Osteofibrous dysplasia-like adamantinoma versus osteofibrous dysplasia in children: A case report of challenging diagnosis

IntroductionOsteofibrous dysplasia (OFD) and Osteofibrous dysplasia-like Adamantinoma have a similar appearance both in clinical and radiography, but different in its histopathology. Despite this similarity, the treatment and prognosis are different, therefore the diagnosis should be established precisely.Case illustrationA three-year-old boy was admitted to hospital after falling on his lower leg. A bead size lump appeared on his tibia with pain and swelling, which later became enlarged. Diagnosis of osteofibrous dysplasia and adamantinoma was considered. We performed limb-salvage procedure by curretage, bone grafting, and internal fixation application. The histology section showed woven bone rimmed by polygonal osteoblast cell with intervening fibrous stroma and small nests of tumour cells raised the possibility of epithelial differentiation. The positivity for cytokeratin immunostaining confirmed the diagnosis as osteofibrous dysplasia-like adamantinoma. In this case it is a very rare spectrum of malignancy in children.DiscussionThese two tumor entities have identical radiographic characteristics, histopathology features the distinction between classic adamantinoma and OFD-like adamantinoma based on the predominant epithelial component. The relationship of osteofibrous dysplasia with adamantinoma is unclear. Several authors considered possible calling relationship osteofibrous dysplasia as “juvenile adamantinoma”. However, does not rule out the possible existence of de novo osteofibrous dysplasia not related to adamantinoma.ConclusionsOFD-like adamantinoma and Osteofibrous Dysplasia had similar histopathology pattern, a pathologist must be aware of this feature and perform immunohistochemical staining for keratin particularly when the histopathological feature of osteofibrous dysplasia showed small nests of tumor cells within the fibrous stroma. diagnostic challenging and require multidisciplinary approach.     

Osteofibrous dysplasia of the ulna

We experienced two patients with osteofibrous dysplasia of the ulna. Both of them showed typical radiographic findings of osteofibrous dysplasia, except for location. One patient underwent surgery at the age of 15 years to confirm the diagnosis histologically. Although the tumor recurred 5 months after the surgery, expansion of the lesion seemed to cease at the age of 25 years. In the other patient, the natural course was observed. The tumor showed a tendency toward spontaneous regression at the age of 11 years. Therefore, the nature of osteofibrous dysplasia in the ulna seems to be the same as that in the tibia and fibula. Surgery must be delayed for as long as possible in osteofibrous dysplasia of the ulna, as well as that of the tibia and fibula. Received: April 26, 2001 / Accepted: July 17, 2001     

骨缝牵引成骨修复腭裂的临床初步研究

目的 临床探索骨缝牵引成骨修复腭裂的新技术。方法 选择2～4岁的腭裂患儿。一期手术在全麻下安置腭裂牵引器，牵引腭骨向中线和向后移动；牵引期持续约4～5个月。裂隙合拢后进行二期手术，修复裂隙。用误差为0．2％的游标卡尺，测量牵引前和牵引后上颌牙弓宽度、裂隙宽度和硬腭长度。结果 临床应用8例。2例因牵引器在安置后l周内脱落而终止牵引。6例经不同时间的牵引后，裂隙缩小，裂隙两侧的组织显著延长。其中牵引时间最长为126d，最短为37d；裂隙宽度平均缩小6．5mm；硬腭长度平均延长4．8mm。结论 临床证实了骨缝牵引具有诱导腭部组织再生、关闭或缩小裂隙和延长硬腭的可能性。     

Successful management of complications from distraction osteogenesis after osteosarcoma resection: a case report

We report a complicated case of osteosarcoma of the proximal tibia. A 15-year-old boy was referred to us and underwent distraction osteogenesis reconstruction. We administered preoperative chemotherapy for five cycles. Clinical response was determined to be complete by radiography. Marginal excision was then performed with preservation of the proximal tibial epiphysis. Metaphyseal reconstruction (type 2) was performed with distraction osteogenesis. Postoperative chemotherapy also was administered for five cycles. Two years later, the patient developed a deep infection. He underwent curettage and a pedicle peroneal flap transfer, which did not cure the infection. Infected tissues were excised, and shortening-distraction was carried out with the Ilizarov frame. The infection was cured; however, a leg length discrepancy and deformity resulted from frame instability. Four years after the initial operation, the patient fractured his reconstructed leg in a traffic accident. We performed osteosynthesis, deformity correction, and lengthening with the Ilizarov method. We were able to correct the defects using distraction osteogenesis, eventually restoring normal function. Epiphyseal preservation and reconstruction by distraction osteogenesis can provide an excellent outcome, resulting in a stable reconstruction that functionally restores the native limb. Distraction osteogenesis avoids some complications but may involve others, which require detection and appropriate management.     

Neonatal osteofibrous dysplasia: a case report

We describe a case of neonatal osteofibrous dysplasia that was diagnosed 3 days after birth. Osteofibrous dysplasia is a fibro-osseous lesion rarely seen in long bones. It is also known as congenital osteitis fibrosa or ossifying fibroma of bones. Although it occurs in children under 10 years of age, it is extremely rare in newborn babies. It must be differentiated from adamantinoma due to their radiological appearance, predilection for tibia and fibula, clinical picture, and certain histological similarities.     

经缝牵引成骨早期矫正儿童面中份发育不全

目的 临床探索经缝牵引、均衡前移面中份骨骼，早期矫治面中份发育不全的新技术新方法。方法 选择6～12岁面中份发育不全的患儿，采用弹性外牵引装置矫正面中份发育不全。牵引系统由经鼻孔插入的骨承力牵引装置、面弓和牙科正畸用的橡皮圈组成。全麻下于梨状孔外下缘的外侧钻孔，通于下鼻道的前部；或在硬腭的前部钻孔，穿透鼻底。经鼻孔将骨牵引承力装置插入梨状孔缘或鼻底的骨孔内。手术后第1～2天用连接于面弓和骨牵引装置的正畸橡皮圈进行牵引。牵引力为600～1200g，随骨骼移动的进度不断调整。骨骼移动达到预定的位置后，减小牵引力维持牵引。结果 临床治疗9例患儿。面中份骨骼移动均衡。患者面容和咬合关系完全恢复正常。面部侧面观显示上、中、下各部分协调匀称，眶下、颧部和上唇部丰满，鼻背挺拔。结论中位缝牵引技术能够早期有效地矫正面中份畸形，骨骼前移均衡，且方法简便，损伤小。     

应用牵引成骨技术治疗中面部骨发育不良畸形

目的 探讨牵引成骨技术对因多颅缝早闭所致的中面部发育不良综合征的治疗效果。方法 冠状切口径路,先用LeFort Ⅲ型截骨法在眶面部截骨,使颅底和中面部先行离断,安装外置REDⅡ式中面部牵引器。术后5d开始牵拉,每天1mm牵拉前移;待中面部到达良好位置和咬合关系时,停止牵拉,并固定该装置2～4个月。结果 完成的8例患者平均手术时间3．5h,术中平均失血300ml,中面部平均前移9．0mm,下移1．5mm;术后面部外形明显改观,咬合关系接近正常;除1例固定脚处的头皮感染和1例头皮血清肿外,术后无严重并发症发生;平均随访5个月,无面部后缩等复发症状。结论 牵引成骨技术可有效治疗学龄儿童和畸形严重的中面部骨发育不良畸形。     

延长成骨术修复颅骨缺损的实验研究

目的探讨以延长成骨修复颅骨缺损的可行性。方法随机挑选1岁龄山羊17只,分为A、B、C三组,A组7只,于顶骨处左右各形成一大小相等面积为3.0 cm×2.2 cm矩形骨缺损;B组5只,于顶骨处右侧形成一2.2 cm×2.2 cm的正方形骨缺损;C组5只,于顶骨处左右各形成一大小为3 cm×1 cm矩形骨缺损。各组于右侧骨缺损形成相应的转移骨瓣并安放延长器,A组三维CT对修复颅骨面积进行测量;B组行成骨后生物力学测量;C组行组织学染色、扫描电镜、透射电镜检查观察成骨过程。结果A组可见实验侧骨缺损大部分逐渐被延长成骨所修复,剩余少量骨缺损与自身对照有统计学意义(P<0.01),B组实验侧与正常对照侧无统计学意义,C组观察成骨过程,为典型的膜内成骨。结论延长成骨术是一种可行的修复颅骨缺损的方法。     

Effects of rapid distraction rate on new bone formation during mandibular distraction osteogenesis in goats

Objective

Distraction osteogenesis typically requires a long treatment period, which can lead to bone and soft-tissue infection and considerable patient discomfort. Use of a rapid distraction rate in craniofacial distraction osteogenesis to shorten the distraction period is possible owing to the unique characteristics of craniofacial bones, including an abundant blood supply and rapid bone healing compared with long bones. The effects of using a rapid distraction rate in the treatment of craniofacial deformities are currently unclear, however. The objective of this study was to investigate the effects of a rapid distraction rate on new bone formation during mandibular distraction osteogenesis in goats.

Methods

Sixteen goats were randomly divided into four groups consisting of four goats each. In Groups A, B, and C, the right mandible of each goat was distracted at a rate of 0.8 mm/d, 1.6 mm/d, and 2.0 mm/d, respectively; Group D was the control group and did not undergo distraction. Six weeks after the conclusion of distraction, bone densitometry and three-point bending testing were performed in all groups.

Results

The mean bone density value of goats in Group A was significantly higher than those of all the other groups (p < 0.05), and the mean bone density value of goats in Group C was significantly lower than those of all the other groups (p < 0.05). The mean curve slope, peak stress, bending modulus, and energy to failure values of Groups A, B, and C were all significantly lower than those of the control group (p < 0.05). As the distraction rate increased, the curve slope and peak stress values gradually declined (p < 0.05).

Conclusions

Use of a rapid distraction rate in mandibular distraction osteogenesis may have detrimental effects on the quality of new bone, despite the abundant blood supply of craniofacial bones.     

Effects of diclofenac and tenoxicam on distraction osteogenesis

Introduction In the management of bone defects, pseudoarthrosis, deformities, chronic osteomyelitis and in extremity lengthening procedures, the technique of distraction osteogenesis (DO) has been frequently used. In this experimental animal study, the effects of two non-steroidal anti-inflammatory drugs, i.e., diclofenac and tenoxicam, on the outcomes of distraction osteogenesis are investigated. Materials and methods In this study, 30 mature New Zealand-type male rabbits (2.5–4.5 kg) were used. The rabbits were randomized into three groups, each consisting of ten animals. Under optimal operating conditions, a pre-reconstructed circular external fixator was applied on the right tibias of rabbits, and osteotomy was performed with a Gigli saw just below the tibial tuberosity. After seven postoperative days, distraction was initiated at a rhythm of 2 × 0.5 mm/day. During the 10 days of distraction, adjunctive therapy was not instituted for group I (control group). For 10 days, group II received i.m. diclofenac sodium (0.5 mg/kg per day) and group III was treated with tenoxicam (8 mg/kg per day i.m.). At the end of 3 weeks postoperatively, five rabbits from each group were killed for histologic examinations. The remaining rabbits were killed at the end of eight postoperative weeks for biomechanical and histological analysis. Besides, radiological examinations were performed at the end of 3, 6 and 8 weeks postoperatively for the radiologic evaluation of calluses. For statistical evaluations between groups, Kruskal–Wallis variance analysis, and for intergroup assessments, Mann–Whitney test were performed. Results For radiological evaluations, the scoring system developed by Lane and Sandhu, and for histopathological assessments, the grading system of Huddlestone et al. were used. Biomechanical tests were realized using torsional loading. During the first 3 weeks, the groups did not differ much in the radiological parameters. However, in the diclofenac group and in especially the tenoxicam group, the histological scores were lower than in the control group. Radiological images obtained at the end of 6 weeks demonstrated inadequate consolidation in the diclofenac and tenoxicam groups when compared with the control group. At the end of 8 weeks postoperatively, in consideration of biomechanical, radiological and histological tests, significantly worse regenerates were obtained in the diclofenac and tenoxicam groups. Conclusions During the distraction osteogenesis period, diclofenac and tenoxicam affected the quality of regenerate unfavorably. This effect is sustained during all periods of bone healing. However, this finding should be supported by experimental and human studies.     

Application of distraction osteogenesis in managing bone cysts

Background

Despite the great amount of research concerning bone cysts, there is still no commonly accepted method of treatment. The aim of this study was to evaluate the effectiveness managing bone cyst with hybrid external fixator by distraction osteogenesis.

Materials and methods

Between 1982 and 2009, 25 patients with unicameral (20 patients) and aneurismal (five patients) bone cysts were treated using this method. Eighteen patients had a history of pathological fracture at the same location. Cysts were located in the humerus, femur, tibia, and radius. Median follow-up was 48 (range 31–91) months. Results were evaluated on plain radiographs according to the classification system of Capanna et al. Functional assessment was done using the modified system recommended by Enneking et al.

Results

In our study group of 25 bone cysts, 15 were classified as completely healed and nine as healed with residual radiolucency. Recurrence was observed in one patient. Absence of response to treatment was not observed. All patients had excellent functional outcomes, except one with recurrence who was rated poor.

Conclusions

As bone cysts are found in long bones in 90–95 % of patients, and taking into account our achieved positive results in almost all patients, we recommend this method of distraction osteogenesis as a treatment option. It is an effective, economical method of treatment, which eliminates deformity and restores bone length, especially in patients with pathologic fractures.     

Complications of distraction osteogenesis in brachymetatarsia: Comparison between the first and fourth brachymetatarsia

BackgroundRecently, distraction osteogenesis has been widely used to treat brachymetatarsia. However, few papers have compared complications associated with this treatment. The purpose of the present study was to compare the complications between the first and fourth brachymetatarsia treated by distraction osteogenesis.MethodsWe performed distraction osteogenesis to 83 metatarsals in 41 patients between 1999 and 2012. A total of 30 metatarsals received treatment for the first metatarsal (Group A) while 53 metatarsals received treatment with the fourth metatarsal (Group B).ResultsThe complication rate in Group A (40%) was higher than that in Group B (18.9%). Those who had high percentage of lengthening gain were more likely to have complications. A cut-off value for lengthening gain developing complication was 41.3%.ConclusionComplication incidence after distraction osteogenesis was increased when lengthening gain was more than 41.3% for brachymetatarsia. In the 1st metatarsal lengthening, the most common complication was stiffness. In contrast, complications of the 4th metatarsal lengthening were pin-track infection and angular deformity.     

富含血小板血浆对牵引成骨过程中新骨生成的影响

目的　探讨富含血小板血浆 (PRP)对成骨细胞以及牵引成骨过程中新骨生成的作用。方法　通过体外成骨细胞的培养及牵引成骨动物模型的制备 ,采用MTT法检测成骨细胞的增殖情况 ,采用组织化学、四环素标记等方法观察骨组织新骨生成情况。结果　PRP可以促进体外培养成骨细胞的增殖 ,促进牵引成骨过程新骨的生成。结论　PRP可以加快牵引成骨过程的新骨生成 ,临床应用可能缩短牵引成骨的治疗过程。     

Management of thromboangiitis obliterans using distraction osteogenesis: A retrospective study

Background:

Thromboangiitis obliterans (TAO), also known as Buerger disease, is characterized by thrombosis in medium-sized arteries and veins along with a marked inflammatory response. TAO can be managed by using the principle of distraction osteogenesis to induce neoangiogenesis. We report thirty patients of TAO in the age-group of 20–50 years were treated with the Ilizarov method.

Materials and Methods:

Patients with severe rest pain (n=30), absent distal pulsation (n=30) and ulcer (n=6) and/or gangrene of the toes (n=4), who had failed the conservative pharmacological modalities of treatment were included. A lateral tibial corticotomy was performed and distraction applied by use of olive wire. A two-ring frame was used for all cases.

Results:

Of the 30 cases, 25 patients became pain free while 1 had partial relief of pain. The four patients worsened on treatment and developed frank infection were amputed. The average distraction was 1.8±0.3 mm (range: 1.5–2.2 cm). The mean consolidation period was 98±11 days, after which the fixator was removed. The total duration of treatment was 122±23 days. There were two cases of osteomyelitis in our series; both healed after debridement and both patients had good result, with relief of pain. The ulcers started healing by 4–6 weeks.

Conclusion:

The principle of the distraction osteogenesis including neoangiogenesis can be used for treatment of TAO and has an acceptable complication rate.     

应用牵引成骨技术治疗颞下颌关节强直继发的小颌畸形

目的 探讨应用牵引成骨技术治疗颞下颌关节强直继发的小颌畸形的临床疗效.方法 应用牵引成骨技术治疗颞下颌关节强直继发的小颌畸形患者43例,10例应用颅骨支持式牵引器行双侧下颌骨体延长;33例应用内置式牵引器行单侧(6例)或双侧(27例)下颌骨体延长,3～6个月的稳定期后,手术拆除牵引器.结果 43例患者80侧下颌骨体部平均牵引长度23.2 mm(14 ～ 35 mm),X线头影测量结果显示后气道间隙由术前的平均4.9 mm增加到术后的10.4 mm,代表下颌突度的SNB角由术前的平均64.2°增加到术后的74.5°,小颌畸形得到了有效治疗,牵引间隙内成骨良好.术后平均复查20.3个月,无不良并发症发生.结论 牵引成骨技术可有效矫治关节强直导致的小颌畸形.颅骨支持式牵引器是治疗儿童青少年小颌畸形的有效手段,手术方法简便、风险小,治疗效果稳定.     

Enhancing bone healing during distraction osteogenesis with platelet-rich plasma

Gradual limb lengthening with external fixators using distraction osteogenesis principles is the gold standard for treatment of limb-length discrepancy. However, long treatment time is a major disadvantage of the current lengthening procedures. Efforts to decrease the treatment include biological and biomechanical factors. Injection of platelet-rich plasma (PRP) is a biological method to enhance bone healing during distraction osteogenesis. We hypothesised that PRP can enhance bone healing during limb lengthening. We report our experience with the use of PRP during distraction osteogenesis. This retrospective study included 19 patients divided into the standard group of 10 patients who did not receive PRP and the PRP group of nine patients who received PRP at the end of the distraction phase. The study variables included external fixator time, external fixation index, and complications during treatment. The PRP group had statistically significantly shorter treatment time (p = 0.0412). Injection of PRP into regenerate bone might be an effective method to shorten treatment time during limb lengthening and lead to better functional outcomes and improved patient satisfaction.Level of evidence: Level IV, therapeutic study.     

以骨延长术为主的半侧颜面短小畸形的综合治疗

目的：探讨以下颌骨延长术为主的半侧颜面短小畸形的现代综合治疗。方法：一期行下颌骨延长术,二期手术取出延长器并根据畸形的特点配合相应的辅助手术,术后接受牙科正畸治疗,共14例,结果：14例患者下同骨延长过程顺利,成骨良好,无成骨不良等并发症,二期手术后面部畸形矫正效果满意,结论：矫正下颌骨畸形是治疗半侧颜面 小畸形的关键,骨延长术是治疗下颌骨畸形的有效方法,二期手术还需要配合相应的辅助方法以完成对该畸形的综合治疗。     

Rh-BMP-2 in distraction osteogenesis: Dose effect and premature consolidation

We asked whether locally applied recombinant-bone morphogenic protein-2 (rh-BMP-2) with a type I collagen carrier could enhance the consolidation phase in distraction osteogenesis and whether a dose effect could be reported. We performed unilateral transverse osteotomy of the tibia in 15 immature male rabbits. In Group I (five rabbits), 750 μg of rh-BMP-2 on the type I collagen sponge (Inductos^®, Medtronic) was locally applied on the day of osteotomy; the Group II animals (five rabbits) received 375 μg of the drug and the Group III (control group, five rabbits) had no local application. After 7 days, 3 weeks of distraction was begun at a rate of 0.5 mm/12 h. Starting week 2 of distraction, we assessed radiographic, ultrasonographic, and densitometric parameters once per week. Animals were sacrificed after a 3-week consolidation period. Radiographic evaluation revealed increased regenerate ossification in the rh-BMP-2 groups compared with the control group. The bone mineral content was significantly higher in the rh-BMP-2 treated groups at each time point. A dose effect is shown as densitometric parameters were significantly higher between Groups I and II. 3/5 of the Group I treated animals developed a premature bony union in the regenerate resulting in premature fusion and incomplete distraction.     

The relationship between distraction length and treatment indices during distraction osteogenesis

 To evaluate the indices used to compare the results of limb lengthening with distraction osteogenesis, we analyzed the relationship between the lengthening and external fixation period in a series of 28 patients (30 limbs). The average lengthening was 4.6  cm (range, 2.0–8.9). The average age at operation was 16.6 years (range, 4–47). Limbs treated included 15 femurs and 15 tibias of 19 male and 9 female patients. The external fixation period and the maturation period had a direct linear relationship with length gain. There was a negative hyperbolic relationship between the external fixation index, maturation index, and length gain. Segments that were lengthened 0–3.0  cm had a maturation index of 38.3 days/cm and external fixation index of 57.6 days/cm, both of which were significantly higher than for those lengthened more than 3.0  cm. When comparing the maturation and external fixation indices between different patients or studies, the length of the distraction must be taken into consideration. Received: June 14, 2001 / Accepted: January 7, 2002     

Correction of severe post-traumatic deformities in the distal femur by distraction osteogenesis using Taylor Spatial Frame: a case report

A case of deformity and shortening after post-traumatic growth arrest treated using the Taylor Spatial Frame (Smith & Nephew, Tennessee, USA) is presented. This is the first report showing the application of the frame for post-traumatic deformity in the distal femur, and successful outcomes promise utilization of the frame even for correction of severe deformity in the distal femur.