A thoracic expansion technique for Jeune's asphyxiating thoracic dystrophy

Todd, David W.; Tinguely, Stephen J.; Norberg, William J.

doi:10.1016/s0022-3468(86)80073-2

Cited by 56 publications

(22 citation statements)

References 2 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…These attempts were either unsuccessful with the patient dying soon after operation, or temporarily successful but cumbersome. On the other hand, the procedure of Todd et al (1986), as applied to the girl we described, is simple and effective. Todd's patient, a 9-month-old girl, received a 2.5 cm-wide prosthesis, while our patient, a 15-month-old girl, received a 3.2 cm-wide prosthesis.…”

Section: Discussionmentioning

confidence: 98%

“…Surgical thoracic expansion was performed at 15 months of age according to the procedure of Todd et al (1986), when the patient was on mechanical ventilation for 4 weeks. Briefly, a sagittal section was made on the midline of the sternum, a 3.2 cm wide, methyl methacrylate prosthesis was placed between the sectioned halves, and fixed with wires (Fig.…”

Section: Case Reportmentioning

confidence: 99%

“…Ravitch for references up to 1977). Todd et al (1986) reported a procedure of sagittal sectioning between the sectioned sternal halves, successfully applied to a 9-month-old girl with a severe form of the disorder. We here describe application of the procedure to a 15-month-old girl with the disorder, together with a 2 year follow-up history.…”

Section: Introductionmentioning

confidence: 99%

“…

SummaryA 15-month-old girl under mechanical ventilation with asphyxiating thoracic dystrophy underwent surgical thoracic expansion according to the procedure of Todd et al (1986). Now aged 4 years, she is free from respiratory distress, is of normal intelligence, and leads an active life.

…”

mentioning

confidence: 99%

See 3 more Smart Citations

Asphyxiating thoracic dystrophy: Surgical correction and 2-year follow-up in a girl

et al. 1994

View full text Add to dashboard Cite

show abstract

Section: Discussionmentioning

confidence: 98%

Section: Case Reportmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…

…”

mentioning

confidence: 99%

See 2 more Smart Citations

Asphyxiating thoracic dystrophy: Surgical correction and 2-year follow-up in a girl

et al. 1994

View full text Add to dashboard Cite

show abstract

“…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. Several articles in the literature refer to rib and sternum procedures that increase the thoracic volume [5,[8][9][10][11], and a sternum osteotomy can be an option in extreme cases if other procedures have been attempted. To the best of our knowledge, all references in the literature regarding this procedure involve populations of siblings; therefore, we aim to describe a case in which sagittal distraction of the sternum was used successfully in a juvenile male.…”

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

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

Long-term survival in typical thanatophoric dysplasia type 1

et al. 1997

View full text Add to dashboard Cite

Thanatophoric dysplasia (TD), a severe skeletal dysplasia, is virtually always lethal neonatally, although a few previous reports have documented survival up to 4.75 years. We present a patient with survival beyond age 9 years and summarize his growth, development and medical history. The common Arg248Cys mutation in the extracellular region of fibroblast growth factor receptor 3 (FGFR3) was identified, eliminating the possibility that his long-term survival is attributable to an atypical mutation. This patient (and at least one other TD long-term survivor) have a rare skin disorder, acanthosis nigricans, which also occurs in Crouzon syndrome when caused by a FGFR3 mutation. Therefore, any molecular model of the origin of acanthosis nigricans secondary to FGFR3 mutations must account for the association of diverse mutations and these cutaneous effects.

show abstract

A thoracic expansion technique for Jeune's asphyxiating thoracic dystrophy

Cited by 56 publications

References 2 publications

Asphyxiating thoracic dystrophy: Surgical correction and 2-year follow-up in a girl

Asphyxiating thoracic dystrophy: Surgical correction and 2-year follow-up in a girl

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

Long-term survival in typical thanatophoric dysplasia type 1

Contact Info

Product

Resources

About