Lena Westbom

Congenital malformations among infants whose mothers had gestational diabetes or preexisting diabetes

BACKGROUND Diabetes type 1 is associated with an increased risk for infant congenital malformations. It is debated whether this is true also at gestational diabetes. AIMS To study occurrence of congenital malformations in infants whose mothers had preexisting or gestational diabetes. STUDY DESIGN A register study covering over 1.2 million Swedish births in 1987-1997 based on the Swedish health registries. SUBJECTS We identified from the Medical Birth Registry 3864 infants born of women with preexisting diabetes and 8688 infants born of women with gestational diabetes. OUTCOMES MEASURES Congenital malformations identified in the Medical Birth Registry, the Registry of Congenital Malformations, and the Hospital Discharge Registry. The rates of congenital malformations among these infants was compared with the population rates. RESULTS At preexisting diabetes, the total malformation rate was 9.5% while the rate at gestational diabetes was similar to the population rate, 5.7%. At preexisting diabetes, certain conditions were more common than expected: orofacial clefts, cardiovascular defects, oesophageal/intestinal atresia, hypospadias, limb reduction defects, spine malformations, and polydactyly. For some of these conditions, an excess was found also for infants whose mothers had gestational diabetes. Infants with multiple malformations were in excess at preexisting diabetes but not at gestational diabetes but the specific type of malformations involved were similar in the two diabetes groups. CONCLUSIONS It is suggested that in the group of gestational diabetes exists a subgroup with an increased risk for a diabetes embryopathy, perhaps due to preexisting but undetected diabetes type 2.

Prevention of dislocation of the hip in children with cerebral palsy. The first ten years of a population-based prevention programme.

In 1994, a register for cerebral palsy and a health-care programme were started in southern Sweden with the aim of preventing dislocation of the hip in children with cerebral palsy. It involved all children with cerebral palsy born in 1992 or later. None of the 206 affected children born between 1992 and 1997 has developed a dislocation following the introduction of the prevention programme. Another 48 children moved into the area and none developed any further dislocation. Of the 251 children with cerebral palsy, aged between five and 11 years, living in the area on January 1, 2003, only two had a dislocated hip. One boy had moved into the area at age of nine with a dislocation and a girl whose parents chose not to participate in the programme developed bilateral dislocation. One boy, whose condition was considered to be too poor for preventative surgery, developed a painful dislocation of the hip at the age of five years and died three years later. Eight of 103 children in a control group, consisting of all children with cerebral palsy living in the area between 1994 and 2002, and born between 1990 and 1991, developed a dislocation of the hip before the age of six years. The decreased incidence of dislocation after the introduction of the prevention programme was significant (p < 0.001). Dislocation of the hip in cerebral palsy remains a serious problem, and prevention is important. Our screening programme and early intervention when lateral displacement of the femoral head was detected appear to be successful.

Cerebral palsy in a total population of 4–11 year olds in southern Sweden. Prevalence and distribution according to different CP classification systems

BackgroundThe aim of this study was to investigate the prevalence of cerebral palsy (CP) as well as to characterize the CP population, its participation in a secondary prevention programme (CPUP) and to validate the CPUP database.MethodsThe study population was born 1990–1997 and resident in Skåne/Blekinge on Jan 1st 2002. Multiple sources were used. Irrespective of earlier diagnoses, neuropaediatrician and other professional medical records were evaluated for all children at the child habilitation units. The CPUP database and diagnosis registers at hospital departments were searched for children with CP or psychomotor retardation, whose records were then evaluated. To enhance early prevention, CP/probable CP was searched for also in children below four years of age born 1998–2001.ResultsThe prevalence of CP was 2.4/1,000 (95% CI 2.1–2.6) in children 4–11 years of age born in Sweden, excluding post-neonatally acquired CP. Children born abroad had a higher prevalence of CP with more severe functional limitations. In the total population, the prevalence of CP was 2.7/1,000 (95% CI 2.4–3.0) and 48% were GMFCS-level I (the mildest limitation of gross motor function).One third of the children with CP, who were born or had moved into the area after a previous study in 1998, were not in the CPUP database. The subtype classification in the CPUP database was adjusted in the case of every fifth child aged 4–7 years not previously reviewed.ConclusionThe prevalence of CP and the subtype distribution did not differ from that reported in other studies, although the proportion of mild CP tended to be higher.The availability of a second opinion about the classification of CP/CP subtypes is necessary in order to keep a CP register valid, as well as an active search for undiagnosed CP among children with other impairments.

Prevention of severe contractures might replace multilevel surgery in cerebral palsy: results of a population-based health care programme and new techniques to reduce spasticity.

During the 1990s three new techniques to reduce spasticity and dystonia in children with cerebral palsy (CP) were introduced in southern Sweden: selective dorsal rhizotomy, continuous intrathecal baclofen infusion and botulinum toxin treatment. In 1994 a CP register and a health care programme, aimed to prevent hip dislocation and severe contractures, were initiated in the area. The total population of children with CP born 1990–1991, 1992–1993 and 1994–1995 was evaluated and compared at 8 years of age. In non-ambulant children the passive range of motion in hip, knee and ankle improved significantly from the first to the later age groups. Ambulant children had similar range of motion in the three age groups, with almost no severe contractures. The proportion of children treated with orthopaedic surgery for contracture or skeletal torsion deformity decreased from 40 to 15% (P=0.0019). One-fifth of the children with spastic diplegia had been treated with selective dorsal rhizotomy. One-third of the children born 1994–1995 had been treated with botulinum toxin before 8 years of age. With early treatment of spasticity, early non-operative treatment of contracture and prevention of hip dislocation, the need for orthopaedic surgery for contracture or torsion deformity is reduced, and the need for multilevel procedures seems to be eliminated.

Prevention of dislocation of the hip in children with cerebral palsy 20-year results of a population-based prevention programme

In 1994 a cerebral palsy (CP) register and healthcare programme was established in southern Sweden with the primary aim of preventing dislocation of the hip in these children. The results from the first ten years were published in 2005 and showed a decrease in the incidence of dislocation of the hip, from 8% in a historical control group of 103 children born between 1990 and 1991 to 0.5% in a group of 258 children born between 1992 and 1997. These two cohorts have now been re-evaluated and an additional group of 431 children born between 1998 and 2007 has been added. By 1 January 2014, nine children in the control group, two in the first study group and none in the second study group had developed a dislocated hip (p < 0.001). The two children in the first study group who developed a dislocated hip were too unwell to undergo preventive surgery. Every child with a dislocated hip reported severe pain, at least periodically, and four underwent salvage surgery. Of the 689 children in the study groups, 91 (13%) underwent preventive surgery. A population-based hip surveillance programme enables the early identification and preventive treatment, which can result in a significantly lower incidence of dislocation of the hip in children with CP.

Scoliosis in a Total Population of Children with Cerebral Palsy.

Study Design. Epidemiological total population study based on a prospective follow-up cerebral palsy (CP) registry. Objective. To describe the prevalence of scoliosis in a total population of children with CP, to analyze the relation between scoliosis, gross motor function, and CP subtype, and to describe the age at diagnosis of scoliosis. Summary of Background Data. Children with CP have an increased risk of developing scoliosis. The reported incidence varies, partly due to different definitions and study groups. Knowledge of the prevalence and characteristics of scoliosis in an unselected group of children with different CP types and levels of function is important for health care planning and for analyzing the risk in an individual child. Methods. A total population of 666 children with CP, aged 4 to 18 years on January 1, 2008, followed with annual examinations in a health care program was analyzed. Gross Motor Function Classification System (GMFCS) level, CP subtype, age at clinical diagnosis of scoliosis, and the Cobb angle at the first radiographical examination were registered. Results. Of the 666 children, 116 (17%) had mild and another 76 (11%) had moderate or severe scoliosis based on clinical examination. Radiographical examination showed a Cobb angle of more than 10° in 54 (8%) children and a Cobb angle of more than 20° in 45 (7%) children. The risk of developing scoliosis increased with GMFCS level and age. In most children, the scoliosis was diagnosed after 8 years of age. Children in GMFCS level IV or V had a 50% risk of having moderate or severe scoliosis by 18 years of age, whereas children in GMFCS level I or II had almost no risk. Conclusion. The incidence of scoliosis increased with GMFCS level and age. Observed variations related to CP subtype were confounded by the GMFCS, reflecting the different distribution of GMFCS levels in the subtypes. Follow-up programs for early detection of scoliosis should be based on the childs GMFCS level and age.

Long-term outcomes five years after selective dorsal rhizotomy

BackgroundSelective dorsal rhizotomy (SDR) is a well accepted neurosurgical procedure performed for the relief of spasticity interfering with motor function in children with spastic cerebral palsy (CP). The goal is to improve function, but long-term outcome studies are rare. The aims of this study were to evaluate long-term functional outcomes, safety and side effects during five postoperative years in all children with diplegia undergoing SDR combined with physiotherapy.MethodsThis study group consisted of 35 children, consecutively operated, with spastic diplegia, of which 26 were Gross Motor Function Classification System (GMFCS) levels III–V. Mean age was 4.5 years (range 2.5–6.6). They were all assessed by the same multidisciplinary team at pre- and at 6, 12, 18 months, 3 and 5 years postoperatively. Clinical and demographic data, complications and number of rootlets cut were prospectively registered. Deep tendon reflexes and muscle tone were examined, the latter graded with the modified Ashworth scale. Passive range of motion (PROM) was measured with a goniometer. Motor function was classified according to the GMFCS and measured with the Gross Motor Function Measure (GMFM-88) and derived into GMFM-66. Parents opinions about the childrens performance of skills and activities and the amount of caregiver assistance were measured with Pediatric Evaluation Disability Inventory (PEDI).ResultsThe mean proportion of rootlets cut in S2-L2 was 40%. Muscle tone was immediately reduced in adductors, hamstrings and dorsiflexors (p < 0.001) with no recurrence of spasticity over the 5 years. For GMFCS-subgroups I–II, III and IV–V significant improvements during the five years were seen in PROM for hip abduction, popliteal angle and ankle dorsiflexion (p = 0.001), capacity of gross motor function (GMFM) (p = 0.001), performance of functional skills and independence in self-care and mobility (PEDI) (p = 0.001).ConclusionSDR is a safe and effective method for reducing spasticity permanently without major negative side effects. In combination with physiotherapy, in a group of carefully selected and systematically followed young children with spastic diplegia, it provides lasting functional benefits over a period of at least five years postoperatively.

Development of lower limb range of motion from early childhood to adolescence in cerebral palsy: a population-based study.

BackgroundThe decreasing range of joint motion caused by insufficient muscle length is a common problem in children with cerebral palsy (CP), often worsening with age. In 1994 a CP register and health care programme for children with CP was initiated in southern Sweden. The aim of this study was to analyse the development of the passive range of motion (ROM) in the lower limbs during all the growth periods in relation to gross motor function and CP subtype in the total population of children with CP.MethodsIn total, 359 children with CP born during 1990-1999, living in the southernmost part of Sweden in the year during which they reached their third birthday and still living in the area in the year of their seventh birthday were analysed. The programme includes a continuous standardized follow-up with goniometric measurements of ROM in the lower limbs. The assessments are made by each childs local physiotherapist twice a year until 6 years of age, then once a year. In total, 5075 assessments from the CPUP database from 1994 to 1 January 2007 were analysed.ResultsThe study showed a decreasing mean range of motion over the period 2-14 years of age in all joints or muscles measured. The development of ROM varied according to GMFCS level and CP subtype.ConclusionWe found a decreasing ROM in children with CP from 2-14 years of age. This information is important for both the treatment and follow-up planning of the individual child as well as for the planning of health care programmes for all children with CP.

Survival at 19 years of age in a total population of children and young people with cerebral palsy.

Aim  The aims were to investigate survival of children with cerebral palsy (CP) and to search for modifiable factors that influence survival in CP.

Family therapy in poorly controlled juvenile IDDM: effects on diabetic control, self‐evaluation and behavioural symptoms

Diabetic control, behavioural symptoms and self‐evaluation were assessed in 25 children with IDDM who were in poor metabolic control (P group), before and subsequent to one of two treatment conditions: family therapy and conventional treatment (C). In addition, data were collected from 12 patients in optimal control (O group). Prior to treatment the patients in poor control were rated higher than those in the O group for symptoms indicating somatization and internalization of conflict and showed a gloomier self‐image. The O group patients had fewer behavioural symptoms and a more positive self‐image than non‐diabetic reference groups. Diabetic control improved after family therapy only. Furthermore, the family therapy group improved on a combined measure of behavioural symptoms and one aspect of self‐evaluation (relations to parents and family). The results suggest that IDDM may either interfere with or foster the childs development towards autonomy, depending on family interaction patterns which affect the childs behaviour and self‐esteem. Family therapy is a treatment option which can mediate improved diabetic control by changing family relationships to allow for a better balance between parental and self‐care of the child with poorly controlled IDDM.