Spinal vascular malformation in a child, revealed by lumbar puncture]

Spinal vascular malformations are exceptional in children. We report a case in 1 year-old patient due to a spinal vascular malformation which was revealed by paraplegia after lumbary punction. The diagnosis was made by MRI. Spontaneous outcome was characterised by the persistence of paraplegia.     

Acute paediatric paraplegia: A case series review

Paediatric paraplegia resulting from spinal cord pathology of any cause is rare; hence prognostic information for children less than 16 years is limited. This case series review aims to ascertain all cases of paediatric paraplegia from 1997 to 2012 in the former Northern Region of England.MethodsChildren presenting with sudden paraplegia before the age of 16 years were multiply ascertained from databases in the regional paediatric neurology, neuroradiology, neuro-oncology and adult spinal injuries units. Data were obtained from retrospective case note review.ResultsA total of 44 cases (24 female) were identified. The incidence is estimated at 0.49 per 100,000 children under 16/year (95% confidence interval 0.41–0.57). Mean age of onset was 8.8 years and the most common aetiology was inflammatory. Twelve months post presentation, mortality was zero and a good outcome (defined as Gross Motor Function Classification System grades I or II) was seen in 66.6%. Motor outcome at 12 months was associated with the presence of bladder/bowel signs at presentation, previous viral illness and initial severity of paraplegia. Bladder signs at presentation were the strongest predictor of prognosis (OR for poor motor outcome 10.3). We were unable to demonstrate a relationship between aetiology and late outcome.ConclusionPaediatric paraplegia is rare. Mortality rates are low and 66.6% have a good outcome with fully or nearly independent walking. Bladder signs are the strongest predictor of prognosis.     

Spinal cord vascular injuries following surgery of advanced thoracic neuroblastoma: an unusual catastrophic complication

BACKGROUND: Spinal cord injury is a possible complication associated with removal of thoracic dumbbell neuroblastomas. Our experience with two children whose postsurgical course was complicated by midthoracic spinal cord ischemia is reported there. Permanent paraplegia resulted in both. PROCEDURE AND RESULTS: Preoperative awareness of the origin and distribution of the Adamkiewicz artery (arteria radiculomedullaris magna, ARMM) and of the possible collateral pathways for spinal cord blood supply may be helpful in the planning of operations that involve dissection in the midthoracic posterior mediastinum. Otherwise, a flaccid paraplegia may result. CONCLUSIONS: The syndrome is presumed to be triggered by a spasm, an embolism, or a iatrogenic interruption of the ARMM.     

Persistent or severe back pain and stiffness are ominous symptoms requiring prompt attention

BACKGROUND: Children with severe or persistent back pain and stiffness often have an underlying organic cause but there is a large differential diagnosis, examination may be difficult and the problem is relatively rare in general paediatric practice. These difficulties appeared to lead to delays in diagnosis and management of children with this problem. OBJECTIVES: To provide an approach to the diagnosis and management children with severe or persistent back pain or stiffness based on our clinical experience and the literature. METHODOLOGY: The case histories of 10 children with severe back pain seen by the authors over a 5-year period were reviewed. They were chosen as illustrative examples of the diagnostic and management problems and did not represent a systematic review of all cases seen by the authors over that time. RESULTS: Underlying causes included infection, inflammation, neoplasm, trauma and vascular malformation. Four of the children had spinal cord compression which required urgent decompression. There was one child with a conversion disorder but three children with organic disease were initially felt to have a conversion disorder. Investigations generally proceeded relatively slowly and the problem was not regarded as a semi-urgent situation carrying the risk of permanent paraplegia. Magnetic resonance imaging (MRI) scan of the spine was the investigation of choice. CONCLUSION: Children with severe or persistent back pain and stiffness have a wide variety of underlying causes. The possibility of underlying spinal cord compression should always be considered in children with this presentation. If the diagnosis is not obvious, MRI scan of the spine should be arranged without delay.     

Transverse symptomatics in lymphosarcoma patients (author's transl)

We report about two patients with compression of the spinal cord caused by lymphosarcomas. In the first patient the tumour was localized in the lumbal region and destroyed two vertebrae. In the second patient the lymphosarcoma also infiltrated the meninx, the cord, and some internal organs. Both children died.     

Secondary osteoporosis in long-term bedridden patients with cerebral palsy

Background: The aim of the present paper was to investigate 20 pediatric patients with cerebral palsy and secondary osteoporosis and consider the efficacy, influence and index of treatment.
Methods: A total of 10 boys and 10 girls, age 1–16 years (mean 7.6 years) with secondary osteoporosis and cerebral palsy treated for 6 months, were studied. Bone mineral density (BMD) was measured. The bone turnover markers were measured just before and 4 months after treatment or at the time of early discontinuation of treatment. The treatment was classified into two groups: vitamin D (alfacarcidol) only; and with bisphosphonate (risedronate).
Results: Monotherapy with alfacarcidol was effective for secondary osteoporosis in children, but when used in combination with risedronate it was even more effective in improving BMD. In the two groups, bone-specific alkaline phosphate (BAP) decreased from pretreatment to post-treatment assessment in all but one case, but there was no significant correlation in the difference in ΔBAP with ΔBMD. ΔBAP assumed changes in BAP in treatment either before or after, and ΔBMD also assumed changes in BMD. N-telopeptides of type I collagen (NTX)/Cr decreased in all cases. The correlation of ΔNTX/Cr with ΔBMD was not significant. The therapy and its efficacy did not correlate to the patients' age, sex, medicine regimen or enteral nutrition.
Conclusions: Risedronate therapy is effective for patients presenting with secondary osteoporosis with cerebral palsy. Moreover, it is desirable to treat patients more aggressively from the early stage because risedronate is not affected by the patients' other factors.     

Asylum-seeking children with severe loss of activities of daily living: clinical signs and course during rehabilitation

Aim:  To investigate whether severe loss of activities of daily living (ADL) in asylum-seeking children is associated with physical disease or toxic influences and to describe the clinical course during the recovery process.
Methods:  A total of 29 asylum-seeking children with severe loss of ADL were regularly assessed by physical examinations, laboratory tests and a structured evaluation of their ADL status during rehabilitation.
Results:  A total of 12 children had previously recorded suicide attempts and 21 were recorded to have experienced traumatic events in their country of origin.
The mean time from turning point to recovery was 6 months. Of the study participants, 22 needed enteral feeding and 18 gained weight during recovery. All children had a pulse rate and systolic blood pressure within the normal range. No sign of intoxication or physical disease was identified in laboratory tests or clinical examinations, with the exception of one case of epilepsy.
Conclusion:  Physical disease, pharmacological sedation or anorexia nervosa was not considered to be a probable cause of the loss of ADL in these children. The high rate of psychosocial risk factors and the stressful event of being in an asylum-seeking process call for further investigation of psychosomatic mechanisms .     

Successful chemotherapeutic decompression of primary endodermal sinus tumor presenting with severe spinal cord compression

Management of spinal cord compression from a primary paraspinal endodermal sinus tumor (EST) is described. A 17-month-old child presented for treatment with near-complete paraplegia secondary to spinal cord compression from a primary paraspinal EST. The child was treated with cisplatin-based chemotherapy without laminectomy or radiation therapy. Rapid resolution of symptoms was observed. The child had an excellent tumor response and complete neurologic recovery with no sequelae. Chemotherapy alone is an alternative to laminectomy or radiation therapy in the management of epidural cord compression from EST, even when the cord compression is severe.     

Paraplegia complicating umbilical artery catheterization]

Two cases of definitive paraplegia following umbilical artery catheterization are reported. In both patients, the paraplegia can be attributed to a myelomalacia secondary to the thrombosis or embolization of the feeding arteries of the cord. The treatment by fibrinolytic agents was unsuccessful and the only therapeutic approach appears to be a preventive one. This prophylaxis would best be accomplished by limiting the indications and duration of umbilical artery catheterization.     

Late Neurological Sequelae of Tuberculous Meningitis

The neurological findings in a follow-up study of 103 children treated for and cured of tuberculous meningitis in 1949—54, are presented. Fifty children were found to be free of late neurological sequelae, 29 had minor neurological sequelae including 2 with cranial nerve palsies, nystagmus, and ataxia, 13 with mild disturbances of coordination and 10 with symptoms of upper motor neuron lesion without subjective complaints. Gross neurological sequelae were found in 22 cases, including 8 with spastic palsies, 9 with spastic palsies and convulsions, and 5 with convulsions without spastic palsies. Two children were found to have sequelae of spinal lesions; one had paraplegia and the other disturbance of sensation on the dorsum of the left foot. Fourteen children had convulsions.     

Atopy, home environment and the risk of childhood-onset type 1 diabetes: a population-based case–control study

Background:  The marked increases in the incidence of type 1 diabetes in recent decades strongly suggest the role of environmental influences. These environmental influences remain largely unknown.
Objective:  To investigate atopy and home environment (such as children living at home, sharing a bedroom and house moves) as potential risk factors for type 1 diabetes.
Subjects and method:  In Northern Ireland, 175 children with type 1 diabetes and 4859 control children completed a questionnaire on atopy experience, family composition and home environment. Control children from two age groups (6–8 yr old and 13–14 yr old) were identified from randomly selected primary and secondary schools across Northern Ireland. Cases were identified from a population-based type 1 diabetes register.
Results:  There was little evidence of a difference in the proportion of participants with a history of atopy in the cases compared with controls. There was a significant reduction in the risk of diabetes in children who lived with more siblings {odds ratio (OR) = 0.58 [95% confidence interval (95% CI) 0.39–0.85] in children who lived with three or more siblings compared with one or none} and in children who moved house more often [OR = 0.59 (95% CI 0.40–0.88) in children who moved house twice or more compared with never].
Conclusion:  The reduced risk of type 1 diabetes in children living with siblings, sharing a bedroom and moving house more often could reflect the protection afforded by exposure to infections in early life and consequently may provide support for the hygiene hypothesis.     

Spinal cord compression: a review of 70 pediatric patients

The authors report their experience with 70 pediatric patients with spinal cord compression (SCC) due to malignancies identified among 898 patients with solid tumors. An extradural tumor was the most frequent cause of SCC (71%); 54% of these were soft tissue sarcomas and neuroblastoma. Most intradural tumors (70%) were outside the spinal cord, 9/12 being metastatic medulloblastomas. The SCC localized mainly to the dorsal and lumbosacral regions (42% each). Pain was the most common symptom (94%). MRI proved diagnostic in all cases in which it was used, while myelography was diagnostic in 85% of 26 patients. CT scan demonstrated the lesion in 83% of the patients. Laminectomy was provided for patients with paraplegia of less than 96 h evolution; isolated recurrence of the main tumor; a primary spinal cord tumor; progression of neurologic symptoms after chemotherapy/radiotherapy; chemotherapy and radiotherapy-resistant tumor (when known); resection of a paraspinal tumor. Surgery was avoided when prognosis of primary disease was poor or risks exceeded possible benefits. Twelve/twenty-one (57%) patients with paraplegia were able to walk after laminectomy only, while 14% (2/14) improved after chemotherapy and radiotherapy. Survival rates were 38% for the former and 36% for the latter. Overall survival was related to the original malignancy. All patients (12) admitted without paraplegia and submitted to laminectomy were able to walk, and of these, 6 presented a primary spinal cord tumor. The remaining had paraspinal tumors that extended to the spinal canal. Almost 87% (20/23) of the patients without paraplegia who submitted to medical treatment were able to walk, while only one progressed to paraplegia. Patients with SCC may entertain radio- and chemotherapy when harboring tumors responding to such therapies and present no evidence of neurologic damage progression. The latter manifestation is a strong indication for laminectomy without delay.     

Lumbar disc herniation in young children

Aim:  This article explores lumbar disc herniation in young children through focusing on matters relevant to patient presentation, physical examination, differential diagnosis, imaging and treatment.
Methods:  Major databases were searched for studies that addressed lumbar disc herniation in young children.
Results:  Diagnosis of lumbar disc herniation in young children is usually delayed because of the rarity and lack of experience with this entity and the difficulty in extracting a reliable medical history. Nevertheless, lumbar disc herniation should be considered in the differential diagnosis of any young child presenting with a chief complaint of back pain and/or radiculopathy, especially in the setting of recent trauma. This should be coupled with a directed physical examination to elicit signs and narrow the differential diagnosis. Imaging studies, mainly magnetic resonance imaging, will help establish a diagnosis; yet radiographs are still required to exclude other spinal lesions. The initial management of lumbar disc herniation in children is the same as that in adults and consists of conservative treatment unless lumbar disc herniation affects the patient's motor and neurological functions in which case, early surgical treatment must be undertaken. Although the latter remains more difficult, current experience suggests a favourable outcome.
Conclusion:  Awareness of lumbar disc herniation will help the paediatrician extract a relevant medical history, perform a directed physical examination, and order appropriate imaging studies. This will aid in initiating early intervention, be it conservative or operative, and achieving a favourable outcome.     

遗传性痉挛性截瘫散发病例的诊断

目的：分析遗传性痉挛性截瘫发病例的临床特点，探讨影响预后的因素。方法：总结了20例散发遗传性痉挛性截瘫患者的临床资料，并进行了随访。结果：起病年龄平均为4岁7个月，首发症状均为步态异常，以进行性痉挛性截瘫为特征，且肌张力增高较肌力减弱更为显著。体感诱发电位检查6例中5例有异常。脊髓MRI检查有2例异常。10例4岁前发病者，8例不能独走，5例发展为复杂型；10例4岁以后发病者，均能维持独直能力且均为单纯型，2例DDST筛查有精神发育落后的患儿均转为复杂型，结论：遗传性痉挛性截瘫散发病例的临床特点与有家族史者相似。体感诱发电位检查可具有重要的诊断价值。4岁前发病者或早期伴有精神发育落的地凰可能较差。     

Spinal cord injury associated with all-terrain vehicle accidents

Five instances of three-wheeler, all-terrain vehicle accidents resulting in spinal cord injury are presented. Age range of victims was from 7 to 18 years. Of the five cases of spinal cord injury, three resulted in quadriplegia and two in paraplegia. Reports of all-terrain vehicle accidents in childhood from the US Consumer Product Safety Committee are also reviewed. Concerns are raised about the need for further data gathering about instances and types of injuries from these types of vehicles and the safety of use in all ages of children for these heavy, potentially high-speed vehicles.     

The social and educational outcome of a cohort of rural children in relation to the racial and socio-economic groupings of their parents

Objective : To evaluate the outcome after a generation of extra health and educational intervention among children whose antenatal and perinatal characteristics appeared influenced by racial origin and socio-economic status.
Methodology : Sixty-five of an original cohort of 103 children born in Cunnamulla, Queensland, 1974-75, remained in the area allowing educational and social outcomes to be assessed: 40 were Aboriginal, 25 were children of unemployed Caucasians and 38 were children of employed Caucasians. The criteria for a successful outcome on leaving school were progression to further education or finding paid employment.
Results : Fifty-three of the children who had left school were assessed (the remaining 12 were still at school). Only four of the 30 assessed Aborigines had successful outcomes compared with 10 of the 13 employed Caucasians and three of the 10 unemployed Caucasians. Gender influenced success, with females under-represented such that among the 23 Aboriginal and unemployed Caucasian girls only one had a successful outcome; 13 of these 23 girls were pregnant before finishing secondary education.
Conclusions : The educational and social success of Aborigines is poor compared with the children of employed Caucasians. Outcomes for children of unemployed Caucasians are similar to those of Aborigines, suggesting failure of a range of government and community intervention programmes.     

An outbreak of hepatitis A in a day-care center: Immunoprophylaxis with human immunoglobulin

Abstract Background : An outbreak of seven cases of hepatitis A (HA) occurred in a day-care center. Five of the cases were children attending the center, one was a nurse and one was the mother of a child. It is probable that the first case with HA was a male child infected by an unknown source.
Methods and Results: Human immunoglobulin (HIG) was administered to both children and staff at the center following which there were no new cases of infection among in-center contacts. However, a new case of HA among household contacts developed 3 weeks following the treatment of in-center contacts.
Conclusions : The outbreak may have been prevented if the sibling (case 2) of the source case of infection (case 1) had been given HIG as soon as infection had been confirmed. Additionally, the data suggest that HIG for prevention of HA should be given not only to children but also to their parents.     

Intramedullary spinal neurenteric cyst with fluid-fluid level

Spinal neurenteric cysts are rare intradural extramedullary lesions of the spine, commonly located in the cervical and thoracic regions. The majority localize ventral to the spinal cord and are associated with other vertebral anomalies. Here, the authors report a rare case of a 3-year-old boy presenting with a 1-week history of meningismus followed by rapid-onset (over a few hours) paraplegia. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the cervicothoracic region of the spinal cord without associated bony or soft tissue abnormalities. To the best of the authors' knowledge, such clinical and radiological presentation of a spinal neurenteric cyst has never been reported. A brief review of the pertinent literature is presented, and the possible pathophysiology of such a presentation is also discussed.     

Malignant lymphoma in childhood

Summary Twenty-nine cases of malignant lymphoma in childhood excluding the leukaemic involvement, seen over a period from 1959 to 1971 have been reviewed. Males (82.8%) were more commonly affected than females (17.2%). One case of lymphosarcoma and four cases of reticulum cell sarcoma were extranodal in origin while ten, thirteen, and one case of lymphosarcoma, Hodgkin’s 3 disease and reticulum cell sarcoma respectively were of nodal origin. In the majority of cases, the age of onset was 2–4 years and 10–12 years in lymphosarcoma, 6–8 years in Hodgkin’s 3 disease and 10–14 years in reticulum cell sarcoma. The age of onset, clinical features and histological patterns have been discussed. From the Department of Pathology and Bacteriology, S.P. Medical College, Bikaner.