The Orthopaedic Management of Arthrogryposis Multiplex Congenita

Bosse, Harold J. P. van; Zlotolow, Dan A.

doi:10.55275/jposna-2021-277

Cited by 4 publications

(7 citation statements)

References 63 publications

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“…Arthrogryposis affects 1: 3000 live births [ 1 ]. Arthrogryposis is characterized by normal neurological examination and the patients have normal intelligence [ 2 ]. Patients can present with upper and lower extremity contractures [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…Arthrogryposis is characterized by normal neurological examination and the patients have normal intelligence [ 2 ]. Patients can present with upper and lower extremity contractures [ 2 ]. Clubfoot is considered one of the most common presentation pictures [ 2 ].…”

Section: Introductionmentioning

confidence: 99%

“…Patients can present with upper and lower extremity contractures [ 2 ]. Clubfoot is considered one of the most common presentation pictures [ 2 ]. Hip involvement is seen in 80% of arthrogryposis patients; either in the form of hip contracture or hip dislocation [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

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Open Reduction at 15 Months of Left Hip Dislocation in a Male Infant Diagnosed with Arthrogryposis

et al. 2022

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Patient: Male, 4-week-old Final Diagnosis: Arthrogryposis Symptoms: Limited hip motion Medication:— Clinical Procedure: Open reduction of hip and femoral shortening Specialty: Orthopedics and Traumatology Objective: Unusual or unexpected effect of treatment Background: Arthrogryposis is a congenital condition of multiple contractures of joints associated with hip dislocation. The outcome of open reduction of hip dislocation in arthrogryposis patients is debatable. Open reduction of arthrogryposis is challenging for shallow acetabulum and extensive adhesions and fibrosis. For this reason, a careful extensive release must be carried out to achieve the open reduction of the hip in arthrogryposis patients. The literature lacks surgical recommendations for open reduction of the hip in arthrogryposis patients and how to deal with cases of the extruded bone segment during open reduction. Case Report: The patient presented in the first few weeks of life with bilateral clubfoot and left hip dislocation. Clinical diagnosis of arthrogryposis was made after referral to a genetics specialist. The hip was clinically irreducible. The patient underwent open reduction and femoral shortening using the Smith Peterson approach at the age of 15 months, with accidental extrusion of the proximal femur, which was retained immediately. The clinical outcome showed a painless, good range of motion. Radiographically, features of avascular necrosis and healed osteotomy site were evident. Conclusions: A difficult hip reduction was expected in this arthrogryposis patient, which required careful dissection of surrounding fibrosis and appropriate femoral shortening. Careful dissection should be carried out during open reduction to avoid jeopardization of femoral head vascularity or even complete devitalization of the proximal femur.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Open Reduction at 15 Months of Left Hip Dislocation in a Male Infant Diagnosed with Arthrogryposis

et al. 2022

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“…The most common etiology is amyoplasia, an abnormality of muscle development 1,3 . Amyoplasia involves all 4 extremities in 46% to 84% of patients, whereas isolated upper extremity involvement presents in 5% to 11% 3 . The goal of treatment is optimization of inde-pendent function 4 .…”

mentioning

confidence: 99%

“…Arthrogryposis is a rare condition that describes congenital contractures of the limbs 1,2 . The most common etiology is amyoplasia, an abnormality of muscle development 1,3 . Amyoplasia involves all 4 extremities in 46% to 84% of patients, whereas isolated upper extremity involvement presents in 5% to 11% 3 .…”

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confidence: 99%

Preoperative Evaluation for Free Functional Muscle Transfer in Upper Extremity Amyoplasia

Sullivan,

Vilkki,

Raja Sabapathy

et al. 2024

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Case: A multicenter series of 3 patients with amyoplasia of the upper extremity were indicated for gracilis free functional muscle transfer (FFMT) to restore elbow flexion and found to have an absent gracilis. A final case is discussed detailing standardized evaluation with ultrasound to confirm gracilis before surgical intervention. Conclusion: In amyoplasia, the gracilis muscle may be absent or have fatty infiltration, making this donor muscle inadequate. Preoperative ultrasound to determine the presence of the gracilis is noninvasive and recommended in patients with amyoplasia of the upper extremity being considered for FFMT.

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Do Not Forget the Spine MRI in Children With Arthrogryposis Multiplex Congenita: High Prevalence of Tethered Spinal Cord and Preliminary Clinical Findings Following Detethering

Nugraha,

Hariharan,

Huser

et al. 2024

Journal of Pediatric Orthopaedics

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Background: Arthrogryposis multiplex congenita (AMC) encompasses congenital conditions with joint contractures in 2 or more joints. Patients with AMC may have scoliosis and neural axis malformations. The study aimed to determine the prevalence of tethered spinal cord (TSC), characterized by a low-lying conus medullaris, and secondarily, present preliminary findings following surgical untethering in children with AMC. Methods: Patients 18 years of age and younger with a diagnosis of AMC and a spine MRI were identified. The presence of a TSC was defined as a low-lying conus with termination at or below the lower third of the L2 vertebral body. A pediatric neurosurgeon and a pediatric orthopaedic surgeon independently reviewed MRIs. The medical records of patients with AMC who underwent untethering were examined to evaluate preoperative and postoperative clinical findings. The prevalence of TSC in our AMC patients was compared with published normative data using χ2 analysis. Results: Forty-two of 105 AMC patients (40%) had TSCs. There was a greater percentage of patients with AMC and a TSC compared with the unaffected population (P<0.0001). Sixteen patients underwent detethering through filum terminale sectioning. Nine patients had preoperative neurological deficits in addition to their AMC. There were no postoperative complications. All patients had improvement in their bowel and bladder symptoms within 3 months after their detethering procedure. Conclusions: TSC is more prevalent in the pediatric population with AMC compared with those without AMC. MRI is recommended for all patients with AMC due to its high prevalence. Although not a benign procedure, surgical detethering may have potential benefits for children with AMC. Level of Evidence: Level IV—therapeutic study.

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The Orthopaedic Management of Arthrogryposis Multiplex Congenita

Cited by 4 publications

References 63 publications

Open Reduction at 15 Months of Left Hip Dislocation in a Male Infant Diagnosed with Arthrogryposis

Open Reduction at 15 Months of Left Hip Dislocation in a Male Infant Diagnosed with Arthrogryposis

Preoperative Evaluation for Free Functional Muscle Transfer in Upper Extremity Amyoplasia

Do Not Forget the Spine MRI in Children With Arthrogryposis Multiplex Congenita: High Prevalence of Tethered Spinal Cord and Preliminary Clinical Findings Following Detethering

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