A case report of child hemiaxial limb skeletal Ollier disease / 中华实用儿科临床杂志

The clinical data of rare hemiaxial limb skeletal Ollier disease in a child admitted to the Affiliated Hospital of Zunyi Medical University in July 2020 were analyzed retrospectively.The literature was reviewed and the methods of diagnosis and treatment of Ollier disease in children were summarized.The patient is a 3-year-old boy, who was hospitalized for 2 days of claudication of the right lower limb.Imaging examination showed hemiaxial limb ske-letal disease and suggested the possibility of histiocytosis.Curettage, bone graft and plaster external fixation were performed on the lesions of the right femoral neck and greater trochanter.The postoperative pathological results indicated endogenous chondroma.The follow-up results revealed that the bone graft healed well, the symptoms of claudication were improved, and there were no complications such as infections and femoral head necrosis.Long tubular bones are the main site of children′s Ollier disease, but multiple lesions in hemiaxial limbs are extremely rare.It is difficult to diagnose this rare case, which is easily misdiagnosed.At present, it′s diagnosis still needs to be confirmed by pathological examination.Surgical treatment and long-term follow-up are needed for children with a wide range of lesions, seriously impaired limb function and obvious limb deformity.

Tricortical-allobone Grafting in Screw Fixation for Intra-articular Calcaneal Fracture via Ollier Approach / 대한족부족관절학회지

PURPOSE: Bone grafting is often necessary to maintain a reduction and prevent delayed collapse of reduced fracture in a treatment of severely displaced comminuted intra-articular calcaneal fractures. Herein, we analyzed the usefulness and necessary conditions to perform tricortical-allobone grafting in open reduction of calcaneal fracture via the Ollier approach. MATERIALS AND METHODS: We performed a retrospective review of 57 intra-articular calcaneal fractures that underwent an operation via the Ollier approach between April 2009 and April 2015. They were divided into two groups: Group 1 (n=17) included those with tricortical-allobone grafts underneath the posterior facet fragment, and group 2 (n=40) included cases without a bone graft. We measured the Böhler angle, Gissane angle, height, and width of the calcaneus at preoperative, postoperative, and final follow-up radiograph. We measured the sagittal rotational angle of the posterior facet fragment of preoperative computed tomography to analyze the effect and necessary conditions for bone grafting. We also reviewed the clinical results by the American Orthopaedic Foot and Ankle Society (AOFAS) scale, visual analogue scale (VAS), and any complications. RESULTS: According to the Sanders classification, there were 3 type-II fractures, 12 type-III fractures, and 2 type-IV fractures in Group 1; whereas in Group 2, there were 26 type-II fractures, 13 type-III fractures, and 1 type-IV fracture (p=0.002). Regarding the preoperative radiologic parameters, there were significant differences in the Böhler angle (p=0.006), Gissane angle (p=0.043), and rotational angle of the posterior facet fragment (p=0.001). No significant difference was observed in the preoperative calcaneal height and width, as well as postoperative radiologic parameters. There was no significant clinical difference between the two groups (p=0.546). CONCLUSION: We suggest that a tricortical-allobone graft may be useful in open reduction and screw fixation via the Ollier approach for displaced intra-articular calcaneal fracture with a bony defect after reduction of collapsed posterior facet fragment. This graft can contribute to the stable reduction via a small approach, even without a plate.

ABNORMALITY IN ENCHONDRAL OSSIFICATION ENCHONDROMATOSIS (A Rare Severe Clinical case with Embryological and Radiological perspective).

In humans sometimes abnormal intracartilaginous mode of ossification can lead to formation of a bony lesion referred to as Enchondroma. Enchondroma can present as a solitary lesion that involves metacarpals, metatarsals, and phalanges in most of the cases or as multiple lesions. We present a case of a 28 year old male who presented with pain in his forearm and digits on both sides. The pain was of mild nature .Rest of the general and skeletal survey was normal. He was followed with radiographs which revealed multiple enchondromata bilaterally. On history there was no familial history of similar or related disease.

Sarcoidosis en pediatría: reporte de 7 casos / Pediatric sarcoidosis: a report of seven cases

La sarcoidosis es una enfermedad granulomatosa crónica de origen no infeccioso que puede comprometer diversos órganos. Su prevalencia es baja, y en Colombia se han reportado casos de manera aislada. Su etiología y fisiopatología aún no se conocen completamente. La presentación clínica y las diferentes manifestaciones de la enfermedad son variables. Cuando su debut se presenta en niños menores de 5 años se denomina sarcoidosis de inicio temprano, mientras que cuando lo hace en niños mayores de 5 años recibe el nombre de sarcoidosis de inicio tardío. En este reporte de caso se presentan 7 pacientes pediátricos, de los cuales 5 correspondieron a sarcoidosis de inicio temprano y 2 a sarcoidosis de inicio tardío. Todos los pacientes tuvieron un diagnóstico tardío de la enfermedad, manifestaciones de varios órganos y sistemas, y recibieron tratamiento inmunosupresor. Cuatro tuvieron curso crónico, 2 remisiones de la enfermedad y 1 recaídas frecuentes. Fue llamativa una asociación poco usual de 2 pacientes con sarcoidosis de inicio temprano quienes adicionalmente presentaron la enfermedad de Ollier.

Sarcoidosis is a chronic granulomatous disease of non-infectious origin which can involve various target organs. Its prevalence is low and there have been only isolated cases reported in Colombia. Its etiology and pathophysiology are not well known. The clinical presentation and signs of the disease vary. When its onset is before five years of age it is recognized as early onset sarcoidosis, while if its onset is after five years of age it is called late onset sarcoidosis. In this case report all patients had a delayed diagnosis and they presented with a multiple organ involvement which required an immunosuppressive treatment. Of the 7 patients, 4 had a chronic course, 2 had remission, and 1 with frequent relapses of the disease. There was an unusual relationship of two patients with early onset disease who additionally presented with Ollier's disease.

Encondromatosis múltiple [Sindrome de Ollier] / Multiple enchondromatosis

Enchondromatosis or Ollier syndrome is defined by the presence of multiple enchondromas with an asymmetrical distribution of low prevalence. Enchondromas are common intra osseous benign cartilage tumors cartilaginous which develop to close proximity growth plate cartilage. Cartilage injuries can be very variable in terms of size, number, location, evolution of enchondroma, age of onset and of diagnosis, requirement for surgery. Clinical problems caused by enchondromas include skeletal malformations, an asymetrics hortening of extremity with limping, and potential risk of malignant change to chondrosarcoma. The condition in which multiple enchondromatosis is associated to soft tissue hemangiomas is known as Maffucci syndrome. So far, both Ollier disease and Maffucci syndrome have occurred only in isolated cases. It has not been established if the disease depends on a single gene or combination of several mutations. The diagnosis is based on clinical and radiological conventional analysis. Histological analysis has a limited role and is used if malignancy is suspected. There is no medical treatment for enchondromatosis. Surgical treatment is recommended only in case of complications. Though, it is difficult to establish a prognosis for Ollier disease, it is found that the early onset forms are usually more severe.

Enfermedad de Ollier: a propósito de un caso / Ollier's disease: a purpose of a acase

Enfermedad de Ollier o encondromatosis múltiple, 1 transtorno infrecuente caracterizado por masas cartilaginosas itraoseas asimétricas, 2 aparecen en la infancia no hereditaria, 3 tiene predominio unilateral principalmente manos y pies, 4 radiológicamente se observan estrías, máximas en metafisis. Existe riesgo de generación sarcomatosa a condrosarcomas (30 por ciento). Se presenta caso; femenina de 6 años con afectación de los cuatro miembros, deformidad, disfuncionalidad y dolor en las manos, limitación de actividades físicas. Se manejo con resección de encondromas en humero proximal, radio distal, F1 anular, F2 de do medio y fèmur distal del lado derecho, igualmente tibia proximal izquierda; evolucionando sin residivas y autolimitaciones de las lesiones.

Treatment of Displaced Intra-articular Calcaneal Fracture using Ollier Approach / 대한족부족관절학회지

PURPOSE: We evaluated the clinical results of the intraarticular calcaneal fractures treated using Ollier approach by inexperienced orthopaedic surgeon. MATERIALS AND METHODS: Between August 2003 and May 2007, Of the total 46 cases, 12 cases (9 patients) of displaced intraarticular calcaneal fracture who underwent open reduction and internal fixation using Ollier approach were evaluated. The means of age was 50.5 years. According to the Sanders classification, there was no type I case and 8 cases of type II, 1 case of type III, and 3 cases of type IV. We evaluated the treatment result by assessing radiologic parameters (Bohler angle, Gissane angle, and calcaneal height/width) and clinical outcomes (VAS and AOFAS score). RESULTS: The means of follow-up period was 25.3 months. The means of Bohler angle was improved from 2.4degrees to 26.1degrees. Radiologic and clinical union was achieved in all cases without additional procedures. Excellent result were noted in 2 cases, good in 5 cases, fair in 4 cases, and poor in 1 case. We experienced 2 cases of minor complications; 1 case of mild wound infection and 1 case of hypoesthesia on foot dorsum. Radiologic findings of subtalar arthritis were present in 2 cases. CONCLUSION: Ollier approach seems to be helpful to inexperienced orthopaedic surgeons for the treatment of intraarticular calcaneal fractures in that it enables them to achieve considerable clinical outcomes without serious complications.

Maffucci' s syndrome complicated by intracranial chondrosarcoma: two new illustrative cases

Maffucci's syndrome is a rare congenital condition, sometimes misdiagnosed as Ollier's disease, characterized by multiple enchondromas combined with hemangiomas and phlebectasia. Coexisting primary malignancies have been described sporadically. We report two cases of Maffucci's syndrome associated with cranial base chondrosarcoma, emphasizing pathophysiological features and the challenging management of intracranial chondrosarcomas. To the best of our knowledge, only twelve similar cases have been reported in the literature.

Síndrome de Maffucci é uma condição congênita rara, às vezes confundida com a doença de Ollier, caracterizada por encondromas múltiplos associados com hemangiomas e flebectasia. A concomitância com neoplasias primárias tem sido relatada esporadicamente. Nós relatamos dois casos de síndrome de Maffucci associada a condrossarcoma da base do crânio, enfatizando aspectos fisiopatológicos e o manejo desafiador dos condrossarcomas intracranianos. Em revisão da literatura, podemos encontrar o relato de apenas doze casos similares.

Enfermedad de Ollier: reporte de un caso / Ollier's Disease: a case report

La enfermedad de Ollier es una encondromatosis múltiple querepresenta el 3 % de los tumores óseos benignos. Las encodromatosisson tumores cartilaginosos que pueden ensanchar el hueso yproducir la clásica característica de la calcifi cación moteada dentrode las lesiones. Se presenta el caso de un escolar masculino de6 años de edad con presencia de múltiples encondromas en lasextremidades torácicas y pélvicas derechas (húmero- radio y cubito,falanges de la mano, fémur, tibia, peroné, y falanges del pie), quehan producido deformidades angulares, rotacionales y asimetría deextremidades pélvicas, que han sido tratadas con osteotomías yfi jación externa tipo Ilizarow a nivel del defecto tumoral en la Unidadde Ortopedia pediátrica del Hospital del Niño de La Paz-Bolivia.

Ollier’s disease is a multiple enchondromatose which constitutes3% of benign bone tumors. Enchondromatoses can thicken thebone and produce the classical characteristics of the cartilaginoustumors: spotted calcifi cations within the lesion. I present the caseof a six year-old school boy who had multiple enchondromes in theright thoracic and pelvic extremities (humero-radius and cubitus,phalanges of the hand, femur, tibia, fi bula, and phalanges of the foot).that had produced angular, rotational deformities and asymmetry ofthe pelvic extremities which had been treated with osteotomy andexternal fi xation according to IIizarow at the level of the tumorousdefect, in the Unit of Pediatric Orthopedics of the Children’s Hospitalin La Paz, Bolivia.