound or underdeveloped areas of bone will allow subsequent better development of these faces. The molding and expansile influences of the rapidly developing brain and eyes can exert a greater effect on facial growth. The operations are easier and more rapidly performed than those in older children, and no distant bone grafts are necessary.

Age Factors↗
PubMed1977

Nasal glioma.

Six cases of nasal gliomas, which are rare ectopic rests of neural tissue found at the root of the nose, are presented. It is important to distinguish nasal tumors from basofrontal encephaloceles to avoid inadvertent exposure of the brain during the surgical removal of mass lesions. Because of their related embryologic origins, the distinction between nasal gliomas and basofrontal encephaloceles may not be clear clinically. Nasal gliomas may be treated by several surgical specialties, and only a proper awareness of their relatonship to encephaloceles can assure the selection of a flexible and adequate surgical approach. This paper emphasizes the salient clinical characteristics of nasal gliomas, their clinical distinction form and embryologic relationship to encephaloceles, and the options for treatment.

Diagnosis, Differential↗
PubMed1976

Midline hamartomas masquerading as meningomyeloceles or teratomas in the newborn infant.

Congenital miline dorsal malformations in infants most commonly represent spina bifida cystica or teratoma. The five patients reported here are a distinct clinicopathologic entity. They presented at birth with a dorsal midline mass, with no neurologic impairment, and no hydrocephalus developed subsequently. The lesions were removed in all infants without significant mobidity. The masses were comprised prinicipally of mesodermal elements, i.e., bone, cartilage, fat, muscle, and fibrous tissue. The well-differentiated cellular components, forming mature structures and the lack of primitive organoid structures as well as the absence of neoplastic characteristics, favors a diagnosis of hamartoma rather than of teratoma.

Child, Preschool↗
PubMed1976

A transmissible subacute spongiform encephalopathy in a visitor to the eastern highlands of New Guinea.

A Caucasian male, clinically ill with a respiratory disease, visited the Eastern Highlands of New Guinea (endemic for kuru in the Fore people) and developed subacute spongiform encephalopathy (Jakob-Creutzfeldt disease) ten weeks later, from which he subsequently died. Brain material was inoculated intracranially into squirrel monkeys, and several of them developed a spongiform encephalopathy. Monkeys that received control material (normal brain) were normal. Electronmicroscopic features in affected brain tissue are described, and the question of a relationship between Jakob-Creutzfeldt disease and kuru is considered.

Animals↗
PubMed1976

Craniofacial surgery: present and future.

The possibilities for radical craniofacial restructuring have increased dramatically in the past 6 years with the development of craniofacial surgery. The field developed from a background of patients with major craniofacial birth defects allowing orderly planning and expansion to correction of a multitude of other craniofacial structural problems. The procedures concentrate upon changing the skeletal structures using extensive subperiostial dissection of soft tissue, and adding bone to fill in areas of deficiency. There are three grades of complexity in craniofacial procedures. After extensive soft tissue sub-periostial stripping about the orbits and upper face, the simplest form consists of onlay bone grafts. The next most complicated involves osteotomies to shift the face into a more normal position. In its most complicated form, abnormal proportions of bone are removed and the orbits or cranium are shifted into a new or normal position. We have had experience with 69 patients since September, 1972. Thirty-six have had intracranial procedures. Infection has been the most serious problem, and there have been no instances of death or blindness. A number of lesser problems occur. Future applications of craniofacial surgery are appearing with great frequency as more experience is gained with its uses. It has particular application in acute and late reconstruction of patients with traumatic defects about the face. Preventive osteotomies are an area with great potential, by releasing stenotic areas of bone and allowing the developing brain to mold the upper face and orbits. There is also applicability in surgery of tumors about the craniofacial structure and in cosmetic surgery.

Adolescent↗
PubMed1976

Intracranial pressure monitoring in Reye's syndrome.

Reye's syndrome is difined as fatty degeneration of the viscera with encephalopathy. The signs are change in consciousness after recovery of a viral illness, vomiting, high fever, and progresseve deterioration of the level of consciousness. Signs of brain stem involvement quickly follow. The clinical findings are: mild elevations of serum transaminases (SGOT and SGPT), normal to slightly elevated bilirubin, abnormal blood-clotting functions, normal to low blood glucose, high blood ammonia, and normal cerebrospinal fluid which may be under increased pressure. Death is due to increased intracranial pressure. Therapy is used to stabilize these signs. It includes: infusion of hypertonic glucose followed by insulin, peritoneal dialysis, and exchange transfusion. Intracranial pressure was monitored continually to determine when therapy should be administered.

Brain Diseases↗
PubMed1976

Response to vincristine of recurrent brain tumors in children.

Seventeen children with clinical evidence of a recurrent brain tumor were treated with vincristine 1.5 mg/sq m weekly for 12 weeks with doses on alternate weeks thereafter. Eight of the 16 patients who received four or more doses of vincristine showed significant objective responses; these included patients with high-and low-grade astrocytomas and patients with medulloblastoma. Six of the eight who responded remained asymptomatic for more than 2 years. The toxicity encountered was minimal except for seizures possibly related to vincristine in three children, who were able to resume treatment. Vincristine therapy results in long-term regression in a wide variety of pediatric brain tumors and causes little or no toxicity.

Astrocytoma↗
PubMed1976

Fractures and dislocations of the cervical spine in children.

Correct diagnosis of fractures and dislocations of the cervical spine in children requires an awareness of the pseudosubluxation and other normal variants noted in x-ray films of patients in this age group. Fractures of the odontoid process occur frequently and almost always can be treated by closed reduction and external fixation until union occurs. Fractures of the lower cervical spine are difficult to detect roentgenographically, and x-ray changes can belie the severity of soft tissue injury and cord trauma. Instability tends to persist in adolescent patients after cervical spine injury because of the combination of epiphyseal and posterior ligamentous disruption. Neoplastic, inflammatory, and congenital lesions render the cervical spine vulnerable to injury and can permit major damage to result from minor stress.

Adult↗
PubMed1975

Slow tremor and macrocephaly: expanded version of the bobble-head doll syndrome.

The clinical features of three children in whom a slow tremor involving the head, trunk, or limbs was associated with macrocephaly are presented. The findings were similar but not identical to those previously reported in four children with the bobble-head doll syndrome. These seven children have many of the signs and symptoms found in patients with surgically or spontaneously arrested hydrocephalus, including motor incoordination, behavior and psychologic deviations, and endocrine dysfunction. The tremor which can be voluntarily inhibited and which disappears following shunt surgery, is unique. The theoretical basis for the tremor is briefly discussed.

Abnormalities, Multiple↗
PubMed1975

The effects of exchange transfusion on intracranial pressure in patients with Reye syndrome.

Intracranial pressure has been monitored in ten patients with Reye syndrome. All of the patients were comatose and all were treated with dexamethasone, mannitol, hyperventilation, and multiple exchange transfusions. Five of these children are alive and well; five died. In each case ICP monitoring allowed for immediate treatment and evaluation of the mode of therapy used to reduce elevated ICP. Exchange transfusions in all patients either reduced the increased ICP or made the pressure rises easier to manage. ICP is not always reflected by lumbar spinal fluid pressure or clinical state. In our hands, ICP monitoring has proved to be a safe technique for the investigation of the significance of raised ICP in the outcome of patients with Reye syndrome and of the effectiveness of a chosen therapy in reducing this pressure.

Brain Diseases↗
PubMed1975

Selection of early treatment of myelomeningocele: a retrospective analysis of selection procedures.

In an attempt to formulate a procedure for the selection of those children with open myelomeningocele most likely to benefit from early surgery, the records of 163 children have been reviewed. No physical findings, either singly or in combination, were found to distinguish accurately between children with good and poor prognoses, and an alternative selection procedure is proposed which takes into consideration the absence or presence of lacunar skull deformity (a finding strongly associated with mental retardation). Early surgery is not recommended for children with lacunar skull deformity and at least two of Lorber's major adverse neonatal criteria, or children with associated gross congenital anomalies. This selection procedure has been applied retrospectively to the 163 children reviewed and has been shown to predict the (known) outcome of these children much more reliably than existing selection criteria.

Follow-Up Studies↗