kyphoscoliosis pregnancy

SESSION TYPE: ICU Safety and Quality PostersPRESENTED ON: Wednesday, October 24, 2012 at 01:30 PM - 02:30 PMPURPOSE: Restrictive lung disease is relatively uncommon in pregnancy, and limited data are available to direct peri-partum and post-partum management of patients with severe disease. This study reviewed the course of pregnant patients with severe restrictive lung diseases, in our tertiary care referral centre over the past 10 years.METHODS: Patients with Forced Vital Capacity (FVC] < 70% predicted were identified from office charts...

PURPOSE: We present the anesthetic management of a parturient with VACTERL association undergoing combined regional and general anesthesia for Cesarean delivery. Defined as a syndrome, VACTERL association comprises at least three of the following abnormalities: vertebral, anal atresia, cardiac, tracheoesophageal, renal, and limb. CLINICAL FEATURES: The patient's anatomic abnormalities and comorbidities comprised severe cervicothoracic scoliosis, kyphoscoliosis, congenitally fused ribs, and severe restrictive lung disease...

INTRODUCTION: Split cord malformation (SCM) is rare congenital disorder of spine in which bony, fibrous, or cartilaginous septum subdivides partially or completely the vertebral canal. SCM can be associated with other spinal abnormalities such as spina bifida, Arnold-Chiari malformation, hemivertebra, butterfly vertebra, or kyphoscoliosis and also can be seen as part of Jarcho-Levin syndrome. Prenatal diagnosis of SCM is possible by ultrasonography (US). Fetal magnetic resonance imaging (MRI) and amniotic fluid acetylcholine esterase (AF-AChE) levels can be helpful to rule out additional anomalies...

Fetal akinesia deformation sequence is a clinically and genetically heterogeneous disorder characterized by a variable combination of arthrogryposis, fetal akinesia, intrauterine growth restriction, developmental abnormalities such as cystic hygroma, pulmonary hypoplasia, cleft palate, cryptorchidism, cardiac defects and intestinal malrotation, and occasional pterygia of the limbs. Multiple pterygium syndrome is a clinically and genetically heterogeneous disorder characterized by pterygia of the neck, elbows and/or knees, arthrogryposis, and other phenotypic features such as short stature, genital abnormalities, craniofacial abnormalities, clubfoot, kyphoscoliosis, and cardiac abnormalities...

We report two male sibs, born from unrelated French Caribbean parents, presenting with an unclassifiable storage disorder. Pregnancy and delivery were uneventful. Stunted growth was noted during the first year of life. Both children have short stature (below - 4SD) with short trunk, barrel chest, micromelia with rhizomelic shortening, severe kyphoscoliosis, pectus carinatum, short hands and feet with metatarsus adductus, and excessive joint laxity of the small joints. Learning difficulties with borderline intelligence quotient (IQ) were noted in one of them...

To determine the sonographic features of body stalk anomaly in the first trimester using 2-dimensional (2D) and 3-dimensional (3D) sonography, we conducted a retrospective analysis of all nuchal translucency sonographic examinations performed between January 1, 2006, and January 1, 2010, at our institution. From a total of 6952 nuchal translucency sonographic examinations, 4 cases of body stalk anomaly were identified. All cases were characterized by an absent umbilical cord and a large ventral wall defect with herniation of the abdominal contents into the extraembryonic coelom...

The improvement of antenatal management and surgical techniques has greatly increased the survival rate of infants with spina bifida. More of these women are reaching adulthood and reproductive age and therefore could become pregnant. Pregnancy complications depend on the kind of spina bifida and subject's condition.We report a case of woman with a severe kyphoscoliosis, that progressively affects lung capacity until 32 weeks of gestation, when she underwent caesarean section.These patients deserve careful obstetric care, genetic counselling and urological, obstetric, neurological and anaesthetic management...

BACKGROUND: Spondyloepimetaphyseal dysplasia, short limb-abnormal calcifications type (SEMD, SL-AC) is a rare autosomal recessive condition with a grave prognosis. OBJECTIVE: We aimed to describe the progression of symptoms from fetal age to adolescence in SMED, SL-AC patients. MATERIALS AND METHODS: We retrospectively evaluated radiological findings on plain films, CT and MRI for eight children with genetically proven SEMD (male:female ratio 4:4, ages 30-week fetus to 18 years) and summarized findings from case reports and case series in the literature...

BACKGROUND: The Ehlers-Danlos Syndrome (EDS) is a heritable connective tissue disorder characterized by hyperextensible skin, joint hypermobility and soft tissue fragility. The classic subtype of EDS is caused by mutations in one of the type V collagen genes (COL5A1 and COL5A2). Most mutations affect the type V collagen helical domain and lead to a diminished or structurally abnormal type V collagen protein. Remarkably, only two mutations were reported to affect the extended, highly conserved N-propeptide domain, which plays an important role in the regulation of the heterotypic collagen fibril diameter...

OBJECTIVES: To review the sonographic features of spinal anomalies in first-trimester fetuses presenting for screening for chromosomal abnormalities. METHODS: Fetuses with a spinal abnormality diagnosed prenatally or postnatally that underwent first-trimester sonographic evaluation at our institution had their clinical information retrieved and their sonograms reviewed. RESULTS: A total of 21 fetuses complied with the entry criteria including eight with body stalk anomaly, seven with spina bifida, two with Vertebral, Anal, Cardiac, Tracheal, Esophageal, Renal, and Limb (VACTERL) association, and one case each of isolated kyphoscoliosis, tethered cord, iniencephaly, and sacrococcygeal teratoma...

OBJECTIVE: To assess the outcome of pregnancies complicated by kyphoscoliosis in modern obstetric and orthopedic care. METHODS: A total of 22 kyphoscoliotic patients with 34 pregnancies were identified from 46,828 pregnancies between 1998 and 2009. Their obstetric records and associated orthopedic problem were studied. RESULTS: The incidence of kyphoscoliosis complicating pregnancy was 0.072%. The mean age of the patients in their index pregnancy was 28...

Use of neuraxial block in a patient with severe kyphoscoliosis is controversial. We describe the anaesthetic management by spinal anaesthesia of a parturient with severe kyphoscoliosis in obstructed labour. The perioperative course was uneventful. We suggest that a patient with severe kyphoscoliosis may be successfully managed by spinal anaesthesia for caesarean section.

Jarcho-Levin syndrome is characterized by short trunk dwarfism associated with rib and vertebral anomalies. The syndrome encompasses a group of disorders with phenotypic and inheritance variations. Here we report a prenatally diagnosed patient with spondylocostal dysostosis (SCD) with accompanying congenital inguinoscrotal hernia. A 28-year-old pregnant women, gravida 4, para 3, was referred to our clinic with a diagnosis of wedging of fetal thoracal vertebra and kyphoscoliosis at 28 weeks of gestation. Upon evaluation, fetal vertebral wedging and kyphoscoliosis were confirmed with the addition of thoracic circumference below 3rd percentile, short thorax length, and mild pyelectasis...

Spinal hamartomas are rare lesions consisting of disorganized ecto- and mesodermal tissues of the spinal region. While postnatal identification of spinal hamartomas has been reported, a literature search did not reveal any published reports of prenatal identification of spinal hamartomas. Here we report a 46,XX fetus who presented at 20 weeks' gestation with a lower thoracic and lumbar kyphoscoliosis, suspected spina bifida, and amniotic fluid alpha-fetoprotein (AFP) levels within the normal range. Interestingly, autopsy at 22 weeks revealed a lumbosacral spinal hamartoma with kyphoscoliosis...

PURPOSE: To describe the anesthetic implications, and management of a medically complex parturient, who presented for Cesarean delivery (CD). The patient had poliomyelitis complicated with severe kyphoscoliosis, which had been treated with extensive spinal surgery. We used ultrasound guidance to facilitate successful spinal analgesia and anesthesia. CLINICAL FEATURES: A 27-yr-old woman, with a history of poliomyelitis and moderate restrictive lung disease secondary to kyphoscoliosis, presented at 38 weeks gestation for elective CD because of cephalopelvic disproportion...

We present a case of amniotic band syndrome diagnosed with two-dimensional (2D), three-dimensional (3D), and four-dimensional (4D) sonography at 14 weeks and 5 days of gestation. Conventional 2D sonography revealed multiple amniotic bands, fetal acrania, deformed hand, gastroschisis, kyphoscoliosis, and club foot. 3D and 4D sonography revealed multiple amniotic bands swinging in the amniotic cavity with 1 of them attached to the fetal head, and the severe deformity of the fetal face.

We report a case of body stalk anomaly which was diagnosed at 12 weeks of gestation on a first trimester scan. The fetus displayed multiple anomalies characteristic of body stalk syndrome including abdominal wall defect, kyphoscoliosis, deformities of the lower limbs and a single umbilical artery. Body stalk anomaly is a rare congenital anomaly with a series of similar clinical manifestations and poor prognosis. The first trimester scan can estimate the risk for chromosomal abnormalities and may also reveal major congenital abnormalities...

We describe a case of omphalocele-exstrophy-imperforate anus-spinal defects (OEIS) diagnosed at 13 weeks' gestation by ultrasound detection of a large multilocular ventral wall mass into which inserted the umbilical cord, and thoracic hemivertebrae with kyphoscoliosis. Subsequent sonographic examinations at 16 and 19 weeks revealed evolution of the ventral wall mass into a classic omphalocele. Clinicians should recognize the significance of a large multicystic ventral wall mass, which should lead to a search for other anomalies suggestive of OEIS complex...

We describe the anaesthetic management of a 21-year-old lady with central core disease for elective Caesarean section. Central core disease is characterised by muscle weakness, skeletal deformities and susceptibility to malignant hyperthermia. Total intravenous anaesthesia was used because of the combination of potential malignant hyperthermia, severe kyphoscoliosis and extensive spinal scarring. The authors believe there is no previous report of propofol and remifentanil being used in these circumstances. A short review of central core disease and its anaesthetic implications is provided...

The commonest cause of neurogenic bladder in children is myelomeningocele. Survival of children is much improved in the Western world, but by 35 years old, about 50% will have died. In adults, the commonest causes of death are lung and heart diseases. All physical aspects deteriorate with age, especially in those with thoracic lesions. Those who walk in childhood have a 20-50% chance of becoming wheelchair dependent as adults. Immobility, poor respiratory reserve, obesity, latex allergy and worsening kyphoscoliosis contribute to the increased risks of surgery...