Atlantoaxial Rotary Subluxation in Marfan Syndrome

Atlantoaxial rotary subluxation (AARS) is a rare condition that may cause persistent torticollis if not treated appropriately. AARS is associated with ligamentous abnormalities, which may result from acquired or congenital disorders. We report the case of a paediatric patient with congenital Marfan syndrome and AARS due to a minor traumatic head injury. A 9-year-old boy with a known diagnosis of Marfan syndrome (and extensive family history) encountered a traumatic head injury that presented as torticollis with a typical “cock-robin” head and neck orientation. AARS was diagnosed through a head and neck CT scan. He underwent initial conservative treatment involving a muscle relaxant (diazepam) and Miami-J collar. This was followed up with manipulation under anaesthesia (MUA) and further cervical traction, which resolved the subluxation without more invasive treatment. To the best of our knowledge, AARS associated with Marfan syndrome has been rarely reported in literature. It is postulated that the ligamentous laxity associated with Marfan syndrome would increase the patient’s predisposition to AARS and more importantly, the propensity to require more invasive treatment (internal fixation). However, our patient unexpectedly responded well to conservative management, namely MUA and cervical traction. This illustrates that despite the increased ligamentous laxity in Marfan syndrome, it is still advisable to conservatively manage AARS before deciding to perform invasive internal fixation.International Journal of Human and Health Sciences Supplementary Issue-2: 2021 Page: S15

Grisel’s syndrome – case report

Introduction: By definition from the literature, Grisel’s syndrome is described as non-traumatic rotational atlantoaxial instability between C1 and C2 vertebrae. It can occur during an infection of a soft tissue in the cervicocranial region or after an operation in the ENT region. Because of the frequent occurrence after operations, we inclined to the definition which includes a traumatic subluxation as a cause of origin, and it’s not defined as non-traumatic only. The instability manifests itself with abnormal head posture that is called torticollis. Increased incidence in adolescence is more common because of a greater ligamentous laxity of the joint capsules, increased perfusion of antlantoaxial regions and longer alar ligaments. In this article, the case of a child with Grisel’s syndrome after adenotomy is described. The pathophysiology, symptomatology, diagnostic management and treatment are discussed. Keywords: Grisel’s syndrome – torticollis – atlantoaxial instability – adenotomy

Atlantoaxial subluxation in the pediatric patient: Case series and literature review

Objective: Atlantoaxial subluxation (AAS) occurs when there is misalignment of the atlantoaxial joint. Several etiologies confer increased risk of AAS in children, including neck trauma, inflammation, infection, or inherent ligamentous laxity of the cervical spine. Methods: A single-center, retrospective case review was performed. Thirty-four patients with an ICD-10 diagnosis of S13.1 were identified. Demographics and clinical data were reviewed for etiology, imaging techniques, treatment, and clinical outcome. Results: Out of thirty-four patients, twenty-two suffered cervical spine trauma, seven presented with Grisel’s Syndrome, four presented with ligamentous laxity, and one had an unrecognizable etiology. Most diagnoses of cervical spine subluxation and/or instability were detected on computerized tomography (CT), while radiography and magnetic resonance imaging (MRI) were largely performed for follow-up monitoring. Six patients underwent cervical spine fusion, five had halo traction, twelve wore a hard and/or soft collar without having surgery or halo traction, and eight were referred to physical therapy without other interventions. Conclusion: Pediatric patients with atlantoaxial subluxation may benefit from limited 3D CT scans of the upper cervical spine for accurate diagnosis. Conservative treatment with hard cervical collar and immobilization after reduction may be attempted, but halo traction and halo vest immobilization may be necessary. If non-operative treatment fails, cervical spine internal reduction and fixation may be necessary to maintain normal C1-C2 alignment.

Surgical management of the congenital dislocation of the knee and hip in children presented after six months of age

Purpose Congenital dislocation of the knee and hip is a rare congenital disorder. The specific aim of the study was to evaluate the clinical and radiological outcomes of the children with congenital dislocation of the knee and hip who presented after six months of age. Methods All the consecutive children with congenital dislocation of the knee and hip joints were retrospectively reviewed. We included cases that were treated after six months of age and followed up for a minimum of two years. Twenty-four children with congenital dislocation of the knee and hip (thirteen with ligamentous laxity, eleven children with stiff joints) were included. The knee was dislocated in 45 limbs; the hip was dislocated in 40 instances. The knee joint dislocation was treated with quadricepsplasty in all twenty-four children (45 knees). The hip dislocation (n = 32) was addressed with either closed reduction (n = 8) or open reduction of the hip (n = 24). Eight hip dislocations were not addressed. The outcome of the hip and knee was evaluated. Results The clinical and radiological outcomes were better in children with ligamentous laxity than without laxity. Twenty-two children were community walkers. An orthosis was needed in eight children. The frequency of spontaneous reduction of unreduced dislocation of the hip was noted in three children (5/8 hips). Conclusion Outcome in combined dislocation of knee and hip is good in most cases with surgical interventions. The outcome is better in children with ligamentous laxity. Spontaneous reduction of the dislocated hips might be achieved after gaining knee flexion following knee surgery for congenital the knee in a few cases.

Achieving Successful Outcomes of Hip Arthroscopy in the Setting of Generalized Ligamentous Laxity with Labral Preservation and Appropriate Capsular Management: A Matched Pair Controlled Study

Objectives: (1) To report minimum two-year PROs in patients with generalized ligamentous laxity (GLL) who underwent hip arthroscopy and (2) to compare clinical results to a matched-pair control group without GLL. Methods: Data from a prospectively collected database was retrospectively reviewed between August 2014 and December 2016. Patients were considered eligible if they received primary arthroscopic treatment for symptomatic labral tears and femoroacetabular impingement (FAI). Inclusion criteria included preoperative and minimum two-year follow-up scores for the following PROs: modified Harris Hip Score (mHHS), Non-Arthritic Hip Score (NAHS), Hip Outcome Score-Sports Specific Scale (HOS-SSS), and Visual Analogue Scale (VAS). Patients were excluded if they had preoperative Tönnis ≥ 2, ipsilateral hip condition, prior hip surgery, worker’s compensation status, or dysplasia. From the sample population, two groups were created: the GLL group (Beighton ≥ 4) and the control group (Beighton < 4). Patients were matched in a 1:2 ratio via propensity-score matching according to age, gender, body mass index (BMI), acetabular and femoral head Outerbridge grade, and preoperative lateral center-edge angle (LCEA). Patient Acceptable Symptomatic State (PASS) and Minimal Clinically Important Difference (MCID) for mHHS and HOS-SSS were calculated. PASS was also calculated for International Hip Outcome Tool-12 (iHOT-12) as well as MCID for VAS. Significant differences were noted if P < 0.05. Results: 95 patients with GLL were matched to 143 control patients. Age, gender, BMI, and follow-up times were not different (P > 0.05). Preoperative radiographic measurements demonstrated no difference. Intraoperative findings and procedures between groups were similar except for capsular treatment, with the GLL group receiving more plications (P = 0.004). Both groups reported similar baseline PROs and VAS. At minimum two-year follow-up both groups showed significant improvement in PROs and VAS (P < 0.001), furthermore, the postoperative PROs at minimum two-year follow-up showed no difference (P > 0.05) and the magnitude of improvement (delta value) was similar for mHHS (P = 0.93), NAHS (P = 0.809), HOS-SSS (0.398) and VAS (P = 0.824). Moreover, groups reached comparable rates of MCID and/or PASS for mHHS, HOS-SSS, iHOT-12, and VAS. Conclusions: Patients with GLL following hip arthroscopy for symptomatic FAI and labral tears, may expect favorable outcomes with appropriate labral and capsular management at minimum two-year follow-up. When compared to a pair-matched control group without GLL, results were comparable for mHHS, NAHS, HOS-SSS, VAS and reaching PASS and/or MCID for mHHS, HOS-SSS, iHOT-12 and VAS.

Achieving Successful Outcomes of Hip Arthroscopy in the Setting of Generalized Ligamentous Laxity With Labral Preservation and Appropriate Capsular Management: A Propensity Matched Controlled Study

Background: Association among generalized ligamentous laxity (GLL), hip microinstability, and patient-reported outcomes (PROs) after hip arthroscopy has yet to be completely established. Purposes: (1) To report minimum 2-year PROs in patients with GLL who underwent hip arthroscopy in the setting of symptomatic labral tears and femoroacetabular impingement syndrome and (2) to compare clinical results with a matched-pair control group without GLL. Study Design: Cohort study; Level of evidence, 3. Methods: Data from a prospectively collected database were retrospectively reviewed between August 2014 and December 2016. Patients were considered eligible if they received primary arthroscopic treatment for symptomatic labral tears and femoroacetabular impingement. Inclusion criteria included preoperative and minimum 2-year follow-up scores for the following PROs: modified Harris Hip Score (mHHS), Non-arthritic Hip Score (NAHS), and visual analog scale for pain (VAS). From the sample population, 2 groups were created: the GLL group (Beighton score ≥4) and the control group (Beighton score <4). Patients were matched in a 1:2 ratio via propensity score matching according to age, sex, body mass index, Tönnis grade, and preoperative lateral center-edge angle. Patient acceptable symptomatic state (PASS) and minimal clinically important difference (MCID) for mHHS, Hip Outcome Score–Sports Specific Scale (HOS-SSS), and International Hip Outcome Tool–12 (iHOT-12) were calculated. Results: A total of 57 patients with GLL were matched to 88 control patients. Age, sex, body mass index, and follow-up times were not different between groups ( P > .05). Preoperative radiographic measurements demonstrated no difference between groups. Intraoperative findings and procedures between groups were similar except for capsular treatment, with the GLL group receiving a greater percentage of capsular plications ( P = .04). At minimum 2-year follow-up, both groups showed significant improvement in PROs and VAS ( P < .001). Furthermore, the postoperative PROs at minimum 2-year follow-up and the magnitude of improvement (delta value) were similar between groups for mHHS, NAHS, HOS-SSS, and VAS ( P > .05). Moreover, groups reached comparable rates of MCID and PASS for mHHS, HOS-SSS, and iHOT-12. Conclusion: Patients with GLL after hip arthroscopy for symptomatic femoroacetabular impingement and labral tears may expect favorable outcomes with appropriate labral and capsular management at minimum 2-year follow-up. When compared with a pair-matched control group without GLL, results were comparable for mHHS, NAHS, HOS-SSS, and VAS and reached PASS and/or MCID for mHHS, HOS-SSS, and iHOT-12.