Recent Follow-up Research Articles

Surgical Treatment and Outcomes of Trans-Ulnar Basal Coronoid Fracture-Dislocations

Trans-ulnar fracture-dislocations of the elbow are complex injuries that can be difficult to classify and treat. Trans-ulnar basal coronoid injuries, in which the coronoid is not attached to either the olecranon or the metaphysis, present substantial challenges to achieve anatomic reduction and stable internal fixation. The purpose of this study was to analyze the outcome of surgical treatment of trans-ulnar basal coronoid fracture-dislocations. Between 2002 and 2019, 32 consecutive trans-ulnar basal coronoid fracture-dislocations underwent open reduction and internal fixation (ORIF) at our institution. Four elbows were lost to follow-up within the first 6 months after surgery and were excluded. Among the 28 elbows remaining, there were 13 females and 15 males with a mean age of 56 (range 28-78) years at the time of injury. The mean clinical and radiographic follow-up times were 37 months and 29 months, respectively. Radiographs were reviewed to determine rates of union, Hastings and Graham heterotopic ossification (HO) grade, and Broberg and Morrey arthritis grade. Union occurred in 25 elbows. Union could not be determined for 1 elbow at most recent follow-up and the remaining 2 elbows developed nonunion of the coronoid. Complications occurred in 10 elbows (36%): deep infection (4), ulnar neuropathy (2), elbow contracture (2), and nonunion (2). There were reoperations in 11 elbows (39%): irrigation and débridement with hardware removal (4), hardware removal (2), ulnar nerve transposition (2), contracture release with HO removal (2), and revision ORIF with iliac crest autograft (1). At most recent follow-up, the mean flexion-extension arc was 106° (range 10-150°), and the mean pronation-supination arc was 137° (range 0-170°). The mean Quick Disabilities of Arm, Shoulder, and Hand score was 11 (range 0-39) points with a mean Single Assessment Numeric Evaluation-Elbow score of 81 (range 55-100) points. At final radiographic follow-up, 16 elbows (57%) had HO (8 class I and 8 class II), and 20 elbows (71%) had arthritis (8 grade 1, 6 grade 2, and 6 grade 3). Trans-ulnar basal coronoid fracture dislocations are severe injuries associated with high rates of reoperation, heterotopic ossification, and post-traumatic arthritis. However, the majority of elbows achieve union, a functional range of motion, and reasonable patient reported outcome measures. Over the study period, surgeons were more likely to utilize multiple deep approaches and separate fixation of the coronoid (either with lag screws or anteromedial plates) to ensure anatomic reduction.

Leadless pacemaker - lead parameters stability and battery longevity in the mid-term follow-up

Abstract Background A leadless pacemaker (L-PM) has been designed to avoid the need for a pacemaker pocket and transvenous lead. Long-term safety will largely depend on the need for replacements, mainly due to battery depletion (end-of-life). While the mean L-PM battery longevity predicted by the manufacturer is 12 years, real-world longevity data are still scarce. We aimed to describe L-PM parameters’ stability and battery longevity during the mid-term follow-up. Methods Single-center registry of consecutive patients undergoing L-PM implantation from May 2015 to November 2023. Procedural characteristics, lead parameters and battery longevity were collected immediately after implantation and during the most recent follow-up visit. Results Overall, 261 consecutive patients had a transcatheter L-PM implanted through a femoral vein (mean age 78±10 years; 66% male). Mean procedural duration was 44±28min and fluoroscopy time was 4,4±3,9min. The device was successfully implanted in all but one patient. The main indications for permanent pacing were high-degree atrioventricular block [N=130 (50%)], atrial fibrillation with pauses [N=78 (30%)], and sinus node disease [N=28 (11%)]. Immediately after implantation, three cases (1,2%) of pericardial effusion (one with tamponade) were reported. In one case (0,4%), a conventional pacemaker was implanted due to L-PM acute dysfunction. No other procedure-related complications, including dislodgment, were reported at discharge. After a mean follow-up of 2,8±1,7years, the pacing threshold and R-wave amplitude remained stable [0,67mV vs. 0,62mV (p=0,9); and 10,3mV vs. 16,1mV (p<0,001), respectively] and mean ventricular pacing was 54±38%. Expected battery longevity was >8 years (maximum value) in 84% of the patients. In the subgroup of patients with >5 years of follow-up (N=20; mean follow up of 6,3±1,8years), the expected battery longevity was >8 years in 85% (N=17) of the patients. In three cases, the L-PM was upgraded to cardiac resynchronization therapy or left bundle branch area pacing due to pacing-induced left ventricular dysfunction. There was one case of L-PM end-of-life 6 years after implant (pacing threshold 0,5mV; ventricular pacing 100%), in which a second L-PM was successfully implanted. Overall, 78 (31%) patients died, a mean of 2,2±1,9 years after L-PM implantation, none related to the device. Conclusion In this real-world cohort, L-PM maintained stable pacing parameters and, in the mid-term follow-up, only one battery depletion was observed while expected longevity remained above 8 years in the large majority of patients. Longer follow up will allow better understanding of both technical and clinical aspects relevant to manage end-of-life devices.Table 1Figure 1

Effect of a participatory whole-systems approach on mortality in children younger than 5 years in Jigawa state, Nigeria (INSPIRING trial): a community-based, parallel-arm, pragmatic, cluster randomised controlled trial and concurrent mixed-methods process evaluation

In 2019, Nigeria reported the highest mortality rate in children younger than 5 years globally. We aimed to assess a whole-systems approach to improving child mortality in northern Nigeria. We conducted a community-based, parallel-arm, pragmatic, cluster randomised controlled trial in Kiyawa local government area, Jigawa state, Nigeria, and a concurrent mixed-methods process evaluation using ethnography and quantitative implementation monitoring. Trial clusters were population catchment areas of 32 government primary health-care facilities. Compounds were randomly sampled, proportional to cluster size, and all women aged 16-49 years and children younger than 5 years who were permanent residents were eligible for inclusion and recruited as the evaluation population. Children younger than 7 days were recruited but excluded from analysis. Evaluation clusters were allocated to intervention or control via simple randomisation with a 1:1 ratio. Cluster names were written on paper, folded, and placed in a container by community representatives. Different community representatives took out names one by one, with the first half assigned to receive the intervention. The intervention consisted of three components: participatory learning and action (PLA) groups for men and women (including compound heads [ie, the member of the compound that residents deemed most senior]), partnership defined quality scorecard (PDQS), and health-care worker capacity building; it was delivered from March 1, 2021, to Dec 31, 2022. We could not mask participants, field staff, or intervention-delivery staff to cluster allocation but baseline, endline, and follow-up data excluded information on cluster allocation. PLA groups involved separate groups of up to 25 men or women from all villages in the intervention clusters. The primary outcome was all-cause mortality in children aged 7 days to 59 months between Oct 1, 2021, and Sept 20, 2022, referred to as the evaluation period. The trial was prospectively registered (ISRCTN 39213655) and the protocol has been published. We recruited 3800 compounds at baseline, with 12 893 children contributing to analysis of the primary outcome (7316 [56·8%] of 12 893 in the intervention group and 5577 [43·3%] in the control group). 6617 (51·3%) of 12 893 children were male, 6275 (48·7%) were female, and one (<0·1%) child had missing sex data. Sampled compounds randomly came from 388 (91·3%) of 425 villages in the 32 clusters. We conducted verbal autopsies for 1182 deaths, of which 369 (31·2%) were children aged 7 days to 59 months during the evaluation period. Of these 369, 91 (24·7%) were classified as pneumonia deaths. Children contributed a median 361 days (IQR 236-365) to the analysis, with 369 (2·9%) of 12 893 children censored on their date of death, 1545 (12·0%) on their 5th birthday, and 3392 (26·3%) on the date of the most recent follow-up in which their residence or survival status was known. We found no significant decrease in all-cause mortality (hazard ratio 0·95, 95% CI 0·68-1·33; p=0·79) or suspected pneumonia mortality (0·79, 0·43-1·46; p=0·46) in the intervention group. The process evaluation showed low coverage and issues in reach of the intervention, but qualitative data highlighted mechanisms for positive effects on health and relationships. Our intervention did not affect mortality. However, due to the high child mortality in this region, further efforts should be made to adapt our participatory whole-systems approach to use communities of action within compounds. GSK and Save the Children UK. For the Hausa translation of the abstract see Supplementary Materials section.

Auer rod-positive acute leukemia with predominantly lymphoid immunophenotype: Report on 11 cases and review of literature.

Auer rods (AuRs) are prominent intracellular structures found almost exclusively in myeloid cell malignancies, such as acute myeloid leukemia (AML), chronic and juvenile myelomonocytic leukemia and myelodysplastic syndrome. Extremely rare AuRs have been reported in patients with acute lymphoblastic leukemia (ALL) or among ambiguous lineage leukemia patients with a dominantly lymphoblastic immunophenotype. We report diagnostic and follow-up data of an international cohort of 11 children suffering from leukemias with AuRs and with significant presence of T and myeloid markers, majority of whom categorized as early T-cell precursor (ETP, n=7); or T-ALL (ETP status unknown, n=2), ALAL (acute leukemia of ambiguous lineage, n=1), and AML reclassified from ALAL (n=1). We described other diagnostic details and treatment types and responses. Moreover, we summarize previously published data. Among the four patients who started and remained on ALL-type therapy, all were in the first complete remission, whereas both patients who started and remained on AML-type therapy relapsed and died. Of the patients who followed either a combined ALL/AML protocol (Interfant 06) or who switched from one of the two types of therapy to the other, one patient died, and the remaining four were in first complete remission at the most recent follow-up. We also searched for similar cases in the literature and found only three additional children with nonmyeloid leukemia and AuRs and 10 adults with this type of leukemia. Briefly, ALL- or combined ALL/AML-type therapy may be effective for treating AuR-positive leukemia patients with a lymphoid immunophenotype.

Respiratory failure and rhabdomyolysis caused by severe hypokalemia in a young female with hypertension: a rare critical condition in primary aldosteronism

BackgroundThe two classic manifestations of primary aldosteronism are hypertension and hypokalemia. However, acute respiratory failure due to hypokalemia in primary hyperaldosteronism is rare.Case presentationThe patient was a 27-year-old female who presented with drowsiness and weakness in all extremities. She had been diagnosed with hypertension three years prior, with irregular follow-up, and had a history of preeclampsia one year later. She exhibited high blood pressure and severe hypokalemia (2 mEq/L), leading to respiratory depression and impending respiratory arrest. Consequently, the patient was intubated and transferred to the intensive care unit (ICU). She also developed rhabdomyolysis. Blood pressure tests, including hormonal tests (aldosterone: 13.2 ng/dL, plasma renin activity: 0.32 ng/mL/h), were conducted. Due to the high aldosterone-renin ratio, an abdominopelvic computed tomography (CT) scan was performed. The CT scan revealed a 14 × 12 mm round mass with a washout value above 60%, consistent with an adrenal adenoma, leading to a diagnosis of primary aldosteronism. The patient was discharged after stabilization, and one and a half months after ICU admission, a laparoscopic left adrenalectomy was successfully performed without post-operative complications. Histopathology showed encapsulated hypertrophy of the adrenal cortex with a predominance of large clear cells, confirming the diagnosis of adrenal adenoma. At the most recent follow-up, the patient had normal potassium levels, was normotensive without any medications, and exhibited no alarming signs or symptoms.ConclusionRespiratory depression to the extent of impending respiratory failure and rhabdomyolysis as a result of hypokalemia in primary aldosteronism are extremely rare. In this patient, who developed respiratory depression due to resistant hypokalemia, timely investigation of secondary causes and diagnosis of adrenal adenoma were crucial. The surgery provided definitive treatment for the patient’s blood pressure and prevented the recurrence of life-threatening complications.

Racial, Ethnic, and Socioeconomic Disparities in Pediatric Aquaporin-4-Positive Neuromyelitis Optica Spectrum Disorder

BackgroundOnly 5% of aquaporin-4-positive neuromyelitis optica spectrum disorder (AQP4+ NMOSD) cases emerge during childhood. Poorer outcomes have been suggested in black/African American (AA) adults with NMOSD; however, conflicting and limited data exist for pediatric-onset NMOSD. This study evaluates racial, ethnic, and socioeconomic disparities in pediatric AQP4+ NMOSD outcomes. MethodsThirty-eight pediatric patients with AQP4+ NMOSD cared for at three pediatric tertiary care centers between 2009 and 2021 were identified. Patient addresses connected to socioeconomic measures available from the US Census. Demographic characteristics, pertinent clinical outcomes, and health care utilization in the two years following diagnosis were captured. ResultsCompared with non-Hispanic White children, Black/AA children had a significantly higher Expanded Disability Status Scale (EDSS) (2.46 vs 0.33, P = 0.003), 2.37 more hospital admissions (P = 0.002), and 28.40 additional inpatient days (P = 0.002) in the two years following their NMOSD diagnosis. Additionally, children with public insurance had higher relapse rates than those with private insurance (P = 0.046). At two years and at the most recent follow-up, a significantly higher EDSS was correlated with children living in census tracts with a lower median income, higher deprivation index, and higher proportion of population on assisted income, in poverty, and with vacant housing (all P < 0.05). ConclusionsWe identified racial, ethnic, and socioeconomic disparities in clinical outcomes and health care utilization in pediatric AQP4+ NMOSD. Further prospective and household-level data are needed to dissect the interplay of genetics, structural racism, and social determinants of health so that interventions to optimize care and outcomes for this population may be developed.

Lentiviral Gene Therapy for Cerebral Adrenoleukodystrophy

BackgroundCerebral adrenoleukodystrophy is a severe form of X-linked adrenoleukodystrophy characterized by white-matter disease, loss of neurologic function, and early death. Elivaldogene autotemcel (eli-cel) gene therapy, which consists of autologous CD34+ cells transduced with Lenti-D lentiviral vector containing ABCD1 complementary DNA, is being tested in persons with cerebral adrenoleukodystrophy.MethodsIn a phase 2–3 study, we evaluated the efficacy and safety of eli-cel therapy in boys with early-stage cerebral adrenoleukodystrophy and evidence of active inflammation on magnetic resonance imaging (MRI). The primary efficacy end point was survival without any of six major functional disabilities at month 24. The secondary end points included overall survival at month 24 and the change from baseline to month 24 in the total neurologic function score.ResultsA total of 32 patients received eli-cel; 29 patients (91%) completed the 24-month study and are being monitored in the long-term follow-up study. At month 24, none of these 29 patients had major functional disabilities; overall survival was 94%. At the most recent assessment (median follow-up, 6 years), the neurologic function score was stable as compared with the baseline score in 30 of 32 patients (94%); 26 patients (81%) had no major functional disabilities. Four patients had adverse events that were directly related to eli-cel. Myelodysplastic syndrome (MDS) with excess blasts developed in 1 patient at month 92; the patient underwent allogeneic hematopoietic stem-cell transplantation and did not have MDS at the most recent follow-up.ConclusionsAt a median follow-up of 6 years after lentiviral gene therapy, most patients with early cerebral adrenoleukodystrophy and MRI abnormalities had no major functional disabilities. However, insertional oncogenesis is an ongoing risk associated with the integration of viral vectors. (Funded by Bluebird Bio; ALD-102 and LTF-304 ClinicalTrials.gov numbers NCT01896102 and NCT02698579, respectively.)

Aberrant right subclavian artery intervention can provide relief of dysphagia.

An aberrant right subclavian artery represents the most common aortic arch vascular anomaly. Conventional wisdom states that these anomalies do not result in dysphagia, but rather serve as "red herrings". Clearly, in the vast majority of cases, this holds true. Nonetheless, one should never say never. Herein, we present a cohort of four children with debilitating dysphagia resulting from an aberrant right subclavian artery. Subclavian reimplantation via a right posterolateral thoracotomy was performed successfully in all cases. Dysphagia resolved postoperatively, and all patients were able to advance to a normal diet. They were able to gain appropriate weight postoperatively and continue to do well at most recent clinical follow-up. This case series suggests that aberrant right subclavian artery anatomy should be considered a potential aetiology of dysphagia, albeit rarely. Surgical intervention for select patients can provide dramatic resolution of symptoms.

7787 Primary Aldosteronism Medical Treatment Outcomes (PAMO): An International Consensus on Outcome Assessment Criteria and Multicentre Study

Abstract Disclosure: J. Yang: None. J. Burrello: None. J. Goi: None. T.A. Williams: None. W.F. Young: Consulting Fee; Self; Arena Pharmaceuticals, Inc.. Other; Self; Crinetics Pharmaceuticals. P.J. Fuller: None. P. Investigators: None. Primary aldosteronism (PA) is the most common endocrine cause of hypertension. Mineralocorticoid receptor antagonists, at adequate doses, lower blood pressure and cardiovascular risk in patients with PA. However, there is a lack of a standardized approach to medical therapy and subsequent outcomes assessment. We aimed to: 1) establish criteria for assessing outcomes of targeted medical treatment of PA, and 2) evaluate outcomes using these criteria across an international cohort. A panel of 31 PA experts from 31 centres across four continents used the Delphi method, including three rounds of online questionnaires, to reach consensus. Clinical data were then collected from consecutive patients with PA who started targeted medical treatment between 2016 and 2021 at the participating centres. Consensus was reached for six outcomes, including complete, partial, or absent biochemical response, and complete, partial, or absent clinical response. “Complete biochemical response” was defined as “correction of hypokalemia without potassium supplementation, and normalization of plasma renin activity or concentration”; while “absent biochemical response” was defined as “persistent hypokalemia and/or persistently suppressed or low plasma renin activity or concentration without change from baseline”. “Complete clinical response” was defined as “normal blood pressure without additional antihypertensive medications”; while “absent clinical response” was defined as “the same or increased blood pressure compared to before targeted treatment with same or more antihypertensive medications, not counting targeted therapy”. Of 1483 patients (mean age 52y, 48% female) from 28 centers, 232 (16%) had a complete clinical response, and 719 (49%) had a complete biochemical response at the most recent follow-up (median 29 months after treatment initiation). Compared to those with partial or absent clinical response, patients with complete clinical response had a significantly higher proportion of women and lower proportion with diabetes, stroke or left ventricular hypertrophy. They also had a significantly shorter duration of hypertension, lower BMI, higher eGFR and lower defined daily dose of antihypertensives at baseline. In contrast, these differences were not observed in those with a complete, partial or absent biochemical response. The defined daily dose of targeted therapy did not differ across the various response categories. In conclusion, we have established an international expert consensus for standardized criteria to evaluate the response to targeted medical treatment of PA, facilitate comparisons of outcome data and guide clinicians who manage PA. In a retrospective international cohort, less than half of medically-treated patients reached the established biochemical treatment targets. Further work is needed to increase the rate of complete response. Presentation: 6/3/2024

7202 CPP with Rapid Pubertal Progression in a 9-Year-Old Boy with Hypothalamic Teratoma

Abstract Disclosure: R. Robilliard: None. G. Tung: None. K. Svokos: None. R. Lulla: None. J.Q. Quintos: None. Background: Central Precocious Puberty (CPP) in males is rare, with incidence of 0.01-2/10,000. The most common identifiable cause of CPP in young children is hypothalamic hamartoma. No case hypothalamic teratoma has been reported to cause CPP in males. Clinical Case: At the age of 10-years and 5-months, a boy presented to the pediatric endocrinology with signs of precocious puberty including facial hair development, voice deepening, and increased growth velocity that began at 9 years old. There was no significant past medical history and he denied headache, blurry vision, or diplopia. Physical examination showed height of 140.4 cm (47%), Tanner 4 genitalia, Tanner 3 pubic hair, and testicular volumes of 15 cc (left) and 12-15cc (right). Bone age (BA) determined by radiography was 13.5 years with a predicted adult height (PAH) of 63 inches. The mid-parental target height was 67 +/- 4 inches. Evaluation including TSH, glucose, serum and CSF β-HCG, DHEA, CSF AFP was normal. Serum LH was 2.9 IU/L and testosterone level was 109 ng/dl, consistent with CPP. MRI of the pituitary showed a small, heterogeneous and fat-containing lesion extending inferiorly from the tuber cinereum consistent with teratoma. Treatment included GnRH agonist Lupron (30mg) every 3 months. Laboratory evaluation 8 months after initial presentation showed suppressed LH of 0.9 IU/L and FSH 0.5 mIU/mL, and testosterone of 57 ng/dL. Repeat radiography for BA one year after GnRHa suppression showed BA of 14 years at CA of 11 years 6 months and PAH of 64.4 inches. Growth velocity slowed to 5.3 cm/year. At the most recent follow-up visit at CA of 12 years, height was 151.5 cm (61%), growth velocity 6.3 cm/year (74% for age), Tanner 5 penis and pubic hair, left testicular volume of 15-20cc and right testicular volume of 15 cc. Most recent radiographic BA is14 years, and PAH is 65.9 inches. Given response to GnRH agonist injections every 3 months, the absence of CNS symptoms referable to the teratoma and no change in MR imaging, no surgical intervention is recommended. Conclusion: This is the first case of a hypothalamic teratoma reported in a male associated with CPP. Despite this rare cause, the patient has responded to standard treatment with GnRH agonist therapy alone. Presentation: 6/1/2024

Ten-year follow-up after face transplantation—A single-center retrospective cohort study

Face transplantation has emerged as reconstructive option for the most challenging facial deformities. A comprehensive analysis of functional outcomes, medical complications, incidence of malignancy, and chronic rejection in face transplantation recipients over an extended follow-up period has not yet been published leaving a notable gap in the literature. We retrospectively collected data of morbidity, rejection, vasculopathy, metabolic side effects, as well as functional outcome of sensory return, facial motor function, and speech from 9 patients who underwent face transplantation at Brigham and Women’s Hospital between 2009 and 2020. The median follow-up was 120 months (54 and 154 months). Four grafts (40%) developed signs of clinical and histopathologic chronic rejection without evidence of vasculopathy on computed tomography angiograms. Sensory return assessed with Weinstein enhanced sensory testing-monofilament showed an increase in 6 patients (66.7%), and facial expression analysis showed improvement throughout the whole cohort at their most recent follow-up. Speech intelligibility was stable or increasing for 5 patients (55.6%). In conclusion, the long-term outcomes reveal promising results in terms of overall graft retention and functional recovery. Metabolic, malignant, and infectious complications, as well as graft rejection episodes, are expected to occur in this population, and some may be related to patient’s age and lifestyle.

Choroid Involvement Secondary to Optic Disc Pit Maculopathy: OCT Analysis And Evolution After Surgical Treatment: Choroidal Involvement In Optic Pit Maculopathy

PurposeTo assess choroidal changes associated to optic disc pit maculopathy (ODP-M) and their evolution after surgical treatment. DesignMulticentric retrospective case series. MethodsAnalysis of 42 patients affected by unilateral ODP-M undergoing surgical treatment between 2013 and 2023. Optical coherence tomography (OCT) were performed at baseline and postoperative months 1, 6, 12, 24 and most recent follow-up. Subfoveal choroidal thickness (SFCT) and peripapillary choroidal thickness (PPCT) were measured in ODP-M and fellow eyes. The presence of retinal pigmented epithelium (RPE) atrophy was used to distinguish between “early” and “advanced” disease, and data regarding fluid localization were collected. ResultsBaseline SFCT in ODP-M eyes was significantly higher than fellow eyes (386.8±88.9 vs. 334.4±72.2 μm, p=0.002), differently from PPCT (192.6±47.8 vs. 181.2±45.7 μm, p=0.46). SFCT significantly decreased 1 month post-operatively (mean reduction 36.5 μm, p=0.009) and remained below pre-operative values throughout the follow-up, showed a mean reduction of 79.4 μm at final follow-up (p<0.001). Conversely, PPCT showed no changes between pre-operative and post-operative values (all p>0.05). Nine eyes (21.4%) showed submacular dilated choroidal vessels, correlated with the presence of subretinal fluid (p=0.008) and reducing in caliber after surgical treatment. The 10 eyes (23.8%) with “advanced” disease had lower baseline SFCT and worse BCVA compared to the “early” subgroup, and showed a delayed reduction of choroidal swelling post-operatively. ConclusionSubfoveal choroid may thicken and remodel in response to ODP-M, eventually returning to physiological values after surgical treatment. Moreover, the presence of RPE atrophy may influence retino-choroidal balance. Conversely, PPCT didn't show comparable modifications.

A Novel Approach for Tetralogy of Fallot-Absent Pulmonary Valve Using Bioresorbable Airway Splints.

Tetralogy of Fallot with absent pulmonary valve (ToF-APV) is associated with severe tracheobronchomalacia (TBM) and significant airway compromise. These patients often require early repair with right ventricle-to-pulmonary artery conduit, pulmonary arterioplasty, tracheostomy, and long-term ventilator support. A bioresorbable, 3D-printed airway splint has shown early success in treating severe TBM and has the potential to obviate the need for early repair with conduit and tracheostomy. A retrospective case series analysis was conducted on consecutive patients with ToF-APV and severe TBM who underwent airway splinting between 2012 and 2021. Clinical data was extracted from the medical record. Patients were grouped and analyzed according to their sequence of procedures. Eight patients with ToF-APV and severe TBM underwent airway splinting with a median follow up of 3.6years (range 1.0-6.4). All patients were alive at the most recent follow-up. Five patients underwent complete cardiac repair first, and one patient underwent concurrent complete cardiac repair and airway splinting. All six of these patients required tracheostomy and long-term ventilator support, even after airway splinting. Five of six remained on ventilator support at the most recent follow up. Two patients underwent airway splinting before complete cardiac repair. Neither required tracheostomy nor prolonged ventilatory support (p = 0.036). Both were discharged home in the neonatal period and returned as infants to undergo elective ToF-APV repair. Patients with TOF-APV and severe TBM may be able to avoid early repair with conduit placement and tracheostomy by undergoing airway splinting prior to complete cardiac repair.

The surgical management of third ventricle region tumors

ObjectivesThe goal of this study was to characterize the largest known cohort of patients presenting with different tumor pathologies in the third ventricle region to better understand outcomes of surgical management. MethodsAll patients undergoing surgical intervention on tumors in or encroaching upon the third ventricle at Loyola University Medical Center between the years 1986–2021 were reviewed. Information recorded included presenting symptoms, pre- and post-operative interventions, tumor pathology, operative technique, extent of resection (EOR), and approach of operation. The primary clinical outcome was Karnofsky Performance Status (KPS) score. ResultsNinety-seven patients underwent 123 operations. Forty-six (47.4 %) patients were female, and the median age at operation was 39 years. Eighty-seven (70.7 %) operations were open, and 36 (29.3 %) were endoscopic. Gross total resection (GTR) was achieved in 34.4 % of operations, near-total resection (NTR) in 31.5 %, subtotal resection in 25.0 %, and biopsy alone in 9.3 %. Median KPS increased pre- to postoperatively, regardless of surgical technique. Adjusting for preoperative KPS, age, and operation number, regression analysis demonstrated a trend that lesser EOR is associated with lower KPS at most recent follow-up (p=0.031 for NTR vs GTR, p=0.022 for biopsy vs GTR). There was no statistically significant association between the most recent KPS and either open or endoscopic surgical technique, with or without adjusting for the previously stated factors (p=0.26). There was no association between postoperative complication rates or age with either surgical technique. ConclusionsHere, we characterize a large cohort of patients presenting for neurosurgical evaluation of tumors in the region of the third ventricle. Our results demonstrate a trend that a more aggressive resection may yield better KPS outcomes. Additionally, both open and endoscopic techniques lead to a similar improvement in clinical outcome and rates of complication. While ultimate surgical approach and technique is determined by individual tumor characteristics, patient health status, and surgeon expertise, ability to resect the tumor in its entirety should be taken into consideration.

The Viability and Success of Noncemented Kinematic Total Knee Arthroplasty.

The prevalence of noncemented total knee arthroplasty (TKA) is increasing as personalized knee alignment strategies deviate from implanting components on a strict mechanical axis. This retrospective study evaluated the outcomes of 74 consecutive noncemented unrestricted kinematic TKA procedures. This study included 74 consecutive noncemented kinematic TKAs performed by one surgeon at a tertiary academic medical center from 2021 to 2023. The technique used was unrestricted femur-first caliper kinematic TKA. The outcomes included revision, pain scores, and radiographic measurements. Of the 74 procedures performed, there were no revisions or readmissions for problems related to TKA. The mean follow-up was 17.6 months, with 74% of patients being followed up for more than 1 year postoperatively. On the day of surgery, postoperative measurements showed that the average tibial mechanical, distal femoral, and anatomic tibiofemoral angles were 3.3°, 7.7°, and 5.8°, respectively. 5 knees were observed initially with signs of radiolucency, which all resolved by the most recent appointment. None of the knees was radiographically loose. Of the patients, 65%, 19%, and 16% reported no pain, minimal pain, and some pain, respectively, at the 6-week follow-up visit. This improved to 78%, 19%, and 3% at the most recent follow-up. Combining kinematic alignment with noncemented fixation showed excellent clinical and radiographic outcomes with short-term survivorship. Although the use of both kinematic alignment and noncemented TKAs has been controversial, these early data suggest that noncemented kinematic TKA is safe and effective.

Prevalence of HLA-B27, clinical characteristics and treatment outcomes in children with enthesitis-related arthritis

BackgroundEnthesitis-related arthritis (ERA) is a subtype of juvenile idiopathic arthritis with high disease burden. The objectives of this study were to explore the prevalence of HLA-B27, clinical characteristics, and treatment outcomes in children with ERA and compare the differences between HLA-B27 positive and negative patients.MethodsA retrospective cohort study at a pediatric rheumatology clinic in a tertiary referral hospital in Bangkok, Thailand, including ERA patients with at least 6 months of follow-up (July 2011-April 2022) was performed. Data were collected from medical records from diagnosis to recent follow-up, assessing disease activity and treatment outcomes, with an analysis comparing HLA-B27 positive and negative patients. Descriptive statistics were used for data analysis.ResultsThere were 59 ERA patients with mean age ± SD at diagnosis 11.2 ± 2.5 years, 53 males (89.8%), and positive HLA-B27 in 38 patients (64.4%). The HLA-B27 positive group had significantly higher levels of inflammatory markers at initial diagnosis (p = 0.001), lower baseline hemoglobin (p = 0.001) and hematocrit (p = 0.002), higher disease activity assessed by the Juvenile Spondyloarthritis Disease Activity score at 6 and 12 months of follow-up (p = 0.028 and 0.040, respectively), increased utilization of bridging systemic corticosteroids (60.5% vs. 14.3%, p = 0.001) and anti-TNF (39.5% vs. 9.5%, p = 0.018), and longer duration of methotrexate (median[IQR] 1.7[1.1–3.1] vs. 1.3[0.6–1.9] years, p = 0.040). The HLA-B27 negative group had more prevalent hip arthritis than the positive group at initial diagnosis (66.7% vs. 28.9%, p = 0.005) and during the course of the disease (71.4% vs. 36.8%, p = 0.011).ConclusionMost of the ERA patients tested positive for HLA-B27. Throughout the follow-up period, these patients demonstrated greater disease activity, greater use of corticosteroids and anti-TNF, and longer duration of methotrexate to control the disease.

Long-Term Outcomes of Nonsyndromic Isolated Unilateral Lambdoid Craniosynostosis

This study combined qualitative and quantitative approaches to evaluate outcomes of isolated nonsyndromic unilateral lambdoid synostosis at the Children's Hospital of Philadelphia. Volumetric, linear, and angular analyses were performed on long-term postoperative and normal control scans. Preoperative and postoperative clinical photographs were evaluated for the presence of dysmorphic frontofacial features. Among 26 included patients, median age of surgery was 10 months and mean postoperative follow-up was 5.9±5.7 years. Two (7.7%) patients underwent secondary cranial vault procedures. At most recent follow-up, 2 (7.7%) subjects reported intermittent headaches. Twenty (90.9%) of 22 patients were assigned Whitaker grade I. Among 9 subjects with long-term imaging at age 11.5±5.3, posterior vault asymmetry, posterior fossa deflection angle, cranial base angle, and ear position asymmetry all remained greater than in the control group. Of subjects included in the frontofacial feature analysis (n=10), 50% had resolution of all dysmorphic frontofacial features present preoperatively. Overall, most subjects who underwent PVR did not require revisional surgery and had good outcomes both aesthetically and functionally. Despite residual abnormalities in the cranial base and posterior vault, most had resolution of parietal bossing and facial scoliosis. Ear position asymmetry was the most common dysmorphic feature at long-term follow-up.

Early Compared with Delayed Reconstruction in Multiligament Knee Injury: A Retrospective Propensity Analysis.

The purpose of this study was to compare outcomes following early compared with delayed reconstruction in patients with multiligament knee injury (MLKI). A retrospective cohort analysis of patients with MLKI from 2007 to 2019 was conducted. Patients who underwent a reconstructive surgical procedure with ≥12 months of postoperative follow-up were included. Patients were stratified into early reconstruction (<6 weeks after the injury) and delayed reconstruction (12 weeks to 2 years after the injury). Multivariable regression models with inverse probability of treatment weighting (IPTW) were utilized to compare the timing of the surgical procedure with the primary outcome (the Multiligament Quality of Life questionnaire [MLQOL]) and the secondary outcomes (manipulation under anesthesia [MUA], Kellgren-Lawrence [KL] osteoarthritis grade, knee laxity, and range of motion). A total of 131 patients met our inclusion criteria, with 75 patients in the early reconstruction group and 56 patients in the delayed reconstruction group. The mean time to the surgical procedure was 17.6 days in the early reconstruction group compared with 280 days in the delayed reconstruction group. The mean postoperative follow-up was 58 months. The early reconstruction group, compared with the delayed reconstruction group, included more lateral-sided injuries (49 patients [65%] compared with 23 [41%]; standardized mean difference [SMD], 0.44) and nerve injuries (36 patients [48%] compared with 9 patients [16%]; SMD, 0.72), and had a higher mean Schenck class (SMD, 0.57). After propensity adjustment, we found no difference between early and delayed reconstruction across the 4 MLQOL domains (p > 0.05). Patients in the early reconstruction group had higher odds of requiring MUA compared with the delayed reconstruction group (24 [32%] compared with 8 [14%]; IPTW-adjusted odds ratio [OR], 3.85 [95% confidence interval (CI), 2.04 to 7.69]; p < 0.001) and had less knee flexion at the most recent follow-up (β, 6.34° [95% CI, 0.91° to 11.77°]; p = 0.023). Patients undergoing early reconstruction had lower KL osteoarthritis grades compared with patients in the delayed reconstruction group (OR, 0.46 [95% CI, 0.29 to 0.72]; p < 0.001). There were no differences in clinical laxity between groups. Early reconstruction of MLKIs likely increases the likelihood of postoperative arthrofibrosis compared with delayed reconstruction, but it may be protective against the development of osteoarthritis. When considering the timing of MLKI reconstruction, surgeons should consider the benefit that early reconstruction may convey on long-term outcomes but should caution patients regarding the possibility of requiring an MUA. Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence.

The Wagner Cone Stem for Atypical Femoral Anatomy in Total Hip Arthroplasty: A Report of 320 Cases with Minimum 5-Year Follow-up.

The Wagner Cone Prosthesis was designed to address complex femoral deformities during total hip arthroplasty (THA), but its mid-term component survivorship and functional outcomes remain undetermined. The objectives of this study were to determine the implant survivorship, patient satisfaction, functional outcomes, osseointegration as seen radiographically, implant subsidence, and complications of THA using the Wagner Cone Prosthesis stem at intermediate-term follow-up. This study involved 302 patients with proximal femoral deformities, including developmental hip dysplasia and Legg-Calvé-Perthes disease, who underwent a total of 320 primary THAs using the Wagner Cone Prosthesis. The average age at the time of surgery was 49.4 ± 14.5 years (range, 18.8 to 85.6 years). Patient satisfaction was recorded using a self-administered questionnaire assessing satisfaction in 4 domains. The University of California at Los Angeles (UCLA) activity score, the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), the Oxford Hip Score (OHS), the Forgotten Joint Score (FJS), radiographic outcomes, and complications were recorded. The mean follow-up time was 10.1 years (range, 5.2 to 15.5 years). Survivorship of the Wagner Cone Prosthesis was 98.7% (95% confidence interval [CI]: 97.2% to 100%) with stem revision as the end point and 95.8% (95% CI: 93.5% to 98.2%) with reoperation for any reason as the end point at 10 years postoperatively. In total, 3 stems were revised: 2 for infection and 1 for chronic hip dislocation. The median patient satisfaction score was 95 (interquartile range [IQR], 80 to 100), median UCLA score was 6 (IQR, 6 to 7), median WOMAC score was 18 (IQR, 16 to 22), median OHS was 40 (IQR, 36 to 47), and median FJS was 80 (IQR, 76 to 88) at the time of final follow-up. All Wagner Cone stems that were not revised showed radiographic evidence of osseointegration, with a mean stem subsidence of 0.9 ± 0.8 mm at the most recent follow-up. The use of the Wagner Cone Prosthesis stem in patients with complex femoral anatomy undergoing primary THA is associated with excellent component survivorship, high levels of patient satisfaction, good functional outcomes, and reliable osseointegration with minimal stem subsidence as seen on radiographs at intermediate-term follow-up. Therapeutic Level IV . See Instructions for Authors for a complete description of levels of evidence.

Aseptic Revision Total Hip Arthroplasty Using Modular Fluted Tapered Stems: Long-Term Follow-up of 515 Cases.

Modular fluted tapered (MFT) femoral components are frequently utilized in aseptic revision total hip arthroplasties (THAs). However, long-term follow-up has been limited. The purpose of this study was to update our prior series at long-term follow-up, with specific emphasis on implant survivorship, radiographic results, and complications in a large cohort of aseptic revision THAs using MFT stems. We retrospectively identified 515 aseptic femoral revisions performed with 2 MFT stem designs in the total joint registry of a single tertiary care academic institution from 1999 to 2013. Serial radiographs were reviewed for subsidence of >5 mm. The mean follow-up (and standard deviation) was 10 ± 5 years (range, 2 to 21 years). A competing risk model accounting for death was utilized. The 15-year cumulative incidence of any revision was 12%. There were 57 revisions, 27 of which involved revision of the fluted tapered component (FTC). Dislocation (n = 19), periprosthetic joint infection (n = 15), and aseptic loosening of the FTC (n = 11) were the most common reasons for revision. The 15-year cumulative incidence of any reoperation was 16%. The 15-year cumulative incidences were 6% for any FTC revision and 2% for FTC revision for aseptic loosening. Stem subsidence of >5 mm occurred in 2% of unrevised cases, and all but 1 stem was stable at the most recent follow-up. This large series of MFT stems used in aseptic revision THAs had a 2% incidence of subsequent revision of the FTC for aseptic loosening at 15 years. Dislocation and infection were the most common reasons for any revision. Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.