Presentation and management of hydromyelia in children with Chiari type-II malformation

Hydromyelia in patients with myelomeningocele and Chiari-II malformation is a relatively frequent finding on MRI studies. However, not all children develop symptoms from the hydromyelia that requires treatment. Furthermore, treatment of hydromyelia in spina bifida patients is rather complex due to the associated malformations. The authors retrospectively analyzed 231 MRI studies carried out on spina bifida patients who presented neurological deterioration. Hydromyelia was found in 48.5% of the patients. Forty-five children with severe hydromyelia required treatment. These patients were first divided into 2 groups: those with holocord hydromyelia, and those with a segmental lesion. Fifteen patients presented symptoms characteristic of symptomatic Chiari-II malformation: neck rigidity; swallowing difficulty; pain in the upper extremeties; weakness or spasticity in the upper extremeties. Eighteen patients presented symptoms typical of the tethered cord syndrome: scoliosis; worsening bladder and/or bowel function; pain in the lower extremeties; weakness or spasticity in the lower extremeties. Twelve patients presented a mixed-type symptomatology. These patients subsequently underwent posterior cervical decompression, tethered cord release or insertion of a hydromyelia-pleural shunt according to the type of presenting symptoms and to the extent of the hydromyelic lesion. A pattern of successful treatment was identified for each type of presenting clinical and radiological picture. This has allowed the authors to determine an algorithm for optimal treatment of hydromyelia associated with Chiari-II malformation and myelomeningocele, which is proposed here.     

A case of lumbosacral lipoma-associated adult onset tethered cord syndrome with initial symptoms of sensory disturbance and intractable foot ulcers]

A 63-year-woman who complained of sensorimotor disturbance of the lower extremities and urinary disturbance was presented. She noted loss of superficial sensation in both feet and foot ulcers at the age of 20 years. Her illness was initially diagnosed as hereditary sensory neuropathy type 1 (HSN1). The foot ulcers were so intractable that she had to have her right leg amputated at the age of 48 years. She had a severely impaired superficial sensation in the lower extremities and buttock, distal weakness of the left leg, and dysuria at the age of 60 years. The neurological examination revealed that she had segmental sensorimotor disturbance below the levels of the 5th lumbar segment. MRI demonstrated tethered cord with a lumbosacral lipoma. Adult onset tethered cord syndrome (TCS) that presents with HSN 1-like symptoms as initial clinical features has not yet been reported. Foot ulcers are often seen in child onset TCS in which the degree of tethered cord is severer than adult onset cases. It is reported that release of the tethered cord promotes healing of the foot ulcers. We recommend MRI for the study of the lumbosacral cord of patients with HSN 1-like symptoms, because there is a possibility that such patients may have TCS and early surgical treatment is effective for TCS.     

Is apoptosis a "programmed cell death"?]

The purpose of this study was to evaluate the results and complications of anterior cruciate ligament surgery in middle-aged patients. Forty-five consecutive patients over 40 years old (average age, 44.6 years) who had arthroscopically assisted anterior cruciate ligament reconstructions with midthird patellar tendon autografts were evaluated. The patients returned for interviews, physical examinations, radiographs, Biodex dynamometer strength testing, and KT-1000 arthrometer testing at an average of 37 months after their surgeries (range, 24 to 96 months). The mean Lysholm and Gillquist score was 91, which corresponds to symptoms only with vigorous activity. The overall scores from the International Knee Documentation Committee form were 29 (64%) normal or nearly normal and 2 (4%) severely abnormal. Side-to-side differences as determined by the KT-1000 arthrometer were < or = 3 mm in 31 of 40 patients (78%), between 3 and 5 mm in 4 patients (10%), and > 5 mm in 5 patients at 30 pounds of anterior displacement. Seventy-six percent of the patients (N = 34) returned to their preoperative activity levels. Three patients required repeat arthroscopic surgery for persistent knee pain and two patients had graft ruptures. This study shows that when middle-aged patients undergo surgery, their results can be successful and satisfying to a degree similar to those of younger patients.     

Anion and osmolal gaps in a patient with alcoholism

Thirty-two patients with failed back syndrome received a trial of spinal cord stimulation. Stimulators were internalized in 26; long-term follow-up was available for 23 of these patients. Seventy-six percent of the 22 patients with stimulators still present at 1 year and 74% of the 19 patients with stimulators still present at 2 years were receiving 50% or better pain relief. Patients routinely underwent pain team evaluation and therapy, psychological testing and a transcutaneous electrical nerve stimulator trial prior to consideration of spinal cord stimulation. The most common complication was electrode migration. Spinal cord stimulation should be considered as an important therapeutic modality in carefully selected patients with failed back syndrome.     

Delayed postoperative tethering of the cervical spinal cord

Tethering of the spinal cord in the lumbar and sacral regions of children with congenital anomalies is a well-recognized problem; however, tethering in the cervical region has rarely been reported. A search of the literature revealed no reports of symptomatic postoperative cervical spinal cord tethering. The authors present five cases of delayed postoperative cervical spinal cord tethering and discuss the benefit of detethering in these patients. All five patients were young (16 to 42 years of age) at presentation. All had done well after an initial surgical procedure but returned between 1 and 31 years postoperatively with symptoms including severe headache, upper-extremity pain, and progressive neurological deficits. In each case, magnetic resonance imaging indicated dorsal tethering of the cord in the cervical region. Surgical exploration with microscopic sharp detethering of the cervical cord was performed on each patient with favorable results. To avoid retethering, wide Tutoplast duraplasty is recommended.     

Risks of surgical management for cavernous malformations of the nervous system

OBJECTIVE: As more information evolves regarding the natural history of cavernous malformations (CMs), the risks of operative intervention must be balanced against nonoperative management. In an attempt to better delineate the surgical risks for operable CMs, we undertook a retrospective analysis of 94 patients with 97 CMs surgically excised at the Massachusetts General Hospital. METHODS: Data regarding surgical complications and outcome measures, including neurological status and seizure outcome, were analyzed. RESULTS: The incidence of transient neurological morbidity was 20.6%, but only 4 of the 97 operations (4.1%) resulted in persistent disabling neurological complications and 2 (2.1 %) in nondisabling deficits. There was no operative mortality. Brain stem lesions (n=14) were associated with the highest incidence of neurological complications, both transient and persistent (odds ratio, 4.8; 95% confidence interval, 1.5-15.7). The overall neurological outcome was excellent or good in 89.7% of all lesions: 96.8% of lobar CMs (n=63), 64.2% of brain stem CMs (n=14), 87.5% of cerebellar CMs (n=8), 100% of cranial nerve CMs (n=4), and 75% of spinal cord CMs (n=8). Patients with brain stem and spinal cord CMs were in poorer preoperative neurological condition than were patients with CMs in other locations and therefore had a significantly reduced level of function after surgery (P < 0.01). There was improvement in 35.7% of the patients with brain stem lesions and 62.5% of the patients with spinal cord lesions after surgery. In the 38 patients presenting with seizures, 97% were seizure-free after surgery. CONCLUSION: The risks of operative management of CMs varies based on location. When evaluating patients with operable CMs for surgery, the incidence of complications as well as final neurological outcome should be carefully weighed against the existing knowledge of the natural history of lesions managed expectantly.     

Value of fast gradient echo magnetic resonance angiography as an adjunct to coronary arteriography in detecting and confirming the course of clinically significant coronary artery anomalies

Between January 1988 and February 1995, 133 tethered spinal cord-release procedures in 88 consecutive patients were performed at our institution and were used to determine survivorship data for surgical release of tethered spinal cord. The diagnoses included spinal dysraphism (67), achondroplasia (nine), isolated tethered cord (nine), cerebral palsy (three), and others (seven). Survivorship data were calculated for the initial and first-revision tethered cord release. There was a 50% revision rate by 5 years after initial tethered-cord release and a 57% revision rate by 2 years after a second release. Thirty-six patients were excluded for having <2 years of clinical follow-up, leaving 97 spinal cord releases in 52 patients available for outcome analysis. At a mean follow-up of 4.4 years (range, 2-11.3), 58% of patients required one or more orthopedic procedures after tethered-cord release. The average number of orthopedic procedures per year before release (0.28/year) was found to increase after initial release (0.39/year; p < 0.05). These data demonstrate the frequent need for operative revision after index tethered-cord release, especially in children with spinal dysraphism. In addition, the need for orthopedic procedures after tethered spinal-cord release frequently persists.     

"Suicide" gene for the control of graft-versus-host disease

OBJECTIVES: To determine the 10-year outcome of patients presenting with rest pain. METHODS: One hundred and three consecutive patients presenting with ischaemic rest pain in 1987 were followed up after 10 years. Hospital notes, death certificates and telephone interviews with patients were used to determine outcome. RESULTS: Follow-up data is available for 97 (94%) patients. Thirteen patients are alive (13.7%) after 10 years, 12 presented with rest pain alone and one had ulceration. Three of these had amputation. The commonest cause of death was myocardial infarction (n = 21, 25%). In those who had died, the median age of onset of symptoms was 72 years (49-93) for rest pain, 74 years (56-87) for ulceration and 71.5 years (45-85) for gangrene. Their survival after admission was a mean of 39 months with rest pain, 33 months with ulceration and 42 months with gangrene. The overall 5-year survival was 31% and the 10-year survival 13%. CONCLUSION: Patients presenting with ischaemic rest pain have a poor prognosis. The presence or absence of ulceration or gangrene does not influence the outcome. Most patients die from smoking-related diseases.     

Renal autotransplantation for the loin pain-hematuria syndrome: long-term followup of 26 cases

PURPOSE: We review the long-term results of renal autotransplantation as a form of nephron sparing renal denervation for patients with the loin pain-hematuria syndrome. MATERIALS AND METHODS: From 1985 to 1997, after exclusion of other urological, nephrological and psychiatric causes for severe intractable flank pain and recurrent hematuria, 22 patients with severe debility and heavy narcotic dependency underwent 26 renal autotransplantations for pain control. Postoperative pain relief, narcotic use, level of function in daily activities and status of the autograft were assessed. RESULTS: Median and mean followup was 78.5 and 84.7 months (range 30 to 138), respectively. There were 2 technical failures. Pain recurred within 2 years after 6 procedures, of which 3 resulted in transplant nephrectomy and 3 were managed with a reduced analgesic requirement. Of the 16 patients with minimum 5 years of followup 12 (75%) were pain-free after surgery with 3 additional patients pain-free after transplant nephrectomy. Overall, 18 of the 26 autotransplant procedures (69.2%) resulted in pain relief, in some cases beyond 10 years, with patients returning to normal daily activities. CONCLUSIONS: Renal autotransplantation results in durable narcotic-free favorable results in the majority of meticulously screened loin pain-hematuria syndrome patients. Although some failures, which usually occur within 2 years after surgery, can be expected, autotransplantation is justified as a nephron sparing denervation therapy for select loin pain-hematuria syndrome patients.     

Social and emotional patterns in adulthood: Support for socioemotional selectivity theory.

Explored 2 hypotheses derived from socioemotional selectivity theory: (1) Selective reductions in social interaction begin in early adulthood, and (2) emotional closeness to significant others increases rather than decreases in adulthood even when rate reductions occur. Transcribed interviews with 28 women and 22 men from the Child Guidance Study, conducted over 34 yrs, were reviewed and rated for frequency of interaction, satisfaction with the relationship, and degree of emotional closeness in 6 types of relationships. Interaction frequency with acquaintances and close friends declined from early adulthood on. Interaction frequency with spouses and siblings increased across the same time period, and emotional closeness increased throughout adulthood in relationships with relatives and close friends. Findings suggest that individuals begin narrowing their range of social partners long before old age. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

The reflex sympathetic dystrophy syndrome in patients who have had a spinal cord injury

Patients suffering from a spinal cord injury often present with a pain syndrome. Although the reflex sympathetic syndrome is a common diagnosis in some forms of neurological disease such as patients with a stroke, it is less frequent in those with a spinal lesion. The authors report eight patients with reflex sympathetic dystrophy who had a spinal cord injury. The diagnosis and treatment are discussed along with a review of literature.     

Strategies to prevent neurologic deficit based on motor-evoked potentials in type I and II thoracoabdominal aortic aneurysm repair

PURPOSE: Motor-evoked potentials (MEPs) were monitored during thoracoabdominal aortic aneurysm (TAAA) repair to assess spinal cord ischemia and evaluate the subsequent protective strategies to prevent neurologic deficit. METHODS: Between January 1996 and December 1997, 52 consecutive patients with type I (n = 24) and type II (n = 28) TAAA underwent surgery (mean patient age, 60 years; range, 21-78 years). The surgical protocol included left heart bypass, cerebrospinal fluid drainage, and monitoring transcranial myogenic MEPs. When spinal cord ischemia was detected, distal aortic pressure and mean arterial pressure were increased. By means of sequential crossclamping, MEPs were used to identify critical intercostal or lumbar arteries. RESULTS: Reproducible MEPs could be recorded in all patients, and spinal cord ischemia was detected within 2 minutes. During distal aortic perfusion, 14 patients (27%) showed rapid decrease in the amplitude of MEPs to less than 25% of baseline, indicating spinal cord ischemia, which could be corrected by increasing distal aortic pressure. The mean distal aortic pressure to maintain adequate cord perfusion was 66 mm Hg; however, it varied among individuals between 48 and 110 mm Hg. In 24 patients (46%), MEPs disappeared after segmental clamping and returned after reattachment of intercostal arteries. In 9 patients (17%), MEPs disappeared completely, but no intercostal arteries were found. After aortic endarterectomy, 6 or 8 mm Dacron grafts were anastomosed to intercostal arteries, and MEPs returned after reperfusion. Using this aggressive surgical approach based on MEPs, no early or late paraplegia occurred in this series. CONCLUSION: Monitoring of MEPs is an effective technique to assess spinal cord ischemia. Operative strategies based on MEPs prevented neurologic deficits in patients treated for type I and II TAAA.     

Epidural spinal cord stimulation for treatment of chronic pain--some predictors of success. A 15-year experience

BACKGROUND: We have used epidural spinal cord stimulation (SCS) for pain control for the past 15 years. An analysis of our series of 235 patients has clarified the value of specific prognostic parameters in the prediction of successful SCS. METHODS: Patients were followed up for periods ranging from 6 months to 15 years with a mean follow-up of 66 months. The mean age of the 150 men and 85 women in the study was 51.4 years. Indications for SCS included failed back syndrome (114 patients), peripheral vascular disease (39 patients), peripheral neuropathy (30 patients), multiple sclerosis (13 patients), reflex sympathetic dystrophy (13 patients), and other etiologies of chronic intractable pain (26 patients). RESULTS: One hundred and eighty-nine patients received permanent devices; 111 (59%) of these patients continue to receive satisfactory pain relief. Pain attributable to failed back syndrome, reflex sympathetic dystrophy, peripheral vascular disease of lower limbs, multiple sclerosis, and peripheral neuropathy responded favorably to spinal cord stimulation. In contrast, paraplegic pain, cauda equina syndrome, stump pain, phantom limb pain, and primary bone and joint disease pain did not respond as well. Cases of cauda equina injury had promising initial pain relief, but gradually declined after a few years. After long-term follow-up, 47 of the 111 successfully implanted patients were gainfully employed, compared with 22 patients before implantation. The successful patients reported improvements in daily living as well as a decrease in analgesic usage. Multipolar stimulation systems were significantly more reliable (p < 0.001) than unipolar systems. Complications included hardware malfunction, electrode displacement, infection, and tolerance. CONCLUSION: Aside from etiologies of pain syndromes as a prognostic factor, we have identified other parameters of success. In patients who have undergone previous surgical procedures, the shorter the duration of time to implantation, the greater the rate of success (p < 0.001). The diagnosis of failed back syndrome must be considered a confounding factor in our analysis. Those patients whose pain did not follow a surgical procedure had better responses to SCS than patients who had multiple surgical procedures prior to their first implant. The advent of multipolar systems has significantly improved clinical reliability over unipolar systems. Age, sex, and laterality of pain did not prove to be of significance.     

On being detectably sane in insane places: base rates and psychodiagnosis

A retrospective study of 60 adult patients with Arnold-Chiari malformation revealed that certain presenting clinical syndromes, although not pathognomonic, seemed to have definite prognostic significance. Surgical management by suboccipital decompression led to remarkable and enduring improvement in 65% of patients followed for as long as 14 years. In some patients, however, the initial postoperative benefit tended to fade into an insidious progression of neurological deficit. Despite operation, 18.6% of patients eventually experienced progressive neurological deterioration. Patients who presented with paroxysmal intracranial hypertension or cerebellar dysfunction had the best prognosis. Evidence of central cord involvement was the single most detrimental factor to neurological recovery.     

Frequency of nucleated red blood cells in maternal blood during the different gestational ages

PURPOSE: To study the course and visual outcome of Vogt-Koyanagi-Harada (VKH) syndrome in children. METHODS: The medical records of 97 consecutive patients with VKH syndrome were reviewed. Patients had been seen at King Khaled Eye Specialist Hospital in Riyadh, Saudi Arabia. The records were divided into two groups: those in whom the onset of the disease occurred at the age of 14 years or younger (pediatric group) and those in whom the disease manifested after the age of 14 years (adult group). RESULTS: Eight (61%) of the 13 children required cataract surgery compared to 14(17%) of 84 adult patients. Despite medical and surgical therapy, eight (61%) of the pediatric group had a final visual acuity of 20/200 or worse compared with 22 (26%) of the 84 adult patients (p=0.0103). CONCLUSION: The course of early-onset VKH (before the age of 14 years) appears to be aggressive. The ocular complications are more severe in children than in adults, leading to rapid deterioration in vision. Despite therapy, the final visual outcome of VKH in children is poor.     

Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults

BACKGROUND: Chronic idiopathic intestinal pseudo-obstruction, a syndrome of ineffectual motility due to a primary disorder of enteric nerve or muscle, is rare. AIMS: To determine the clinical spectrum, underlying pathologies, response to treatments, and prognosis in a consecutive unselected group of patients. METHODS: Cross sectional study of all patients with clinical and radiological features of intestinal obstruction in the absence of organic obstruction, associated with dilated small intestine (with or without dilated large intestine), being actively managed in one tertiary referral centre at one time. RESULTS: Twenty patients (11 men and nine women, median age 43 years, range 22-67) fulfilled the diagnostic criteria. Median age at onset of symptoms was 17 years (range two weeks to 59 years). Two patients had an autosomally dominant inherited visceral myopathy. Major presenting symptoms were pain (80%), vomiting (75%), constipation (40%), and diarrhoea (20%). Eighteen patients required abdominal surgery, and a further patient had a full thickness rectal biopsy. The mean time interval from symptom onset to first operation was 5.8 years. Histology showed visceral myopathy in 13, visceral neuropathy in three, and was indeterminate in three. In the one other patient small bowel motility studies were suggestive of neuropathy. Two patients died within two years of symptom onset, one from generalised thrombosis and the other from an inflammatory myopathy. Of the remaining 18 patients, eight were nutritionally independent of supplements, two had gastrostomy or jejunostomy feeds, and eight were receiving home parenteral nutrition. Five patients were opiate dependent, only one patient had benefited from prokinetic drug therapy, and five patients required formal psychological intervention and support. CONCLUSIONS: In a referral setting visceral myopathy is the most common diagnosis in this heterogeneous syndrome, the course of the illness is usually prolonged, and prokinetic drug therapies are not usually helpful. Ongoing management problems include pain relief and nutritional support.     

Ovarian neoplasms in children

OBJECTIVE: To review the clinical presentation, treatment, and outcome in a series of children with ovarian neoplasms. DESIGN: A retrospective review of the medical records in a case series of 29 girls with ovarian neoplasms. The length of follow-up ranged from 6 months to 7 1/2 years and averaged 3.0 years in the girls with malignant tumors. SETTING: The patients were treated at a large referral children's hospital. PATIENTS: Twenty-nine girls with ovarian neoplasms were treated from 1976 to 1992. The average age of the patients was 10 years and ranged from 2 to 16 years. MAIN OUTCOME MEASURES: The principal outcomes examined were mortality and surgical morbidity. RESULTS: The most common presenting symptoms for these ovarian tumors in pediatric patients included chronic abdominal pain, an abdominal mass, or distention. Three girls presented with precocious puberty or hirsutism. In 27 cases, the tumor was a primary ovarian lesion. In two patients, the ovarian mass was the presenting finding for a stage IV non-Hodgkin's lymphoma. Seventeen tumors were benign and 12 were malignant. Tumors originating from the germ-cell line predominated (n = 17). Seven of the 10 ovarian malignant neoplasms were stage I at the time of diagnosis. All but one of the girls with malignant tumors received either adjunctive radiation therapy or multiple-agent chemotherapy. Two girls with sex cord/stromal cell tumors who presented with stage I disease ultimately developed widespread metastases. Both girls with large epithelial tumors survived. All of the girls with benign tumors and seven (70%) of 10 with malignant lesions survived. CONCLUSION: Ovarian tumors are unusual lesions in the pediatric population. Unlike in adults, such neoplasms generally originate from the germ-cell line. Whereas most ovarian tumors in girls are benign, some children have malignant tumors that are very aggressive and do not respond well to adjuvant therapy. In particular, malignant sex cord/stromal cell tumors, even when they present at an early stage, may behave unpredictably.     

Effect of a bicycle safety program and free bicycle helmet distribution on the use of bicycle helmets by elementary school children

A retrospective follow-up study was performed on 40 patients, in which tuberculous spondylitis was treated conservatively between 1969 and 1985 with orthotic supports for an average of 16 months (range, 10-30 months) and with anti-tuberculous agents. All had persistent back pain, but none had neurological deficits. The mean follow-up period was 17 years (range, 10-26 years). Diagnosis was confirmed histopathologically. The spinal segments involved ranged from T5 to L5. The kyphotic angle was calculated according to Cobb. At final follow-up, 22 patients were pain free, 11 had occasional pain, 6 complained of pain in the morning, and 1 had chronic pain and needed frequent analgesics. Solid bony union was found in 75% of patients. The kyphotic deformity occurred in the thoracic spine with a mean angle of 20 degrees (range, 13-28 degrees) and in the lumbar spine with a mean angle 12 degrees (range, 5-26 degrees). The long-term follow-up of conservative treatment showed only slightly increased kyphosis. Conservative treatment is an alternative to surgical intervention in cases with kyphosis < 35 degrees.     

Clinical features and MR imaging in children with repaired myelomeningocele

The aim of the study is to define the role of associated malformations in the clinical evolution of children affected by myelomeningocele. MRI investigation of the spinal cord was carried out on 25 patients between the age of 7.3 and 18.10 with MMC repaired and followed up for at least 7 years. The relation between associated malformations demonstrated by MRI and clinical trend was analysed. The results are the following: 1) presence of asymptomatic tethered cord in all cases; 2) high frequency (92%) of Chiari malformation; 3) presence of syringomyelia in 20% of patients, symptomatic in 1; 4) presence of ventricular enlargement in 72% of cases without increased intracranial pressure syndrome. For a better therapeutic approach prospective MRI studies are needed in order to follow up associated malformations.     

Long-term results of pedicle stabilized thoracolumbar fractures in relation to the neurological deficit

88 patients with thoracolumbar fractures and short-segment (mono or two segment) pedicle instrumentation from the years 1985-92 had a follow-up examination after an average time of 5.6 years. The 56 men and 32 women had an average age of 32.6 years at the time of injury, 24 patients primarily had a complete and 43 patients an incomplete paraplegia, 21 patients showed no neurological deficits. The operative decompression of the spinal cord and stabilization of the injured spine by short segment pedicle instrumentation led to a complete or partial remission of the neurological deficits in 93% of the patients with incomplete paraplegia. Operative stabilization allowed an early mobilization and rehabilitation of these patients. We found no tendency to an increased complication rate in patients with neurological deficits compared to patients without neurological deficits. Patients with initially incomplete paraplegia complained more often about pain than all the other patients. Despite intensive rehabilitation and retraining programs handicapped patients had obvious disadvantages regarding their further careers.