Management of Thoracic Insufficiency Syndrome in Patients with Jarcho-Levin Syndrome Using VEPTRs (Vertical Expandable Prosthetic Titanium Ribs)

Karlin, Joshua G.; Roth, Megan K.; Patil, Vishwas; Cordell, Davin; Trevino, Hope; Simmons, James W.; Campbell, Robert M.; Joshi, Ajeya P.

doi:10.2106/jbjs.m.00185

Cited by 31 publications

(22 citation statements)

References 34 publications

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“…These results are consistent with findings after VEPTR treatment of other spinal and chest wall deformities, including SCD 1 and congenital scoliosis 9 , 10 as well as Jeune syndrome 11 , myelomeningocele, and cerebrocostomandibular syndrome (all unpublished data). In these patients, thoracic growth, improved respiratory functioning, and improved survivability are also positive outcomes of treatment.…”

Section: Resultssupporting

confidence: 90%

“…VEPTR treatment in patients with STD is associated with increased thoracic spine height and reduced thoracic width-to-height ratio, suggesting a greater gain in height than in width 1 ( Video 2 ). In addition, these patients display a decreased requirement for ventilation support, representing an important improvement in quality of life.…”

Section: Resultsmentioning

confidence: 99%

“…Although surgical treatment of spondylothoracic dysplasia (STD) is controversial, we have found that an expansion thoracoplasty using a Vertical Expandable Prosthetic Titanium Rib (VEPTR; DePuy Synthes Spine, Raynham, Massachusetts) results in favorable outcomes, including 100% survivability (at an average follow-up of 6.2 years), increased thoracic spinal length, and decreased requirements for ventilation support 1 .…”

Section: Introductionmentioning

confidence: 99%

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Expansion Thoracoplasty for Thoracic Insufficiency Syndrome Associated with Jarcho-Levin Syndrome

Joshi

Roth

Simmons

et al. 2015

JBJS Essential Surgical Techniques

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OverviewIntroductionAlthough surgical treatment of spondylothoracic dysplasia (STD) is controversial, we have found that an expansion thoracoplasty using a Vertical Expandable Prosthetic Titanium Rib (VEPTR; DePuy Synthes) results in favorable outcomes, including 100% survivability (at an average follow-up of 6.2 years), increased thoracic spinal length, and decreased requirements for ventilation support.Step 1: Preoperative PreparationMake anteroposterior and lateral radiographs of the spine.Step 2: Position the Patient for the ProcedureThe patient is placed in the prone position.Step 3: The IncisionA curvilinear skin incision is made, starting proximally between the spine and the medial edge of the scapula.Step 4: The OsteotomyPerform the v-osteotomy.Step 5: Placement of the VEPTR DeviceA number-4 VEPTR-I device is wedged in, starting laterally within the osteotomy sites, wedging the osteotomies apart, distracting the superior ribs proximally and the inferior ribs distally, lengthening the hemithorax, and stopping approximately at the posterior axillary line, when there is maximum stress on the superior and inferior ribs, to avoid fracture, and the lamina spreaders are then removed.Step 6: Wound ClosureInsert drains and local anesthetic catheters and close the wound.Step 7: Expansion and Replacement ProceduresLengthen the devices with the standard VEPTR technique of limited 3-cm incisions every three to six months.ResultsVEPTR treatment in patients with STD is associated with increased thoracic spine height and reduced thoracic width-to-height ratio, suggesting a greater gain in height than in width.What to Watch ForIndicationsContraindicationsPitfalls & Challenges

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Section: Resultssupporting

confidence: 90%

Section: Resultsmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Expansion Thoracoplasty for Thoracic Insufficiency Syndrome Associated with Jarcho-Levin Syndrome

Joshi

Roth

Simmons

et al. 2015

JBJS Essential Surgical Techniques

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“…In short, one or more vertically oriented distracting devices are attached superiorly to the ribs and inferiorly to the ribs, spine, or pelvis and serially lengthened as the child grows. The technique has been described in detail by Campbell et al [ 23 , 24 ] and multiple studies have demonstrated improved clinical respiratory function in SCD patients [ 24 , 25 , 26 ]. In a recent study by Ramirez et al of VEPTR treatments performed in 20 SCD patients, spinal deformity did not progress in 70% of the patients and improvements in assisted ventilation rates were observed in 5 patients; there was no change in 14 patients and no deaths over the mean 4-year follow-up [ 26 ].…”

Section: Discussionmentioning

confidence: 99%

Spondylocostal Dysostosis: A Literature Review and Case Report with Long-Term Follow-Up of a Conservatively Managed Patient

Southam

Schumaier

Crawford

2018

Case Reports in Orthopedics

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Introduction Patients with spondylocostal dysostosis (SCD) have congenital spine and rib deformities associated with frequently severe thoracic insufficiency and respiratory compromise. The literature is largely composed of case reports and small cohorts, and there is little information regarding adults with this condition. In this report, we describe the natural history of a conservatively treated patient and include quality-of-life issues such as childbearing, athletic participation, and occupational selection. Case Presentation We present a patient with SCD who was conservatively treated by a single physician from birth for 31 years. Our patient was capable of a reasonably good quality of life through adulthood, including participation in gymnastics and employment. At age 18, she became pregnant and subsequently terminated the pregnancy due to obstetrical concerns for compromised respiration. She has had intermittent respiratory complaints and occasionally experiences dyspnea with exertion, but this only has limited her during certain activities in the past three years. Currently, she takes naproxen for chronic back pain with periodic exacerbations. Discussion Other cases in the literature have described adult SCD patients who have received nonoperative treatment and achieved a wide range of functional outcomes. This provides some limited evidence to suggest that select patients with SCD may be treated conservatively and achieve a reasonable quality of life. However, close clinical follow-up with these patients is recommended, particularly early on, considering the high rates of infant morbidity and mortality. Chest physiotherapy and early pulmonary care have been associated with favorable outcomes in infancy. Surgery to increase thoracic volume and correct scoliosis has been shown in some cases to improve respiratory function. Treatment depends on the degree of thoracic insufficiency and quality of life. The natural history of SCD remains largely unknown, but some patients are capable of relatively favorable life spans, employment, and participation in athletics.

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“…Algunos autores como Mayer, Campbell y Karlin recomiendan cirugía. Esta cirugía consiste en una osteotomía en forma de "V" en la fusión de las costillas para alargar cada hemitórax y la implantación de las VEPTR costilla a costilla 8,12,13 (Figura 2.C). Por otro lado, A B c autores como Ramírez y Cornier, no recomiendan intervención quirúrgica alguna.…”

Section: Tratamientounclassified

Deformidad de la caja torácica

Ramirez-Lluch

Acevedo-Echevarría

2018

Rev Chil Cir

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Chest wall deformities are divided as an abnormal development during the growth or those secondary to a congenital malformation. The developmental type is the most common: pectus excavatum or pectus carinatum. The less common are the congenital types of chest wall abnormalities: Poland's syndrome, Jeune's syndrome, espondylothoracic dysplasia, espondylocostal dysplasia and defects of the ribs or sternum. The congenital type usually affects the relationship between the spine, rib cage and the lungs. Therefore, many of these patients will develop a progressive respiratory disturbance of restrictive type known as Thoracic Insufficiency Syndrome. Thoracic insufficiency syndrome is defining as a deficiency of the rib cage to maintain a normal respiration and to sustain the physiological growth of the lungs. In this article will discuss several conditions that will affect the development and function of the chest wall.

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Management of Thoracic Insufficiency Syndrome in Patients with Jarcho-Levin Syndrome Using VEPTRs (Vertical Expandable Prosthetic Titanium Ribs)

Abstract: VEPTR treatment improved thoracic symmetry, controlled spinal deformity, and was associated with improved clinical respiratory function.

Cited by 31 publications

References 34 publications

Expansion Thoracoplasty for Thoracic Insufficiency Syndrome Associated with Jarcho-Levin Syndrome

Expansion Thoracoplasty for Thoracic Insufficiency Syndrome Associated with Jarcho-Levin Syndrome

Spondylocostal Dysostosis: A Literature Review and Case Report with Long-Term Follow-Up of a Conservatively Managed Patient

Deformidad de la caja torácica

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