Carbohydrate Restriction-Induced Elevations in LDL-Cholesterol and Atherosclerosis: The KETO Trial.

Background: Increases in low-density lipoprotein cholesterol (LDL-C) can occur on carbohydrate restricted ketogenic diets. Lean metabolically healthy individuals with a low triglyceride-to-high-density lipoprotein cholesterol ratio appear particularly susceptible, giving rise to the novel "lean mass hyper-responder" (LMHR) phenotype. Objectives: The purpose of the study was to assess coronary plaque burden in LMHR and near-LMHR individuals with LDL-C ≥190 mg/dL (ketogenic diet [KETO]) compared to matched controls with lower LDL-C from the Miami Heart (MiHeart) cohort. Methods: There were 80 KETO individuals with carbohydrate restriction-induced LDL-C ≥190 mg/dL, high-density lipoprotein cholesterol ≥60 mg/dL, and triglyceride levels ≤80 mg/dL, without familial hypercholesterolemia, matched 1:1 with MiHeart subjects for age, gender, race, hyperlipidemia, hypertension, and smoking status. Coronary artery calcium and coronary computed tomography angiography (CCTA) were used to compare coronary plaque between groups and correlate LDL-C to plaque levels. Results: The matched mean age was 55.5 years, with a mean LDL-C of 272 (maximum LDL-C of 591) mg/dl and a mean 4.7-year duration on a KETO. There was no significant difference in coronary plaque burden in the KETO group as compared to MiHeart controls (mean LDL 123 mg/dL): coronary artery calcium score (median 0 [IQR: 0-56]) vs (1 [IQR: 0-49]) (P = 0.520) CCTA total plaque score (0 [IQR: 0-2] vs [IQR: 0-4]) (P = 0.357). There was also no correlation between LDL-C level and CCTA coronary plaque. Conclusions: Coronary plaque in metabolically healthy individuals with carbohydrate restriction-induced LDL-C ≥190 mg/dL on KETO for a mean of 4.7 years is not greater than a matched cohort with 149 mg/dL lower average LDL-C. There is no association between LDL-C and plaque burden in either cohort. (Diet-induced Elevations in LDL-C and Progression of Atherosclerosis [Keto-CTA]; NCT057333255).

Increasing the Knee Arc of Motion in Patients With Arthrogryposis: Minimum 2-year Follow-up.

BACKGROUND: Surgery for knee flexion contractures in patients with arthrogryposis multiplex congenital (AMC) have achieved extension to redirect the arc of motion and improve ambulation but has not demonstrated maintained increases in total range of motion (ROM). This study aimed to review the clinical outcomes of combined posterior knee release, proximal femoral shortening, and nerve decompression in patients with arthrogryposis. METHODS: A retrospective chart and radiographic review were performed on patients with AMC who underwent treatment for knee flexion deformities ≥30 degrees. ROM, ambulation status, and orthotic use were reviewed and analyzed. Complications were recorded. RESULTS: Twenty-nine patients with 51 knees and a mean age of 5.7 years were included. The mean follow-up was 36.9 months. The mean ROM increased from 49 to 80 degrees between pre-op and latest follow-up (P<0.0001). The mean final follow-up flexion deformity was 10 degrees (P<0.0001). Preoperative ROM was moderately correlated with final ROM (rs=0.51). The percentage of ambulatory patients improved from 39% to 93%. Five limbs experienced a fracture either intraoperatively or postoperatively, and 5 limbs required a return trip to the operating room. CONCLUSIONS: Improved ambulation and knee ROM can be maintained in patients with AMC at a minimum 2-year follow-up. Prospective investigation and longer follow-up are required to validate these findings. LEVEL OF EVIDENCE: Level IV-therapeutic.

Androgen-Induced, ß-Catenin-Activated Hepatocellular Adenomatosis with Spontaneous External Rupture.

Androgens have long been recognized as oncogenic agents. They can induce both benign and malignant hepatocellular neoplasms, including hepatocellular adenoma (HCA) and hepatocellular carcinoma, though the underlying mechanisms remain unclear. Androgen-induced liver tumors are most often solitary and clinically silent. Herein, we reported an androgen-induced HCA complicated by spontaneous rupture. The patient was a 24-year-old male presenting with fatigue, diminished libido, radiology-diagnosed hepatocellular adenomatosis for 3 years, and sudden-onset, severe, sharp, constant abdominal pain for one day. He used Aveed (testosterone undecanoate injection) from age 17 and completely stopped one year before his presentation. A physical exam showed touch pain and voluntary guarding in the right upper quadrant of the abdomen. An abdominal CT angiogram demonstrated multiple probable HCAs, with active hemorrhage of the largest one (6.6 × 6.2 × 5.1 cm) accompanied by large-volume hemoperitoneum. After being stabilized by a massive transfusion protocol and interventional embolization, he underwent a percutaneous liver core biopsy. The biopsy specimen displayed atypical hepatocytes forming dense cords and pseudoglands. The lesional cells diffusely stained ß-catenin in nuclei and glutamine synthetase in cytoplasm. Compared to normal hepatocytes from control tissue, the tumor cells were positive for nuclear AR (androgen receptor) expression but had no increased EZH2 (Enhancer of Zeste 2 Polycomb Repressive Complex 2 Subunit) protein expression. The case indicated that androgen-induced hepatocellular neoplasms should be included in the differential diagnosis of acute abdomen.

Surgery for Spinal Stenosis in Achondroplasia: Causes of Reoperation and Reduction of Risks.

BACKGROUND: Individuals with achondroplasia are prone to symptomatic spinal stenosis requiring surgery. Revision rates are thought to be high; however, the precise causes and rates of reoperation are unknown. The primary aim of this study is to investigate the causes of reoperation after initial surgical intervention in individuals with achondroplasia and spinal stenosis. In addition, we report on surgical techniques aimed at reducing the risks of these reoperations. METHODS: A retrospective review was conducted over an 8-year period of all patients with achondroplasia at a single institution that serves as a large referral center for patients with skeletal dysplasias. Patients with achondroplasia who underwent spinal surgery for stenosis were identified and the need for revision surgery was studied. Data collected included demographic, surgical, and revision details. Fisher exact test was used to determine if an association existed between construct type and the need for revisions. RESULTS: Thirty-three of the 130 (22%) patients with achondroplasia required spinal stenosis surgery. Twenty-four individuals who met the criteria were selected for analysis. The initial spine surgery was at an average age of 18.7 years (SD: 10.1 y). Nine patients (38%) required revision surgeries, and 3 required multiple revisions. Five of 9 (56%) of the revisions had primary surgery at an outside institution. Revision surgeries were due to caudal pseudarthrosis (the distal instrumented segment) (8), proximal junctional kyphosis (PJK) (7), and new neurological symptoms (7). There was a significant association found between construct type and the need for revision ( P =0.0111). The pairwise comparison found that short fusions were significantly associated with the need for revision compared with the interbody group ( P =0.0180). PJK was associated with short fusions when compared with the long fusion group ( P =0.0294) and the interbody group ( P =0.0300). Caudal pseudarthrosis was associated with short fusions when compared with the interbody group ( P =0.0015). Multivariate logistic regression found long fusion with an interbody was predictive of and protective against the need for revision surgery ( P =0.0246). To date, none of the initial cases that had long fusions with caudal interbody required a revision for distal pseudarthrosis. CONCLUSIONS: In patients with achondroplasia, the rate of surgery for spinal stenosis is 22% and the risk of revision is 38% and is primarily due to pseudarthrosis, PJK, and recurrent neurological symptoms. Surgeons should consider discussing spinal surgery as part of the patient's life plan and should consider wide decompression of the stenotic levels and long fusion with the use of an interbody cage at the caudal level in all patients to reduce risks of revision. LEVEL OF EVIDENCE: Level IV-Retrospective case series.

Peroneal Nerve Decompression in Patients with Multiple Hereditary Exostoses: Indications, Complications, and Recurrence.

BACKGROUND: To our knowledge, there have been no studies examining peroneal nerve decompression and proximal fibular osteochondroma excision exclusively in patients with multiple hereditary exostoses (MHE). The purpose of this study was to evaluate the indications, complications, and recurrence associated with nerve decompression and proximal fibular osteochondroma excision in patients with MHE. METHODS: The records on patients with MHE undergoing peroneal nerve decompression from 2009 to 2023 were retrospectively reviewed. Indications, clinical status, surgical technique, recurrence, and complications were recorded and were analyzed using the Fisher exact test, logistic regression, and the Kaplan-Meier method. RESULTS: There were 126 limbs identified in patients with MHE who underwent peroneal nerve decompression. The most common indications were pain over the proximal fibula, tibialis anterior and/or extensor hallucis longus weakness, and dysesthesias and/or neuropathic pain. Seven cases experienced postoperative foot drop as a complication of the decompression and osteochondroma excision. Logistic regression found significant relationships between complications and excision of anterior osteochondromas (odds ratio [OR], 5.21; p = 0.0062), proximal fibular excision (OR, 14.73; p = 0.0051), and previous decompression (OR, 5.77; p = 0.0124). The recurrence rate was 13.8%, and all recurrences occurred in patients who were skeletally immature at the index procedure. The probability of skeletally immature patients not experiencing recurrence was 88% at 3 years postoperatively and 73% at 6 years postoperatively. CONCLUSIONS: Indications for peroneal nerve decompression included neurologic symptoms and pain. The odds of a complication increased with excision of anterior osteochondromas and previous decompression. Recurrence of symptoms following decompression and osteochondroma excision was found exclusively in skeletally immature patients. LEVEL OF EVIDENCE: Therapeutic Level III . See Instructions for Authors for a complete description of levels of evidence.

The Ethics of Hope-A Moral Imperative for Oncologists.

This Viewpoint suggests that giving patients with terminal cancer unrealistic hope based only on cure is ethically inferior to redirecting patients toward noncurative goals and noncancer-related hope.

Hope during somber times: a defining moment.

In addition to the diverse educational materials the National Cancer Institute provides to patients, caregivers, and health-care professionals, the NCI maintains an online dictionary that provides concise definitions of terms used in modern oncology. The dictionary is regarded as a valuable data reserve because it is updated frequently and employs language that facilitates comprehension among lay users and professionals alike. In this commentary, we call attention to the absence of the word hope as an entry in this important digital resource. Moreover, we propose not only including the concept of hope in this vital reference text but also basing its meaning on C. R. Snyder's model of "hope theory," which stresses the importance of 3 entities: goals, pathways thinking, and agency. Finally, we argue that hope can be taught as a skill to relevant stakeholders (eg, patients, caregivers, and health-care professionals) through brief workshops that have recently been expanded from in-person encounters to virtual platforms revolving around a user-friendly mobile app called "Hopetimize."

Multi-professional development of a competency framework for patients with a Port-a-Cath (PAC).

PURPOSE: In France, 40,000 Port-a-Cath (PAC) are inserted each year. These medical devices are prone to complications during their insertion or use. The education of patients wearing these devices could be a lever to reduce the risk of complications. The objective of this work was to develop, in a multi-professional and consensual manner, a unique and specific skills reference framework for patients with PAC and to propose it as a reference tool for health professionals. METHODS: A multidisciplinary working group was set up to draw up this reference framework of skills. The first stage of the work consisted of a reflection leading to an exhaustive list of competencies necessary for the patient. These skills were then classified according to three different fields of knowledge (theoretical, know-how and attitudes). Finally, the working group identified priority competencies and established a grid that can be used to evaluate the level of acquisition of these competencies. RESULTS: Fifteen competencies were identified: five relating to theoretical knowledge, six relating to know-how and four relating to attitudes. These competencies were broken down into sub-competences. Seven competencies or sub-competencies were selected to constitute the list of priority competencies. DISCUSSION: This competency framework provides a reference framework for the education of patients with PAC and will help to harmonise practices within the different teams that care for patients with PAC.

Feasibility and acceptability of an online intervention to enhance hopefulness among oncology professionals.

BACKGROUND: Patients prefer medical communication including both hopefulness and realism, though health-care professional (HCPs) struggle to balance these. Providers could thus benefit from a detailed personal understanding of hope, allowing them to model and convey it to patients. Additionally, given that hope is associated with lower levels of burnout, HCPs may benefit from tools designed to enhance their own personal hopefulness. Several investigators have proposed offering HCPs interventions to augment hope. We developed an online workshop for this purpose. METHODS: Feasibility and acceptability of the workshop were assessed in members of the SWOG Cancer Research Network. Three measures were used: the Was-It-Worth-It scale, a survey based on the Kirkpatrick Training Evaluation Model, and a single item prompting participants to rate the degree to which they believe concepts from the workshop should be integrated into SWOG studies. RESULTS: Twenty-nine individuals signed up for the intervention, which consisted of a single 2-hour session, and 23 completed measures. Results from Was-It-Worth-It items indicate that nearly all participants found the intervention relevant, engaging, and helpful. Mean ratings for Kirkpatrick Training Evaluation Model items were high, ranging from 6.91 to 7.70 on 8-point scales. Finally, participants provided a mean rating of 4.44 on a 5-point scale to the item "To what degree do you believe it may be useful to integrate concepts from this workshop into SWOG trials/studies?" CONCLUSIONS: An online workshop to enhance hopefulness is feasible and acceptable to oncology HCPs. The tool will be integrated into SWOG studies evaluating provider and patient well-being.

Vitamin D and malabsorptive gastrointestinal conditions: A bidirectional relationship?

This paper is one of the outcomes of the 5th International Conference "Controversies in Vitamin D" held in Stresa, Italy from 15 to 18 September 2021 as part of a series of annual meetings which was started in 2017. The scope of these meetings is to discuss controversial issues about vitamin D. Publication of the outcomes of the meeting in international journals allows a wide sharing of the most recent data with the medical and academic community. Vitamin D and malabsorptive gastrointestinal conditions was one of the topics discussed at the meeting and focus of this paper. Participants to the meeting were invited to review available literature on selected issues related to vitamin D and gastrointestinal system and to present their topic to all participants with the aim to initiate a discussion on the main outcomes of which are reported in this document. The presentations were focused on the possible bidirectional relationship between vitamin D and gastrointestinal malabsorptive conditions such as celiac disease, inflammatory bowel diseases (IBDs) and bariatric surgery. In fact, on one hand the impact of these conditions on vitamin D status was examined and on the other hand the possible role of hypovitaminosis D on pathophysiology and clinical course of these conditions was also evaluated. All examined malabsorptive conditions severely impair vitamin D status. Since vitamin D has known positive effects on bone this in turn may contribute to negative skeletal outcomes including reduced bone mineral density, and increased risk of fracture which may be mitigated by vitamin D supplementation. Due to the immune and metabolic extra-skeletal effects there is the possibility that low levels of vitamin D may negatively impact on the underlying gastrointestinal conditions worsening its clinical course or counteracting the effect of treatment. Therefore, vitamin D status assessment and supplementation should be routinely considered in all patients affected by these conditions. This concept is strengthened by the existence of a possible bidirectional relationship through which poor vitamin D status may negatively impact on clinical course of underlying disease. Sufficient elements are available to estimate the desired threshold vitamin D level above which a favourable impact on the skeleton in these conditions may be obtained. On the other hand, ad hoc controlled clinical trials are needed to better define this threshold for obtaining a positive effect of vitamin D supplementation on occurrence and clinical course of malabsorptive gastrointestinal diseases.

Hope and cancer.

Two models of hope have predominated in the literature pertaining to cancer-Snyder's "Hope Theory" and Herth's hope model-both of which have produced brief self-report measures. Growing evidence demonstrates that hope, as operationalized in these models, is associated with a number of psychological variables in individuals with cancer, including depression, distress, coping, symptom burden, and posttraumatic growth. Emerging evidence also suggests that hope may predict probability of survival in advanced cancer. Surveys show that patients and families generally prefer healthcare communication that authentically conveys prognosis; such communication is not associated with decreased hope, but may actually foster hope. Finally, several interventions have been developed to enhance hope in people with cancer, which generally demonstrate medium effects.

Rare Case of Mixed Phenotype Acute Leukemia Presenting as a Myeloid Sarcoma Without Leukemic Involvement.

INTRODUCTION: Mixed phenotype acute leukemia (MPAL) is a rare type of acute leukemia with immunophenotypic features of both myeloid-derived and lymphoid-derived lineages. CASE PRESENTATION: We present an atypical case of a 32-year-old woman presenting with an anterior mediastinal mass and pericardial/pleural involvement that was initially diagnosed as primary mediastinal diffuse large B-cell lymphoma. However, flow cytometry on pleural fluid confirmed the diagnosis of MPAL of B-cell/myeloid lineage without peripheral blood/bone marrow involvement. The patient was treated with an acute lymphoblastic leukemia-type regimen and proceeded with myeloablative allogeneic hematopoietic cell transplantation in first complete remission. CONCLUSION: MPAL can rarely present with isolated extramedullary disease without leukemic involvement and can often be misdiagnosed as a non-Hodgkin lymphoma. Careful integration of all the clinical data, particularly flow cytometry results, can clarify the diagnosis and change the treatment plan.

Development of a measure of clinicians' self-efficacy for medical communication (SEMC).

OBJECTIVES: Studies of clinician-patient communication have used varied, ad hoc measures for communication efficacy. We developed and validated the Self-Efficacy for Medical Communication (SEMC) scale as a standard, quantitative measure of clinician-reported skills in communicating difficult news. METHODS: Using evidence-based scale development guidelines, we created two 16-item forms of the SEMC, one assessing communication with patients and one assessing communication with families. Clinicians providing oncological care in four organisations were invited to participate and provided consent. Participant demographics, responses to the SEMC items and responses to convergent and discriminant measures (those expected to relate strongly and weakly to the SEMC) were collected online. We performed analyses to determine the convergent and discriminant validity of the SEMC as well as its reliability and factor structure. RESULTS: Overall, 221 oncology clinicians (including physicians, residents, fellows, medical students, nurses, nurse practitioners and physician assistants) participated. The patient and family forms both demonstrated high internal consistency reliability (alpha=0.94 and 0.96, respectively) and were strongly correlated with one another (r=0.95, p<0.001). Exploratory factor analysis demonstrated that the SEMC measures a unitary construct (eigenvalue=9.0), and its higher mean correlation with convergent (r=0.46) than discriminant (r=0.22) measures further supported its validity. CONCLUSIONS: Our findings support the SEMC's validity and reliability as a measure of clinician-rated communication skills regarding conducting difficult conversations with patients and families. It provides a useful standard tool for future research in oncology provider-patient serious illness communication.

Transtemporal Sigmoid Sinus Decompression: A Novel Surgical Procedure for the Treatment of Idiopathic Pulsatile Tinnitus.

OBJECTIVE: Idiopathic pulsatile tinnitus (IPT) is associated with high patient morbidity although treatment methods remain unsatisfactory. In the present study, the transtemporal sigmoid sinus decompression is used in the treatment of idiopathic pulsatile tinnitus. STUDY DESIGN: Retrospective case study. SETTING: Tertiary referral center. PATIENTS: From 2005 to 2020, 287 patients presented with a complaint of pulsatile tinnitus. After exclusion criteria, 25 patients were diagnosed with IPT. Those patients underwent treatment and were included in a retrospective study. INTERVENTIONS: Following failed conservative therapies, the primary author performed a transtemporal sigmoid sinus decompression surgery on the patients under general anesthesia. MAIN OUTCOME MEASURES: Long-term resolution of IPT was measured using the Tinnitus Handicap Inventory (THI). Outcome measurements were taken preoperatively, immediately postoperatively, three months postoperatively, and the status of all 25 patients is known at the time of this study. RESULTS: Transtemporal sigmoid sinus decompression was performed on 25 patients (mean age: 51.7 years, 80.0% female). Out of the 25 patients, 23 (92.0%) patients experienced complete resolution of their IPT. Statistically significant differences based on preoperative THI (mean THI: 4.19) were evident immediately after surgery (mean THI: 1.31; p < 0.001), at 3 months postoperatively (mean THI: 1.19; p < 0.001), and over a mean follow-up time of 68.7 months (range, 3-168 months) (mean THI: 1.38; p < 0.001). Out of the two patients considered unsuccessful, Case 21 experienced a partial resolution. No major postoperative complications occurred. CONCLUSIONS: Transtemporal sigmoid sinus decompression is a safe and effective surgical procedure demonstrated to give near total resolution in properly selected patients and provides long-term relief for patients with IPT.

Developing an adolescent and adult Fontan Management Programme.

Patients with single-ventricle CHD undergo a series of palliative surgeries that culminate in the Fontan procedure. While the Fontan procedure allows most patients to survive to adulthood, the Fontan circulation can eventually lead to multiple cardiac complications and multi-organ dysfunction. Care for adolescents and adults with a Fontan circulation has begun to transition from a primarily cardiac-focused model to care models, which are designed to monitor multiple organ systems, and using clues from this screening, identify patients who are at risk for adverse outcomes. The complexity of care required for these patients led our centre to develop a multidisciplinary Fontan Management Programme with the primary goals of earlier detection and treatment of complications through the development of a cohesive network of diverse medical subspecialists with Fontan expertise.

Dispositional hope as a potential outcome parameter among patients with advanced malignancy: An analysis of the ENABLE database.

BACKGROUND: Hopefulness, whether inherently present (dispositional hope) or augmented (by enhancement techniques), may affect outcomes. This study was performed to determine the association of dispositional hope with survival among patients diagnosed with advanced cancer. METHODS: Data from ENABLE (Educate, Nurture, Advise, Before Life Ends), a palliative care intervention, were reanalyzed to determine the association of higher dispositional hope and patient survival. This was a secondary analysis of data combined from the ENABLE II and ENABLE III randomized controlled trials (RCTs) with respect to dispositional hope and survival. A dispositional hope index was created from 3 hope items from 2 validated baseline questionnaires. Dispositional hope and survival data were collected during the 2 RCTs. In ENABLE II, participants were randomly assigned to the ENABLE intervention or to usual care. In ENABLE III, participants were randomly assigned to receive the intervention immediately or 12 weeks after enrollment. RESULTS: In all, 529 persons were included in Cox proportional hazards regression analyses to model the effects of dispositional hope on survival. An initial analysis without covariates yielded a significant effect of hope (Wald statistic, 8.649; hazard ratio, 0.941; confidence interval, 0.904-0.980; P = .003), such that higher dispositional hope was associated with longer survival. In a subsequent analysis that included all covariates, the effect of dispositional hope approached statistical significance (Wald statistic, 2.96; hazard ratio, 0.933; confidence interval, 0.863-1.010; P = .085). CONCLUSIONS: Higher levels of dispositional hope were associated with longer survival in patients with advanced cancer. Prospective trials are needed to determine the effects of dispositional and augmented hope on the outcomes of patients with advanced cancer.

Prediction of Radial Head Subluxation and Dislocation in Patients with Multiple Hereditary Exostoses.

BACKGROUND: Multiple hereditary exostoses (MHE) is a rare bone disease that results in growth of benign cartilage-capped tumors and a number of skeletal deformities. Forearm deformities are present in up to 60% of patients with MHE, and radial head subluxation or dislocation occurs in 20% to 30%. Radial head subluxation/dislocation results in a shortened forearm and loss of motion. The purpose of this study was to identify radiographic variables that are most predictive of radial head subluxation/dislocation in an effort to determine the need for prophylactic treatment. METHODS: We retrospectively reviewed the cases of consecutive patients with MHE treated in our center between April 2007 and December 2019. Radiographic measurements included the presence or absence of distal ulnar osteochondromas, total ulnar bow, total radial bow, and percent ulnar length. Participants were separated into 3 groups based on the status of the radial head: located, subluxated, and dislocated. Radiographic measurements were compared using a Kruskal-Wallis H test with Dunn post-hoc analysis. A prediction model was run using a binomial logistic regression, and a prediction matrix was created. RESULTS: A total of 88 patients were included in the study. There were significant differences in the located group compared with the dislocated group in terms of pronation, supination, and extension. The percent ulnar length, total ulnar bow, and total radial bow differed significantly between the located and dislocated groups (p < 0.0001); however, in the binomial regression analysis, only the percent ulnar length and total ulnar bow could be used to distinguish between the located group and the subluxated/dislocated group. Both of these measurements were significant predictors of subluxation/dislocation. There was no radial head subluxation/dislocation in patients with an ulnar bow of <17°. CONCLUSIONS: The data indicate that total ulnar bow and percent ulnar length are good predictors of radial head subluxation/dislocation. These 2 parameters can be utilized to monitor forearm deformity and guide timing for prophylactic treatment. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Lengthening the Lower Extremities of Children with Ollier's and Maffucci's Enchondromatosis Using Implantable Lengthening Nails.

There are multiple forms of enchondromatosis with Ollier's and Maffucci's being the most prevalent types. Limb length discrepancy is a common problem in patients with Ollier's and Maffucci's enchondromatosis. There are multiple reports about lengthening bones in patients with enchondromatosis using external fixators. However, there are no case series regarding the use of implantable lengthening technology. The purpose of this paper is to describe our experience with implantable nail lengthening in patients with enchondromatosis. A retrospective chart and radiographic review of patients with enchondromatosis who underwent implantable nail limb lengthening was performed. Seven patients with 14 bony segments were reviewed. A total of 11/14 lengthenings were completed without difficulty. There were no issues in terms of fixation location in patients with Ollier's disease. One patient with Maffucci's syndrome experienced migration of the nail during two lengthenings due to a combination of intralesional fixation and preconsolidation. One patient with Ollier's disease developed a knee extension contracture requiring manipulation under anesthesia. No other complications were recorded. The use of implantable nail lengthening to resolve limb length discrepancies in patients with Ollier's disease appears to be safe and effective.