Weight Gain Following Vertical Expandable Prosthetic Titanium Ribs Surgery in Children With Thoracic Insufficiency Syndrome

Skaggs, David L.; Sankar, Wudbhav N.; Albrektson, Josh; Wren, Tishya A. L.; Campbell, Robert M.

doi:10.1097/brs.0b013e3181bd09f5

Cited by 40 publications

(21 citation statements)

References 6 publications

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“…Several studies used weight gain as an indirect marker of improved pulmonary function and found that up to 50% of patients with EOS demonstrated a mean 24-to 26-percentagepoint improvement after VEPTR or growing rod treatment. 58,59 Overnight polysomnography in children with EOS demonstrates an increased Apnea-Hypopnea Index 8…”

Section: Pulmonary Outcomes Of Recent Eos Treatmentmentioning

confidence: 99%

Early-Onset Scoliosis: A Review of History, Current Treatment, and Future Directions

et al. 2016

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Early-onset scoliosis (EOS) is defined as curvature of the spine in children >10° with onset before age 10 years. Young children with EOS are at risk for impaired pulmonary function because of the high risk of progressive spinal deformity and thoracic constraints during a critical time of lung development. The treatment of EOS is very challenging because the population is inhomogeneous, often medically complex, and often needs multiple surgeries. In the past, early spinal fusion was performed in children with severe progressive EOS, which corrected scoliosis but limited spine and thoracic growth and resulted in poor pulmonary outcomes. The current goal in treatment of EOS is to maximize growth of the spine and thorax by controlling the spinal deformity, with the aim of promoting normal lung development and pulmonary function. Bracing and casting may improve on the natural history of progression of spinal deformity and are often used to delay surgical intervention or in some cases obviate surgery. Recent advances in surgical implants and techniques have led to the development of growth-friendly implants, which have replaced early spine fusion as the surgical treatment of choice. Treatment with growth-friendly implants usually requires multiple surgeries and is associated with frequent complications. However, growth-friendly spine surgery has been shown to correct spinal deformity while allowing growth of the spine and subsequently lung growth.

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Section: Pulmonary Outcomes Of Recent Eos Treatmentmentioning

confidence: 99%

Early-Onset Scoliosis: A Review of History, Current Treatment, and Future Directions

et al. 2016

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“…In severe respiratory insufficiency, even oral nutritional intake may represent a challenge because the patient may not tolerate the small breathing pause due to eating [18][19][20]. Our patient was described as having major difficulties in gaining weight all his life and had suffered from a poor appetite for years due to abdominal pain immediately after oral food intake, which worsened his nutritional status.…”

Section: Discussionmentioning

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

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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.

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“…Vitale and co-workers [9] noted a very poor quality of life questionnaire scores for untreated children with thoracic insufficiency syndrome. Skaggs et al [10] found that in 76 patients with TIS, 79 % were under the 5th percentile for weight, and after VEPTR surgery 40 % improved with weight gain. VEPTR appears to make a positive difference for children with thoracic insufficiency syndrome clinically, but the lack of an objective lab test or imaging technique to measure thoracic insufficiency syndrome has made it difficult for orthopedists to fully understand the effect of VEPTR treatment on TIS.…”

Section: Keyword Veptrmentioning

confidence: 99%

“…These volumes were also compared to the retrospective spontaneously breathing pediatric normative CT scan lung volumes reported by Gollogly et al [21], and there was no increase in the age-corrected volumes. The authors also found nearly normal quality of life both before and after VEPTR treatment based on a modified SRS-22 instrument, although a validated Child Health Care Questionnaire study of children by Vitale et al [10] in 2008 showed a marked decrease in quality of life for children with untreated thoracic insufficiency syndrome. The authors' final conclusion was that ''pulmonary function, lung volume, and patient subjective assessments did not increase dramatically after VEPTR placement'', which apparently was their working hypothesis, but the marked heterogeneity of their patient population and their suboptimal age of intervention may have influenced pulmonary outcomes in an unpredictable fashion, and their choice of a quality of life instrument may be unsuited for thoracic insufficiency syndrome.…”

Section: S114mentioning

confidence: 99%

VEPTR: past experience and the future of VEPTR principles

Campbell

2013

Eur Spine J

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145

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Purpose This review summarizes the experience with the vertical expandable prosthetic titanium rib (VEPTR) device, which enables five new procedures to treat complex spine and chest wall abnormalities in pediatric patients, which cause thoracic insufficiency syndrome, the inability of the thorax to support normal respiration or lung growth. Methods The literature on VEPTR was reviewed and discussed by the author, the inventor of the VEPTR. Results The central VEPTR treatment principle is to correct volume depletion deformity of the thorax, and maintain the correction until skeletal maturity, at which time procedures such as spinal fusion can be considered. For individual cases of complex deformity, VEPTR strategies can differ remarkably. The goal of VEPTR surgery is to pursue the surgical strategy that provides the largest, most symmetrical, most functional thorax that can grow as normally as possible. Assessment of these results is difficult, since natural history of VEPTR-treated diseases are not clearly known and no current imaging test can measure thoracic insufficiency syndrome, but dynamic lung MRI have promise for the future in better defining this potentially lethal condition. Conclusion VEPTR and its principles of use have become an important first step toward improving the quality of life and longevity of children with thoracic insufficiency syndrome, but much work remains to advance both its design and its use. Keyword VEPTRThe vertical expandable prosthetic titanium rib (VEPTR) was based on a crude Steinmann pin chest wall prosthesis that was placed in an 8-month-old ventilator dependent infant in 1987 who was dying from respiratory insufficiency due to severe rib agenesis and scoliosis. The pins were placed longitudinally to provide a stable perpendicular load orientation for the attachment of the pins to the vestigial proximal and distal ribs, but also in the hope of stabilizing the curve in case the infant did manage to survive. To everyone's surprise, post-operatively the infant was weaned off the ventilator in days, and then oxygen weeks later, going on to grow and thrive. Even the scoliosis improved, likely through rib distraction correcting it indirectly. Once the euphoria wore off, though, the reality of the new long-term problems for the child became apparent. As he became older, his chest and spine would grow, but the non-expandable Steinmann Pin construct would soon became a tether to both spinal growth and the underlying concave lung. Two apparent choices were obvious to continue the clinical success of the chest wall construct. The Steinmann pin prosthesis could be completely changed out periodically through a full thoracotomy with probable skin slough, infection, and eventual failure, or a true chest wall prosthesis could be developed that was easy to implant, providing a stable chest wall and curve control, and would be expandable through periodic out-patient surgery through a limited incisions, which would likely decrease risk of infection and skin slough. The latter course wa...

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Weight Gain Following Vertical Expandable Prosthetic Titanium Ribs Surgery in Children With Thoracic Insufficiency Syndrome

Cited by 40 publications

References 6 publications

Early-Onset Scoliosis: A Review of History, Current Treatment, and Future Directions

Early-Onset Scoliosis: A Review of History, Current Treatment, and Future Directions

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

VEPTR: past experience and the future of VEPTR principles

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