Chest Reconstruction in Thoracic Dystrophy

Barnes, N. D.; Hull, D.; Milner, Anthony; Waterston, David

doi:10.1136/adc.46.250.833

Cited by 54 publications

(15 citation statements)

References 4 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…At least two other attempts to improve similar conditions have been described in the literature. The methods described included releasing several ribs at the costo-chondral junction and using a methyl methacrylate strut, but these attempts were unfortunately unsuccessful [7,9]. Lateral thoracic expansion has also been described for the treatment of Jeune syndrome [8], but due to initial unilateral fused ribs and later bilateral posterior wall fusions, we concluded that the procedure was not well suited in our case.…”

Section: Discussionmentioning

confidence: 65%

“…In severe conditions, thoracic insufficiency syndrome (TIS) will ultimately develop [4]. The literature contains several suggestions for treatment of SRS [5][6][7][8], but the outcomes are divergent, with reports of early deaths despite surgical interventions. The most commonly used surgical technique is a vertical expandable prosthetic titanium rib (VEPTR, DePuySynthes, Switzerland), but a limitation of this treatment is that the posterior and lateral thoracic cage tends to auto-fuse after long-term use.…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Sagittal Distraction of the Sternum in a Juvenile Male with Severe Short Rib Syndrome and Thoracic Insufficiency Syndrome - A Case Report

Kibsgård¹

2017

MOJOR

View full text Add to dashboard Cite

Background: This report describes a case of thoracic insufficiency/short rib syndrome treated with sagittal distraction of the sternum in a juvenile male.Methods: An acute increase in the thoracic cage volume was achieved in an 8-year-old male with thoracic insufficiency syndrome by sternum splitting and distraction of 3 centimeters. He had previously been treated with bilateral opening wedge and thoracic vertical expandable prosthetic titanium rib instruments. At 8 years, his respiration was rapidly deteriorating, and posterior revision surgery with resection of the posterior wall at this stage had no further positive influence on his respiratory function. Two years later, a second distraction of the sternum and acute distraction were performed as part of the thoracic expansion procedure. Results:The procedure resulted in an increase in his respiratory functions, and his pulmonary artery parameters decreased to normal values. His clinical condition improved, and after surgery, he could ride a bicycle to school and even participate in sports, such as cross-country skiing and soccer. Conclusion:Short rib syndrome presenting with respiratory failure in a neonate is potentially fatal without surgical intervention to expand the thoracic cage. Our patient had severe thoracic insufficiency syndrome and experienced clinical improvement from the procedure. Sternum distraction has been previously used in neonates, but to the best of our knowledge, this is the first distraction of the sternum addressing short rib syndrome in a juvenile.

show abstract

Section: Discussionmentioning

confidence: 65%

Section: Introductionmentioning

confidence: 99%

Sagittal Distraction of the Sternum in a Juvenile Male with Severe Short Rib Syndrome and Thoracic Insufficiency Syndrome - A Case Report

Kibsgård¹

2017

MOJOR

View full text Add to dashboard Cite

show abstract

“…Two previous attempts to improve this condition have been described in the literature and included releasing a number of ribs at the costochondral junction, and using a methylmethacrylate strut but these were unsuccessful. 4,5 Given the young age of our patient, he was 3 months old (only 2 months old when corrected for prematurity), lateral thoracic expansion or insertion of vertical expansion prosthetic titanium ribs were not feasible options. Lateral thoracic expansion has been described in the treatment of Jeune syndrome 12 but is not recommended in children <1 year of age.…”

Section: Discussionmentioning

confidence: 95%

“…4 The potential for normal lung development and growth has led to several surgical techniques for chest wall expansion including resection of costochondral cartilages, sternal splitting with bone graft interposition, and lateral thoracic expansion. [4][5][6] To date, these techniques have had limited success in very young children, especially those <1 year of age. The Modular Internal Distraction System (MID, Stryker Leibinger Germany) has been used in a number of craniofacial disorders.…”

mentioning

confidence: 98%

Sternoplasty and Rib Distraction in Neonatal Jeune Syndrome

Conroy

Eustace²,

McCormack³

2010

Journal of Pediatric Orthopaedics

View full text Add to dashboard Cite

show abstract

“…Therefore, it is theorized that if the thorax is expanded, further lung growth can be expected. Various surgical techniques have been suggested for thoracic expansion in patients with JATD including sternal splitting, posterolateral rib cage expansion by placing plates, and vertical expandable prosthetic titanium rib (VEPTER) opening thoracotomy [12][13][14]. The primary goal of these procedures is to increase the thoracic volume and allow for lung expansion and growth.…”

Section: Discussionmentioning

confidence: 99%

Anesthetic Care During Lateral Thoracic Expansion for Asphyxiating Thoracic Dystrophy

2016

View full text Add to dashboard Cite

Jeune syndrome or asphyxiating thoracic dystrophy (JATD) is a rare autosomal recessive disorder with an estimated incidence of 1 in 100,000 to 130,000 live births. The typical phenotype of JATD is osteochondrodysplasia (narrow thorax and shorted ribs) in association with short limb dwarfism. The thoracic dysplasia results in respiratory insufficiency, usually requiring tracheostomy and chronic mechanical ventilation beginning in infancy and childhood. Lateral thoracic expansion is one surgical procedure that has been described in an attempt to enlarge the thoracic cage and alleviate the progressive respiratory failure. In addition to respiratory involvement, patients with JATD may have other co-morbid conditions involving the kidneys, pancreas, liver, and eyes. Given the underlying thoracic dysplasia and other associated comorbid conditions, the intraoperative care of such patients can be challenging. We present two patients with JATD who presented for lateral thoracic expansion. Previous reports of the intraoperative care of such patients are reviewed and options for anesthetic care are discussed.

show abstract

Chest Reconstruction in Thoracic Dystrophy

Cited by 54 publications

References 4 publications

Sagittal Distraction of the Sternum in a Juvenile Male with Severe Short Rib Syndrome and Thoracic Insufficiency Syndrome - A Case Report

Sagittal Distraction of the Sternum in a Juvenile Male with Severe Short Rib Syndrome and Thoracic Insufficiency Syndrome - A Case Report

Sternoplasty and Rib Distraction in Neonatal Jeune Syndrome

Anesthetic Care During Lateral Thoracic Expansion for Asphyxiating Thoracic Dystrophy

Contact Info

Product

Resources

About