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Evolution of Experience and Practise in Two Nations
Published in Alaaeldin (Alaa) Azmi Ahmad, Aakash Agarwal, Early-Onset Scoliosis, 2021
During my training in Cardiff, I had developed the first web-based scoliosis registry and database for the spinal unit at University Hospital of Wales (UHW) with help from Dr John Howes, Consultant Spine Surgeon. He had invited Dr Robert Campbell from the United States who had developed VEPTR (vertical expandable titanium prosthetic rods). He demonstrated the technique of application in children with thoracic insufficiency syndrome [23,24] [Figure 9f.4]. This technique indirectly fixes scoliosis without fusion. VEPTR treatment has demonstrated continued spinal growth with serial expansion improving the coronal curves [25]. Over the next few years, a hybrid technique using growing rods with VEPTR was introduced to reduce the complications. The hybrid technique incorporates the VEPTR concept by using ribs as proximal anchor sites but also uses pedicle screws for distal anchors [26,27].
Hybrid Approach to Repair of Acquired Thoracic Dystrophy in an Adult Patient after Failed Childhood Ravitch Procedure
Published in Wickii T. Vigneswaran, Thoracic Surgery, 2019
Acquired thoracic dystrophy (ATD), also sometimes called asphyxiating thoracic dystrophy or thoracic insufficiency syndrome, is an uncommon condition in which the growth of the thoracic cage is limited, leading to varying degrees of restriction in cardiopulmonary function [1]. This condition is most commonly associated with prior open repair of congenital pectus excavatum at a young age, during which extensive resections of costal cartilages were performed. Most patients with ATD require correction before adulthood, and only a small fraction present as adults. Management of ATD in adults is extremely challenging with limited evidence available on how best to correct the deformity and reconstruct the chest wall in these patients.
Congenital thoracic deformities
Published in Prem Puri, Newborn Surgery, 2017
Konstantinos Papadakis, Robert C. Shamberger
Spondylothoracic dysplasia is an autosomal recessive deformity characterized by short-trunk dwarfism associated with multiple vertebral and rib malformations.70 The ribs have a crab-like appearance (Figure 36.7). Death occurs early in infancy from respiratory failure and pneumonia. Patients have multiple alternating hemivertebrae, which affect most of the thoracic and lumbar spine. The ossification centers rarely cross the midline. Multiple posterior fusions of the ribs as well as remarkable shortening of the thoracic spine result in a crab-like radiographic appearance of the chest. One-third of the patients with this syndrome have associated malformations including congenital heart disease and renal anomalies. Its occurrence has been reported primarily in Puerto Rican families (15 out of 18 cases).71 Bone formation is normal in these patients. Successful prenatal diagnosis can be established by sonographic examination.72 Thoracic deformity is secondary to the spinal anomaly, which results in close posterior approximation of the origin of the ribs. The VEPTR procedure has been successfully performed in the neonatal period for this highly lethal syndrome.73 Spondylothoracic dysplasia has a mortality rate approaching 50% from respiratory complications due to thoracic insufficiency syndrome. Most infants with this syndrome succumb before 15 months of age.74 In spite of severe restrictive respiratory disease, adult survivors of spondylothoracic dysplasia appear to do well clinically for unknown reasons.75
Features of Marfan syndrome not listed in the Ghent nosology – the dark side of the disease
Published in Expert Review of Cardiovascular Therapy, 2019
Yskert von Kodolitsch, Anthony Demolder, Evaldas Girdauskas, Harald Kaemmerer, Katharina Kornhuber, Laura Muino Mosquera, Shaine Morris, Enid Neptune, Reed Pyeritz, Svend Rand-Hendriksen, Alexander Rahman, Nina Riise, Leema Robert, Ingmar Staufenbiel, Katalin Szöcs, Thy Thy Vanem, Stephan J. Linke, Marina Vogler, Anji Yetman, Julie De Backer
Chest wall deformities such as scoliosis and pectus deformities are common with Marfan Syndrome often resulting in restrictive lung disease [172]. With severe restriction, thoracic insufficiency syndrome (TIS) develops punctuated by impaired ventilation and gas exchange [173]. In pediatric Marfan syndrome, lung growth is further compromised by these disorders directly impacting peak lung function attainment and accelerating the onset of TIS. Scoliosis is the most common spinal deformity in Marfan syndrome afflicting more than 60% of patients [174]. Up to one-half of affected persons require surgery for progression and/or associated respiratory compromise [175]. Pectus deformities, namely pectus excavatum and to a lesser degree pectus carinatum, are common congenital disorders that are highly represented in Marfan syndrome [176]. Most pectus deformities are mild and do not produce marked alterations in lung function. However, more severe pectus defects, especially when coupled to scoliosis, can cause clinically consequential restrictive disease [177]. Less invasive and more customizable surgical options (e.g., vertical expandable prosthetic titanium ribs (VEPTR) magnetic rods, or Nuss bars) for both scoliosis and pectus deformities are now employed early in disease development leading to fewer complications and lung function stabilization in selected patients [178–182]. As yet, no long-term studies documenting stability or improvement in lung function with these newer interventions in the Marfan population are available.
A review of the hemivertebrae and hemivertebra resection
Published in British Journal of Neurosurgery, 2022
Beixi Bao, Hui Yan, Jiaguang Tang
The future of congenital scoliosis lies in the early detection of the disease and preventing its consequences, particularly thoracic insufficiency syndrome. A three-dimensional understanding of deformity and the chest cavity will assist clinicians in achieving better goals. We also need to look at the genetic aspect of congenital scoliosis to understand how to prevent it. In the future, we may also be able to treat the vertebral anomaly during fetal life and avoid the problematic toll and consequences that the disease has on its patients, despite receiving the best treatment efforts.
Anaesthetic considerations in a child with fibrodysplasia ossificans progressiva
Published in Southern African Journal of Anaesthesia and Analgesia, 2019
The median age of survival of patients with FOP is 45 years and the two leading causes of death are pulmonary infections and right-sided heart failure secondary to thoracic insufficiency syndrome.4 Campbell et al.15 define thoracic insufficiency syndrome as ‘the inability of the thorax to support normal respiration or lung growth’.