Ollier's disease (multiple enchondromatosis) can cause severe lower limb length discrepancy and deformity in children. Osteotomy and limb lengthening with external fixation can correct the lower extremity deformity. There may be lesions in the osteotomy part (OP), and the internal fixation part of the external fixation(FP). This study aimed to evaluate: (1) whether lesions in OP and FP influence the lengthening length, speed, and deformity correction; (2) the number of intact sides of the OP and FP that are necessary to provide enough stability to achieve the lengthening correction aim.

Fifteen children with Ollier's disease underwent treatment of 21 lower limb segments using distraction osteogenesis. All osteotomies were performed at the center of rotation and angulation, resulting in a total of fourteen OP intralesionally and nineteen FP intralesionally. The lengthening length, speed, and correction of angular deviation were compared in different groups (lesions vs. non-lesions in OP /FP).

Full correction of the deformity and full restoration of length were achieved in all cases. There were no significant differences between intralesional (14 cases) and extralesional (7 cases) distraction groups in new bone formation speed (OP). Although 19/21 of the FP were inserted intralesionally, all the wires and half-pins were well stabilized throughout the external fixation period. There were no significant differences between these groups in the incidence of complications, such as infection, pathological fractures, and early consolidation.

In Ollier's disease, the stability provided by newly formed callus and the external fixation were sufficient to lengthen and correct lower limb deformities successfully, even when OP and FP were performed intralesionally with / without intact sides.

Leg length discrepancy (LLD) and malalignment of long bones are frequent orthopedic problems encountered in Maffucci syndrome and Ollier disease (OD). Orthopedic surgeons used historically external fixators to address the deformities. In this multicentric case series, we propose the use of motorized intramedullary nails.

We retrospectively reviewed for 9 years, in four different centers, patients with OD and Maffucci's syndrome that had lengthening nails for LLD with or without associated deformities. The minimum follow-up period was 24 months. We reported complications, clinical tolerance of lengthening, lengthening rate and target, bone healing index, and EQ-5D-Y functional and visual analog scores (VAS). We also saw on X-rays the whole lengthened bone and its regenerate zone to assess the evolution of the enchondromas.

we used the nailing technique in 8 femurs and 2 tibias in 8 patients (mean age: 13.3 years, range: 11-16, mean follow-up time: 53.8 months, range: 26-108). The mean correction amount was 6.44 cm for the femur over 76.8 days and 3.75 cm over 44 days for the tibia with a mean VAS score of 6.63/15 and mean EQ-5D-Y of 81/100. The lengthening goal was achieved in all patients. No mechanical complications were noted. The medullary canal of the operated bones showed improvement and healing in 8 out of 10 segments.

Besides achieving the goals of surgery with good functional outcomes, lengthening nails has a therapeutic effect on enchondromas with fewer complications than traditional correction methods.

Ollier disease is a rare skeletal dysplasia characterized by the formation of multiple enchondromas (enchondromatosis), typically in the long bones of the extremities. These tumors are benign but can become complicated by the development of pathologic fractures, limb deformity, and malignant transformation to chondrosarcoma. Ollier disease has a highly variable presentation and is associated with a range of presenting findings; however, the most common presentation is a pathologic fracture. Surgical options include curettage and grafting of the enchondromas and, when displaced, fracture reduction and fixation. Of note, these fractures will heal without surgery. Regardless, all patients must be routinely monitored with yearly radiographs in order to detect malignant transformation as early as possible.  In this report, we describe the case of an 11-year-old female who presented to her physician with pain and swelling of her right ring and small fingers after playing in a swimming pool with no obvious mechanism of trauma. A routine, plain radiographic evaluation of her hand revealed the presence of multiple enchondromatosis. We hope to use this case to highlight the surgical management options for young patients with Ollier disease and discuss circumstances in which surgical management may not be indicated.

Ollier's disease can cause severe length discrepancy of the lower extremities and deformity in children. Osteotomy and limb lengthening with external fixation can correct the limb deformity. This study evaluated (1) whether the duration of external fixation was reduced in patients with Ollier's disease, and (2) the incidence of complications such as pin tract infection, external fixation loosening, and joint stiffness.

Two groups were compared with respect to age, angular correction (AC), lengthening gap (LG), distraction index (DI), lengthening length (LL), lengthening length percentage (L%), lengthening index (LI), bone healing index (BHI), and external fixation index (EFI). Group 1 (Ollier's disease) comprised nine patients undergoing 11 lower limb lengthening procedures using external fixators; group 2 (control, normal lengthened bone) comprised 28 patients undergoing 29 lengthening procedures with external fixators.

In patients with Ollier's disease, full correction of the deformity and full restoration of length were achieved in all cases. In the femur, the mean AC (15.97° vs. 6.72°) and DI (1.11 mm/day vs. 0.78 mm/day) were significantly larger, while the LI (9.71 days/cm vs. 13.49 days/cm), BHI (27.00 days/cm vs. 42.09 days/cm), and EFI (37.86 days/cm vs. 56.97 days/cm) were all significantly shorter in group 1 than in group 2 (p < 0.05). In the tibia, the mean AC and L% were larger, while the LG, LI, BHI, and EFI were all shorter in group 1 than in group 2. There was no significant difference between the two groups in the incidence of complications.

In children with Ollier's disease, new bone formation accelerated and the healing speed of the lengthened segments was faster throughout the whole lengthening period with external fixation, and full correction of the deformity and full restoration of length could be achieved.

The aim of this study was to explore the symptoms, treatment, and pathogenesis of ovarian juvenile granulosa cell tumors with Ollier's disease in children.

From October 2019 to October 2020, clinical data were retrospectively analyzed for one case of ovarian juvenile granulosa cell tumors with Ollier's disease. Whole-exome sequencing and Sanger sequencing were used to detect gene mutation in ovarian tumor and chondroma tissue. NADP-dependent isocitrate dehydrogenase-1 (IDH1) and S6 ribosomal protein expression levels in cells transfected with wild-type or mutant plasmid were analyzed by Western blot.

The 4-year-old female showed multiple skeletal deformities, bilateral breast development with chromatosis, and vulvar discharge. Sex hormone assay suggested that estradiol and prolactin were elevated, and the x-ray of limbs suggested enchondroma. Pelvic ultrasound and abdominal CT revealed a right ovarian solid mass. Pathologic examination of the right ovarian solid mass showed a juvenile granulosa cell type. A c.394C>T (p. Arg132Cys) mutation of the IDH1 gene was detected in both the ovarian juvenile granulosa cell tumors and enchondroma. Transfection of HeLa cells with either WT or Mut plasmid caused 4.46- or 3.77-fold overexpression of IDH1 gene compared to non-transfected control cells, respectively. R132C mutation inhibited the phosphorylation of S6 ribosomal protein, which is central to the mTOR pathway. Postoperatively, estradiol and prolactin levels fell to values normal for her age and bilateral breast gradual retraction.

The incidence of ovarian juvenile granulosa cell tumors with Ollier's disease in children may be caused by generalized mesodermal dysplasia; IDH1 gene mutation may play a facilitated role in this process. Surgical operation is the main treatment. We suggest that patients with ovarian juvenile granulosa cell tumors and Ollier's disease should undergo regular investigation.

Limb lengthening and deformity correction in patients with abnormal bone associating fragility often require an approach combining methods of external and internal fixation. This study demonstrates results of simultaneous application of external fixator, and telescopic rod for femoral lengthening and deformity correction in three children with osteogenesis imperfecta or severe form of Ollier's disease.

Three patients (two boys with Ollier's disease and a girl with osteogenesis imperfecta, type I) were operated on for femoral lengthening with combined technique associating Ilizarov frame and titanium telescopic intramedullary rodding.

Planned amount of lengthening and deformity correction were achieved for all patients. We found neither rod bending nor pull out of threaded tips. There was no difficulty of expanding of telescopic intramedullary rods made of titanium alloy during distraction phase of lengthening procedure.

This short series proved feasibility of performing one-stage surgery with external frame and telescopic rodding in limb lengthening. The technique of telescopic rods in lengthening procedure is promising method requiring meticulous insertion of rod in centralized positioning in epiphysis. Acute alignment of the segment been elongating should be achieved at surgery. No any progressive angular deformity correction in postoperative period is authorized in order to avoid bending of telescopic rod. This combined approach does not affect bone healing.

Limb length discrepancy and deformities resulting from Ollier's disease are challenging to treat and have increased complications. We aimed to assess the safety of intralesional osteotomy for distraction osteogenesis and report the results of guided growth as a method of deformity correction in such conditions. We retrospectively reviewed 13 patients (eight boys and five girls), 28 segments (12 femora and 16 tibias), treated using Ilizarov circular ring fixator in one center. Nine patients had an oblique plane deformity, whereas four had a coronal plane deformity. Femoral shortening ranged from three to 11 cm. Tibial shortening ranged from 3.5 to 12 cm. Intralesional osteotomy was carried out in all patients, and guided growth (hemiepiphysiodesis) was used in seven segments (25%). The median age was 11 years (6-14 years) at surgery, with a median follow-up of 4.5 years (3-18 years). The median achieved lengthening in the femur was 7 cm (5-11 cm) and in the tibia was 5 cm (3-9 cm). The average Bone Healing Index (BHI) for the femur was 32 days/cm (28-38 days/cm), and for the tibia was 36 days/cm (28-40 days/cm). Before frame removal, the mechanical axis was restored to the knee joint center in all cases. Normal radiographic bone regeneration was evident in all cases. Hemiepiphysiodesis successfully corrected the angular deformities. Intralesional osteotomy for distraction osteogenesis is well-tolerated and reliable in Ollier's disease. Radiological normal bone was formed at the distraction site. Guided growth is also a reproducible method for deformity correction in Ollier's disease, similar to other conditions.

There is a clear clinical need for efficient physiotherapy and rehabilitation programs during and after bone lengthening and reconstruction for gaining the optimal effect and also prevention or treatment of lengthening side effects. Pin tract infection is the most prevalent side effect during lengthening which could be prevented and treated initially via proper wound care. Muscle contractures are typically a consequence of the generated tension on the distracted muscle. It can be managed by physiotherapy initially and surgically in later severe stages. Furthermore, it is essential to avoid muscle contracture development, which is the demonstration of the imbalanced muscle appeals on the joint to inhibit the following subluxation. The knee is the furthermost affected joint by the aforementioned problem due to the inherent lack of ligamentous and bony stability. Joint stiffness is the other possible unfavorable effect of lengthening. It happens because of extensive muscle contractures or may possibly be attributed to rigidity of the joint following the amplified pressure on the joint surface during the process of lengthening. Physiotherapy and occupational therapy including endurance and strength exercise as well as stretching play an important role during the rehabilitation periods for the prevention and also the treatment of muscle contracture and the following deformity and also joint stiffness. Likewise, the effect of mental and physical rehabilitation programs should not be overlooked.

The Ilizarov method is one of the current methods used in bone reconstruction. It originated in the middle of the past century and comprises a number of bone reconstruction techniques executed with a ring external fixator developed by Ilizarov GA. Its main merits are viable new bone formation through distraction osteogenesis, high union rates and functional use of the limb throughout the course of treatment. The study of the phenomenon of distraction osteogenesis induced by tension stress with the Ilizarov apparatus was the impetus for advancement in bone reconstruction surgery. Since then, the original method has been used along with a number of its modifications developed due to emergence of new fixation devices and techniques of their application such as hexapod external fixators and motorized intramedullary lengthening nails. They gave rise to a relatively new orthopedic subspecialty termed "limb lengthening and reconstruction surgery". Based on a comprehensive literature search, we summarized the recent clinical practice and research in bone reconstruction by the Ilizarov method with a special focus on its modification and recognition by the world orthopedic community. The international influence of the Ilizarov method was reviewed in regard to the origin country of the authors and journal's rating. The Ilizarov method and other techniques based on distraction osteogenesis have been used in many countries and on all populated continents. It proves its international significance and confirms the greatest contribution of Ilizarov GA to bone reconstruction surgery.

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.

Reconstruction of the distal femur in children following resection of bone sarcoma is challenging. The main problem in children is the small size of bone and a possible limb-length discrepancy at the end of skeletal growth secondary to the loss of the physes. We reported the results of a new surgical technique for distal femur reconstruction after bone tumor resection in children.

We analyzed 5 patients with distal femoral sarcomas who underwent intra-articular resection and reconstruction with resurfaced allograft-prosthetic composite at a mean follow-up of 70 months. There were 2 males and 3 females, with a mean age of 10 years (range 8-12) at the time of the diagnosis. All patients were affected by high-grade osteosarcoma. The patients' medical records were reviewed for clinical and functional outcomes as well as post-operative complications. The functional evaluation of the patients was done at the end of the follow-up using Musculoskeletal Tumor Society scoring system. The minimal follow-up was 24 months.

At the last follow-up, 4 patients were continuously disease-free. We excluded one patient who died of disease secondary to lung metastases 16 months after the surgery. Complications occurred in 2 of 4 patients at 17 months and 24 months, respectively. One patient developed deep infection who required the removal of the original reconstruction and, once the infection was treated, the patient underwent reconstruction with an expandable prosthesis. An allograft fracture occurred in another of the 4 patients at 24 months after the first surgery, thus the original reconstruction was removed and the patient underwent reconstruction with modular prosthesis. In the two patients who retained the original reconstruction at the time of their latest follow-up, the mean implant survival time was 70 months. These patients had an excellent MSTS score (29.5 points) and walked without support or limitations with an active knee range of motion of > 90° and complete active extension of the knee. No degenerative changes of the articular surface of the proximal tibia and the patella were observed at the time of the last follow-up. Growth of the physis of the proximal tibia was observed in all the patients during follow-up and no angular deformity of the joint was observed. The limb discrepancy was 4 cm and 2 cm, respectively.

Resurfaced allograft-prosthetic composite may represent an alternative surgical technique for distal femur reconstruction in children with bone sarcomas. Although its success is limited by high risk of complications, resurfaced allograft-prosthetic composite seems to be a viable option to preserve the bone stock and the physis of the proximal tibia in selected young patients, minimizing a potential limb-length discrepancy at the end of the skeletal growth.