Adolescents with myelodysplasia: impact of physical disability on emotional maturation.

Certain physical, psychological and social characteristics of 20 adolescents with myelodysplasia are compared to those of age and gender-matched controls. In addition to the obvious physical differences the areas of greatest concern are self-esteem and social-sexual adjustment. Family relations, feelings, and modes of expression were not different in the two groups. Lack of appropriate chores, decreased opportunities to interact and compete with peers, plus uncertainties to interact and compete with peers, plus uncertainties about bowel and bladder continence appear to be the greatest impediments to emotional growth in this physically handicapped group of teenagers. Early recognition of such problems and finding strategies to overcome them are important aspects of the comprehensive care of any person with a chronic disability.

Combined pressure-radionuclide evaluation of suspected cerebrospinal fluid shunt malfunction: a seven-year clinical experience.

A simple, safe radionuclide technique for evaluating the functional status of a cerebrospinal fluid shunt has been used in the clinical management of hydrocephalus in 306 patients over a seven-year period. Results of 526 studies showed greater than 99% sensitivity and accuracy in diagnosing distal shunt obstruction and 96% correct correlation with clinical outcome overall. Intrareservoir pressure measurements are especially valuable to diagnose proximal catheter shunt malfunction and to assess shunt dependency.

Fetal puncture during midtrimester amniocentesis.

Four cases of fetal puncture during midtrimester amniocentesis are reported; in one instance the baby apparently sustained temporary neurologic damage. None of the lesions was detected at the initial neonatal examination. From our data and a review of the literature, we estimate that fetal puncture occurs in 1-3% of midtrimester amniotic taps. The implications of the accident are not clear. We conclude that midtrimester amniocentesis should not be considered a routine benigh procedure; and whenever the use of this diagnostic modality is being considered, the prospective parents should be informed of its hazards.

Psychological adjustment of adolescents with myelodysplasia.

The psychosocial development of 20 adolescents with congenital paralysis due to myelodysplasia is compared to 20 age- and gender-matched subjects with no physical handicap. On many of the measures the myelodysplasia group showed poorer adjustment and lower self-esteem than the controls. Consistent with hypotheses regarding adjustment during adolescence the paralyzed males did show greater concern on Offer's scales of Body and Self-Image and External Mastery and showed poorer adjustment on his Sexual Attitudes Scale. The paralyzed girls, particularly those 13 years or younger, showed the poorest emotional adjustment with a significant variability in responses on many of the tests. This degree of variability may indicate a lack of integration of self-concepts and is consistent with an emotional immaturity discordant with advanced physiologic maturation of these girls.

Rose bengal sodium I 131 studies in infants with suspected biliary atresia.

Twenty-eight infants suspected of having biliary atresia were studied with radionuclide imaging and quantitation of liver activity clearance using rose bengal sodium I 131. Scanning the abdomen for the presence or absence of gut radioactivity was a sensitive indicator of complete biliary obstruction. Absence of gut radioactivity correctly predicted biliary atresia in all 11 infants proved to have that diagnosis. Biliary atresia was excluded by the presence of gut radioactivity in 13 of 17 infants with other forms of neonatal juandice. When present, gut activity was identifiable within 24 hours after injection. Serial tests improved specificity. Plotting the rate of clearance of liver activity did not aid diagnosis.

Experience with 1201 cerebrospinal fluid shunt procedures.

This report of our experience with 1,201 cerebrospinal fluid shunt procedures over a 17-year period includes two time periods: from 1965 to 1974 and from 1975 to 1982. The overall operative infection rate fell from 10.7% in the former period to 3.6% in the latter period. The overall average number of revisions fell from 1.8 to 0.77. The number of revisions for patients with infected shunts in the latter period was 4.07. Three hundred and eighty-four shunts were studied in the latter period for the effects of prophylactic antibiotics. No significant difference was found between the infection rate of 5.6% in the nonantibiotic group and the infection rate of 2.1% in the group receiving intravenous and intraventricular methicillin and gentamicin. Differences in the operative infection rates for patients with and without meningomyelocele and those operated on by different surgeons were not significant. The only mortalities were from nonoperative and noncerebrospinal fluid shunt causes.

A longitudinal study of shunt function in 360 patients with hydrocephalus.

Data from an eight-year clinical study of shunt-treated hydrocephalus indicated continued shunt dependency in 289 of 360 patients. 919 combined pressure-radionuclide clearance studies were performed. In 307 studies in which the shunt was identified as nonfunctional, 208 patients were symptomatic for increased intracranial pressure and had the shunt revised. Of the other 99 patients, 71 have remained asymptomatic for three to 60 months (mean 27 months) and 28 subsequently required shunt revision after one to 67 months (mean 24 months). Five other clinical patterns are described. Various methods of monitoring shunt function are recommended.

Serum atropine concentrations after inhalation of atropine sulfate.

Six male subjects with chronic bronchitis were given a single aerosol dose of atropine sulfate (0.05 mg/kg). Spirometry and venous blood samples were obtained before and at 0.25, 0.5, 1.0, 1.5, 2.0, and 4.0 h after inhalation of drug. All subjects had a satisfactory bronchodilator response and detectable serum concentrations of atropine within 15 min. Measurable serum concentrations persisted for 4 h with apparent continued slow absorption occurring throughout the entire time interval. The maximal concentrations achieved ranged from 1.3 to 5.8 ng/ml in five subjects. A sixth subject achieved much higher concentrations (as high as 21 ng/ml) and experienced systemic side effects. This latter concentration is comparable to those achieved with doses of 1.5 to 2.0 mg of parenteral atropine. Significant systemic absorption may occur after inhalation of atropine sulfate, although the degree of absorption is variable.

Influence of prognosis on decisions regarding the care of newborns with myelodysplasia.

Treatment of newborns with myelodysplasia (meningomyelocele and related disorders) continues to be a controversial subject. We have used a consistent plan of care and have employed the same prognostic criteria over the period from 1965 to 1982 to address the needs of 212 affected newborns. A good prognosis and early surgical care were given to 42 per cent of 53 newborns during the period 1965 to 1970, to 58 per cent of 65 newborns from 1971 to 1976, and to 71 per cent of 94 newborns from 1977 to 1982. Of the newborns with an initially poor prognosis, 19 per cent of 31 received early surgery between 1965 and 1970, as compared with 33 per cent of 27 between 1971 and 1976 and 52 per cent of 27 between 1977 and 1982. Life-table analyses of survival in the three periods revealed significant improvement over time in the survival of newborns receiving early surgical care, regardless of the initial prognosis (log-rank statistic = 8.240, P = 0.016) and in comparison to recipients of supportive care alone (log-rank statistic = 5.975, P = 0.05). We conclude that early surgery permits the survival of an increasing percentage of patients with myelodysplasia.

Nasal dermoid sinus cysts: association with intracranial extension and multiple malformations.

Nasal dermoid and sinus cysts (NDSC) are uncommon congenital anomalies that may have intracranial extension and can be associated with other anomalies. We identified 22 patients in a retrospective review of cases diagnosed with NDSC at our institution over the past 10 years. Nine (41 percent) had associated anomalies and ten (45 percent) had intracranial extension of the sinus. In half of the patients with intracranial extension, the sinus transversed either the cribriform plate or foramen cecum and attached to the dura; in the other half, the sinus extended to cysts within the falx or other brain structures. Of the patients with multiple anomalies, six (67 percent) had intracranial extension. Presurgical complications occurred in a total of eight patients (36 percent): two had meningitis, two had osteomyelitis, four had periorbital-nasal cellulitis, three had nasal abscess, and four had nose anomalies requiring rhinoplasty.

Myelodysplasia. Problems of long-term survival and social function.

Problems of ninety-eight patients with myelodysplasia, ages 13 to 72, were reviewed. They were grouped as follows: Those having thoracic and high lumbar level (L(2) upward arrow) lesions and confined to wheel chairs, those with intermediate paralysis (L(3-5) nerve roots) as walking with aids and those with less paralysis (S(1) downward arrow) as fully ambulatory. Fifty-two percent of the L(2) upward arrow and only 15 percent of of the less severely paralyzed patients were retarded below an IQ level 70 (P<0.01). Thirty-six patients (62 percent) were fully and 26 partially, but appropriately, self-sufficient. Thirty-six patients were found in some form of dependent care. Two of the 71 more paralyzed patients (L(3-5) and L(2) upward arrow) and five of the 28 S(1) downward arrow patients were "naturally continent" but reported stress incontinence of urine. Thirteen of 23 female and five of 28 male patients between ages 16 and 72 years reported sexual activity and accounted for 17 normal offspring. All 23 retarded patients were in some form of custodial care. Dependency among the normal intellect patients could be attributed to neglect of physically deforming complications and emotional disorders, primarily low self-esteem centering around social and sexual identity problems associated with excrement soiling.