Carcinoids of unknown origin: comparative analysis with foregut, midgut, and hindgut carcinoids

The purpose of this study was to analyze our results of surgical treatment of arthrogryposis of the elbow and to compare our tendon transfer results using range of motion (ROM) criteria versus functional use criteria. Eighteen tendon transfers for elbow flexion in 14 children with arthrogryposis with an average follow-up period of 4 years (range, 1-14 years) and 6 elbow capsulotomies with triceps lengthening in 6 children with arthrogryposis with an average follow-up period of 5 years (range, 2-9 years) were evaluated. Each child was assessed by a questionnaire regarding functional use of the upper extremity, physical examination of ROM and strength, and a videotaped activities of daily living evaluation. Tendon transfer results were classified and compared using 2 methods of evaluation: postoperative strength and ROM and effective functional use of the tendon transfer to perform activities of daily living. The 6 elbow capsulotomies improved from an average preoperative arc of 17 degrees of motion (average extension, -2 degrees; average flexion, 19 degrees) to an average final follow-up arc of 67 degrees (average extension, -25 degrees; average flexion, 92 degrees). The 18 tendon transfers evaluated by strength and ROM criteria showed 9 triceps to biceps transfers in 9 arms (7 good, 1 fair, and 1 poor), 5 pectoralis to biceps transfers in 4 arms (1 good, 3 fair, and 1 poor), and 4 latissimus dorsi to biceps transfers in 3 arms (2 good and 2 fair). Evaluation by functional use criteria gave the same result in 13 transfers and downgraded the result in 5; the downgraded results were due to resultant flexion contracture or limited functional use because the transfer was in the nondominant arm. Based on this review, optimal surgical candidates for tendon transfer are children older than 4 years, who have full passive ROM of the elbow in the dominant arm, and at least grade 4 strength of the muscle to be transferred.     

Deformity of the foot following anterior transfer of the posterior tibial tendon and lengthening of the Achilles tendon for spastic equinovarus

Twenty-four children with spastic equinovarus deformity due to cerebral palsy were treated by anterior transfer of the posterior tibial tendon and Achilles tendon lengthening. In five patients, the operation was performed on both sides, making a total of 29 feet available for evaluation after an average follow-up of five years. Only 38 per cent of the results were graded "good" or "satisfactory." Sixty-two per cent were rated as "poor" because of valgus, calcaneus or equinus deformity severe enough to require re-operation. The post-operative deformity was generally evident one or more years after surgery, often progressive, and more disabling as well as more difficult to correct than the original condition. Although the percentage of acceptable results was considerably higher for hemiplegic patients than for others in the study, we conclude that in this group and in all other categories of spastic patients anterior transfer of the posterior tibial tendon should not be performed.     

Potentially asphyxiating conditions and spastic cerebral palsy in infants of normal birth weight

OBJECTIVE: Our purpose was to examine the association of cerebral palsy with conditions that can interrupt oxygen supply to the fetus as a primary pathogenetic event. STUDY DESIGN: A population-based case-control study was performed in four California counties, 1983 through 1985, comparing birth records of 46 children with disabling spastic cerebral palsy without recognized prenatal brain lesions and 378 randomly selected control children weighing > or = 2500 g at birth and surviving to age 3 years. RESULTS: Eight of 46 children with otherwise unexplained spastic cerebral palsy, all eight with quadriplegic cerebral palsy, and 15 of 378 controls had births complicated by tight nuchal cord (odds ratio for quadriplegia 18, 95% confidence interval 6.2 to 48). Other potentially asphyxiating conditions were uncommon and none was associated with spastic diplegia or hemiplegia. Level of care, oxytocin for augmentation of labor, and surgical delivery did not alter the association of potentially asphyxiating conditions with spastic quadriplegia. Intrapartum indicators of fetal stress, including meconium in amniotic fluid and fetal monitoring abnormalities, were common and did not distinguish children with quadriplegia who had potentially asphyxiating conditions from controls with such conditions. CONCLUSION: Potentially asphyxiating conditions, chiefly tight nuchal cord, were associated with an appreciable proportion of unexplained spastic quadriplegia but not with diplegia or hemiplegia. Intrapartum abnormalities were common both in children with cerebral palsy and controls and did not distinguish between them.     

Assessment of upper-extremity function in children with spastic diplegia before and after selective dorsal rhizotomy

The upper-extremity function of 26 children (mean age 6.0 years) with spastic diplegia was assessed prior to and one year following selective dorsal rhizotomy (SDR). No significant change was found in upper extremity range of motion, muscle tone, or strength (MMT). A significant increase was noted in grasp strength bilaterally which could not be explained simply on the basis of maturation. Manipulation patterns were noted to improve significantly one year after SDR; however, this was proposed to be a maturation rather than intervention effect. Standardized test results revealed no significant improvement in functional hand use when the scores of children with spastic diplegia were compared with age-matched norms, to control for the effect of maturation. The significant improvement found in ability to toilet, dress and undress independently was most likely due to the effect of SDR on lower-extremity muscle tone. Further investigation with a matched control group would provide a conclusive result regarding the effect of SDR on upper extremity function and assist in understanding the maturation of hand function in children with spastic diplegia.     

Early high dose antioxidant vitamins do not prevent bronchopulmonary dysplasia in premature baboons exposed to prolonged hyperoxia: a pilot study

The effect of two different types of functional ankle-foot orthosis on the gait of patients with spastic hemiplegia was studied. A gap was cut into the conventional stiff orthosis to allow a limited dorsiflexion of 10-15 degrees, while plantarflexion was blocked (spring type). This gap was bridged by an aluminum bar to stiffen the orthosis for the experiment. Fourteen patients of different ages (6.5-20.1 years) walked barefoot and with the orthosis, once springy and once stiff. Kinetics and kinematics were analyzed. Gait was significantly improved into normal pattern by using any type of ankle-foot orthosis. While walking barefoot with a toe-heel-toe gait, the physiological heel-toe gait was restored with any type of orthosis. The spring type of orthosis was significantly superior to the stiff orthosis. This was most clearly expressed in the general parameters of gait-like cadence, velocity, and step length. Kinetic data gave a significant improvement by using any ankle-foot orthosis. Whereas break force was similar with both types, push-off was further improved with the spring type. Kinematics revealed little statistical difference between the two types, although gait was more dynamic and physiological with the spring type of orthosis. Any functional ankle-foot orthosis ameliorates the gait pattern in patients with spastic hemiplegia, but a spring type of orthosis renders the gait more dynamic and best corrects the pathology of gait.     

Does walking speed influence the time pattern of muscle activation in normal children?

Dynamic electromyography (EMG) of the extrinsic muscles of the ankle is used more and more frequently to assist in the planning of tendon transfers in children with equinovarus deformities. Since these children walk at low speeds (1 to 4km/h), and since walking speed modifies the EMG-time pattern, the clinical investigator must be able to differentiate EMG modifications due to pathology versus those due to slow walking speed. The aim of this work was to study the effect of walking speed on the EMG-time pattern of the extrinsic ankle muscles in healthy children between 4 and 11 years of age. This pattern was found to change significantly with speed of progression but is independent of growth over this age range. A nomograph of EMG timing, taking into account walking speed, is proposed for clinical gait analysis.     

Iliopsoas transfer in cerebral palsy: the long-term outcome

Thirty-three patients with cerebral palsy had iliopsoas transfers as part of their surgery for hip instability. All had total-body involvement, spastic cerebral palsy and none could walk. At the time of surgery, eight hips were subluxated and 39 were dislocated or severely subluxated. Mean follow-up was 8 years in patients between 8-25 years old. Forty-five of the 47 hips were located. Thirty patients had an accompanying scoliosis, and, in 10 patients, the rib cage impinged on the pelvis. Hip flexion had decreased in most patients. Sitting ability had not improved in any patient, and had in fact deteriorated in 50%.     

Three-dimensional gait analysis in spina bifida

This study was designed to determine gait patterns in children with lumbar and sacral neurologic level spina bifida. We studied a group of 28 children: 10 had L4-level lesions and a mean age of 11 years; eight had L5-level lesions and a mean age of 8 years; and 10 had S1-level lesions with a mean age of 12 years. A group of 15 normal children, mean age 10 years, was used for comparison. Each child underwent three-dimensional gait analysis using the Vicon system. We found that there were recognisable gait patterns for each level of spina bifida and that the abnormalities accurately reflected the muscle deficiencies present. The gait patterns approximated more closely to those of the normal group as the neurological level descended. The most important findings were of increased pelvic obliquity and rotation with hip abduction in stance (reflecting the gross Trendelenburg-type gait seen in these children) and persistent knee flexion throughout stance as a result of the absence of the plantar flexion-knee extension couple. We found that gait was not improved by tendon transfers performed either at the hip (posterolateral psoas transfer) or at the ankle (tibialis anterior transfer).     

Selective peripheral neurotomy of the hamstring branches of the sciatic nerve in the treatment of spastic flexion of the knee. Apropos of a series of 11 patients

BACKGROUND: A series of 11 patients (9 adults and 2 children) were operated on by selective peripheral neurotomy for spastic knee in flexion. The objective of surgery was to restore bipodal stance and therefore walking for the adult patients and to improve walking for the diplegic children. METHODS: Spasticity was secondary to stroke or cranial trauma for the adult patients. The two children presented with Little disease. The delay between onset and surgery was on the average of 2 years. All the patients had a passive flexum of the knee of 20 degrees, and an excessive spasticity of the hamstrings (average Held score of 4.55). Peripheral selective neurotomies consist of a partial section of the spastic muscle's motor fascicles. The partial section concerns the afferent fibers to the spinal cord whose interruption leads to the disappearance of spasticity. The section also involves the motoneurones' axons and thus leads to partial denervation. RESULTS: The spasticity of the treated muscles disappeared in the 11 patients. Average follow-up was 16 months. The neurotomies led to improvement of walking without splint for 3 walking patients, and restored walking with a splint for 5 patients. One patient failed to walk. The two children had a dramatic improvement of their walk. CONCLUSION: Our results incite us to increasingly indicate this type of neurotomy in the treatment of the spastic lower limb.     

Home medical equipment suppliers: a critical home care partner. Interview by Sarah F Zarbock

Conventional management of gastroesophageal reflux (GER) and hiatus hernia in children affected with encephalopathy can deteriorate their pulmonary function, already compromised by their leaning position and their spine deformations. The results after laparoscopic surgery for GER in 6 encephalopathy children are reviewed; their ages ranged from 9 to 14 years, their accompanying diseases were: spastic tetraparesia, hydrocephalus, scoliosis, epilepsy and Reye's syndrome. The laparoscopy procedures followed the same surgical steps as open surgery; the surgical time was 3 to 5 hours; the average hospital stay was 3 days. Oral intake started 8 hours after surgery. The successful results of these first cases in this group of patients with neurologic anomalies, suggest that laparoscopic Nissen funduplication is the treatment of choice for GER and hiatus hernia.     

Gait analysis and muscle strength in children with surgically treated clubfeet

Twenty-three children who had unilateral surgery for idiopathic clubfeet underwent gait analysis and isokinetic muscle-strength testing at an average of 10 years after surgical release. Ankle sagittal-plane kinematics were disturbed in 20 clubfeet. Fifteen children had an internal foot-progression angle. Genu valgum and knee hyperextension were common. Plantarflexion power was decreased by 23% on the side of surgery (p = 0.00005). Quadriceps weakness (defined as a decrease in strength >10% of the nonoperated-on limb's strength) was seen in nine of 22 operated-on limbs and hamstring weakness in eight of 22. Gastrocsoleus weakness exceeded 10% in 16 of 21 clubfeet. Average weakness of the gastrocsoleus was 27% (p < 0.05). Ten-year analysis of children treated with clubfoot releases revealed disturbances in ankle motion and strength. Loss of plantarflexion power and gastrocsoleus strength can be predicted by Achilles tendon lengthening. Knee kinematic abnormalities and weakness also were seen. These abnormalities produce functional difficulties in gait and may lead to degenerative changes later.     

Shearing injuries of parasagittal white matter, corpus callosum and basal ganglia: possible radiological evidences of hemiplegia in diffuse axonal injury

The relationship between spastic hemiplegia in diffuse axonal injury (DAI) and neuroradiological findings was studied in 100 cases. These cases were prospectively collected from the files of Automobile Insurance Rating Organization in Japan between 1993 from to 1996. Requirements for entry to this study were as follows: presence of initial unconsciousness after head injury without any lucid interval. Existence of CT scan or MRI film obtained within 12 hours of injury showing no significant mass effects, as well as follow-up CT scan or MRI film obtained more than 3 months after the injury. Psychosocial outcome was described both by the medical professional and the caregiver. The hemiplegia was rated severe, mild, or none. The outcome and diffuse ventriculomegaly were classified as reported by the authors previously. Spastic hemiplegia or quadriplegia was documented in the chronic stage in 63 cases, including 53 severe cases with difficulty in walking and 10 mild cases with only pyramidal signs detected. Chi-square analysis showed significant correlation between hemiplegia and the DAI outcome level or ventriculomegaly rating. Focal brain contusion was noticed in 33 cases, but did not correlate with the hemiplegia at all. Radiological findings included 25 cases of parasagittal white matter injury (gliding contusion), 20 cases of callosal injury, 19 cases of basal ganglionic region injury, 5 cases of brain-stem injury, and 3 cases of cerebellar injury. Chi-square analyses of hemiplegia and contralateral presence of these injuries were significant in the former three types of injury. Presence of at least one of these 3 lesions was defined as GCB injury. There were altogether 46 GCB injury cases which were significantly correlated with contralateral hemiplegia by chi-square analysis and by Spearman rank analysis. Partial correlation analysis with hemiplegia as the target variable indicated highly significant correlation only with GCB injury and outcome level. In conclusion, spastic hemiplegia in DAI is a manifestation of primary shear injury. Neuroradiological findings of GCB injury were statistically able to be significantly correlated with the presence of hemiplegia, and suggested pyramidal tract injury either at the corona radiata or the internal capsule level.     

American Cancer Society perspective on the American College of Preventive Medicine''s policy statements on skin cancer prevention and screening

Six surgical procedures, consisting of tendon transfers and releases, were performed in five patients with idiopathic focal dystonia involving the lower extremity. All patients were female. Surgical management was performed to correct clinically significant foot abnormalities. The goal of each procedure was functional improvement and obtaining a plantigrade foot. The SPLATT (split anterior tibial tendon transfer) procedure was performed in each foot with a flexible equinovarus foot abnormality. Follow up at a mean of 27.2 months (range, 8-40 months) yielded satisfactory clinical results without significant complications. Clinical equinovarus has not recurred after this procedure. All patients remain brace-free ambulators.     

Substance abuse: infant and childhood outcomes

IOL implantation in childhood is still controversial. INTENTION: The purpose of this retrospective study was to evaluate functional results and complications in children after IOL implantation. METHODS AND RESULTS: Posterior chamber IOLs were implanted in 52 eyes of 45 children. Cataract extraction was necessary because of traumatic, congenital/developmental and secondary lens opacities. Nine IOLs were implanted in children between the ages of 1.9 and 4 years, 17 IOLs from 4 to 8 years and 26 IOLs in patients 8 to 14.3 years old. In 38 of the 52 eyes (73%) vision improved after the surgery. Fifty-four percent had an acuity of at least 0.5. The functional results of children more than 8 years old were superior to those of younger patients. Children with traumatic cataracts had better visual acuities than those with other types of cataract. Except for secondary capsular opacities (75%) that could not be prevented by performing a primary posterior capsulotomy or capsulectomy and iris capture (31%), postoperative complications were rare. CONCLUSION: Favorable functional results can be achieved by IOL implantation in children with cataracts. Many patients require secondary surgical procedures because of capsular opacities and iris capture.     

Outcome, cost analysis, and long-term follow-up in preterm infants with massive grade IV germinal matrix hemorrhage and progressive hydrocephalus

OBJECTIVE: The benefit of aggressive management and surgical intervention in preterm infants with massive Grade IV intracranial hemorrhage has been questioned based on the poor outcome of this group of patients despite such therapy. To further delineate this problem, we reviewed the records of premature neonates in this category as to outcome and initial hospital cost. METHODS: We performed a retrospective review of the medical records at our institution from 1977 to 1987 to identify premature neonates who had sustained massive hemorrhagic infarction of one hemisphere in addition to having blood in both ventricles and progressive hydrocephalus. RESULTS: During the study, a total of 52 such patients were identified, only 19 (6 female and 13 male patients) of whom survived. Intellectual function was observed to be greater than 2 standard deviations below the mean in 15 of the 19 patients, between 1 and 2 standard deviations below the mean in 1 of 19, and 1 standard deviation below the mean in 3 of 19. Motor function was as follows: 12 of 19 had marked spastic quadriparesis, 2 of 19 had moderate spastic quadriparesis, 3 of 19 had spastic hemiplegia, 1 of 19 had spastic diplegia, and 1 of 19 had mild spastic hemiparesis. Eleven of 19 had chronic seizure disorders. The first hospitalization cost for the group of patients exceeded, on the average, $150,000 per patient for the 19 long-term survivors. CONCLUSION: As we have previously reported, logistic regression analysis determined that grade of hemorrhage was the only significant predictor of cognitive and motor outcomes. Most premature neonates with massive intracranial hemorrhages do not survive. The outcomes in those who do is very poor and the cost so high that we suggest that until therapeutic intervention exhibits efficacy, the consideration of withdrawal of life support should be presented as an option to the parents of these unfortunate children.     

Follow-up study of a right- and a left-hemispherectomized child: implications for localization and impairment of language in children

Two hemispherectomized girls, one operated on the right, the other on the left, were followed from time of surgery until 9 and 10 years of age and compared with respect to course of language acquisition following surgery. At conclusion of follow-up, receptive and expressive language, phoneme perception and production, and sentence processing of the two hemispherectomized children were compared with those of two control groups of similar age, one developing language normally, the other language-impaired. The left-hemispherectomized child's abilities were similar to those of the language-impaired children; the right-hemispherectomized child's abilities resembled those of the language-normal children. Implications for localization of developmental anomalies in language-impaired children are discussed.     

Granulomatous prostatitis on needle biopsy

Sixty-four children with malignant brain tumours diagnosed at less than 3 years of age were reported to the Finnish Cancer Registry from 1975 to 1993. The survival rate has improved significantly: the 5-year survival rate was 26% for all children, 13% for children diagnosed during 1975-85 (n = 30) and 40% for those diagnosed during 1986-93 (n = 34). Of the surviving children in 1986-93, 43% were categorized in Bloom's group I or II and could lead active lives without major disabilities. The remaining children had severe neurologic late complications, such as hemiplegia, intractable seizures, and mental retardation.     

Application of a colorimetric DNA-DNA hybridization method for identification of Aeromonas genomic species, isolated from diarrheal stools

In this report we describe a male patient with the interesting combination of a large congenital scalp defect, distal limb reduction anomalies, right spastic hemiplegia and hypoplasia of the left arteria cerebri media. Follow-up data from birth up to the age of 18 years revealed positive evolution with low to normal intelligence. The findings in the present patient, i.e. an Adams-Oliver syndrome associated with a severe neurological deficit, are best explained as resulting from interruption of the early embryonic blood supply.     

Responses of the skin over the dorsum to sunlight in hairless descendants of Mexican hairless dogs

18 consecutive cases of delayed rupture of the extensor pollicis longus tendon were recorded during 5 years; 4 were spontaneous, and 14 after distal radius fracture, most of which were undisplaced or only slightly displaced. 15 cases were operated upon with tendon transfers: 13 had extensor indicis proprius transfer, 1 transfer of the extensor carpi radialis longus, and 1 reoperated with the extensor communis to the little finger as a motor unit. Subjectively, nearly complete satisfaction was reported; all patients were able to elevate the thumb to the level of the palm and full independent index finger movements were noted. In this 5-year-period 4,400 patients with distal radius fractures were treated, giving an incidence of delayed tendon rupture after distal radius fracture of 0.3 percent.