¿Cuándo pensar en pseudoartrosis congénita de clavícula en pediatría? Presentación de 2 casos / When to think about pediatric congenital pseudoarthrosis of the clavicle? Presentation of 2 cases

La pseudoartrosis congénita de la clavícula es una malformación rara y benigna, caracterizada por la ausencia del tercio medio de la clavícula. Suele ser unilateral y mayoritaria en el lado derecho. La etiología es desconocida y se postulan diversas teorías etiopatogénicas (vascular, embriológica y genética).Puede detectarse en el período neonatal o, más frecuentemente, durante la infancia. En ocasiones, puede ser sintomática. Puede requerir tratamiento mediante reconstrucción quirúrgica por injerto óseo.Se presentan 2 casos, uno de diagnóstico neonatal y otro de 3 años de edad realizados con 24 h de diferencia. Se destaca la consideración de este diagnóstico como diferencial de fractura obstétrica o postraumática, displasia cleidocraneal y neurofibromatosis de tipo 1.

The congenital pseudoarthrosis of the clavicle is a rare and benign malformation, characterized by the absence of the middle third of the clavicle. It is usually unilateral and the majority on the right side. The etiology is unknown, postulating diverse etiopathogenic theories (vascular, embryological and genetic).It can be detected in the neonatal period or, more frequently, during childhood. Occasionally it can be symptomatic. It may require treatment by surgical reconstruction by bone graft. Two cases are presented, one of neonatal diagnosis and another one of 3 years of age performed with 24 hours of difference. We emphasize on its consideration as a differential diagnosis of obstetric or post-traumatic fracture, cleidocranial dysplasia and neurofibromatosis type I.

Compressão da artéria subclávia por pseudoartrose de clavícula: apresentação na quinta década de vida / Late presentation of critical upper limb ischemia caused by pseudarthrosis of the clavicle

A compressão da artéria subclávia no desfiladeiro torácico é um fenômeno amplamente conhecido. Anormalidades ósseas, como a pseudoartrose da clavícula, podem raramente causar compressão arterial a esse nível. A pseudoartrose pode desenvolver-se em decorrência de um trauma, que é a forma mais comum, ou ser congênita. Os autores descrevem o caso de uma paciente de 44 anos com quadro de isquemia crítica de membro superior direito. Apresentava história de fratura não tratada de clavícula direita aos 9 meses de idade que evoluiu com pseudoartrose e compressão extrínseca com oclusão da artéria subclávia. O segmento da clavicula acometido pela pseudoartrose foi ressecado e realizada uma tromboembolectomia tardia das artérias subclávia, braquial e distais, com boa evolução.

Compression of the subclavian artery in the thoracic outlet is a well-known phenomenon. In rare cases, bone abnormalities, such as pseudarthrosis of the clavicle, can cause arterial compression at this level. Pseudarthrosis may develop as a result of trauma, which is the more common form, or it may be congenital. Here, the authors describe the case of a 44-year-old patient with critical ischemia of the right upper limb. She had a history of untreated right clavicle fracture at 9 months of age which had progressed to pseudarthrosis and extrinsic compression of the subclavian artery causing occlusion. The segment of the clavicle involved was resected and late thromboembolectomy of the subclavian, brachial, distal arteries was performed, with good results.

Coracoclavicular and costoclavicular joints at a common juncture: a rare phenomenon / Articulaciones coracoclavicular y costoclavicular en una unión común: un raro fenómeno

Movements at the human shoulder girdle are the result of complex interplay of glenohumeral, acromioclavicular, sternoclavicular and scapulothoracic articulations. Clavicle apart from articulating with the scapula and sternum is also connected with first rib by costoclavicular ligament and with coracoid process by coracoclavicular ligament. At times the area of attachment of these ligaments on clavicle, first rib and scapula show faceted apophysis suggesting the presence of additional diarthrodial articulations. Costoclavicular joint exists between clavicle and first rib and coracoclavicular joint between clavicle and coracoids process. Both these joints are described in the literature, but the concurrent occurrence of them in the same bone has not been reported yet. We found two clavicles, one of right and other of left side, both of them showed faceted apophysis for costoclavicular and coracoclavicular joint simultaneously, which is rare phenomenon.

Los movimientos de la cintura escapular humana son el resultado de la interacción compleja de las articulaciones glenohumeral, acromioclavicular, esternoclavicular y escapulotorácica. La clavícula, aparte de la articulación con la escápula y el esternón también está conectada con la primera costilla por el ligamento costoclavicular y con el proceso coracoides por el ligamento coracoclavicular. A veces la zona de unión de estos ligamentos de la clavícula, la primera costilla y la escápula muestran procesos facetarios que sugieren la presencia de nuevas articulaciones diartrodiales. La articulación costoclavicular existe entre la primera costilla y la clavícula y la articulación coracoclavicular entre la clavícula y proceso coracoides. Ambas articulaciones están descritas en la literatura, pero la aparición simultánea de ellas en el mismo hueso no se ha informado aún. Se encontraron dos clavículas, uno del lado derecho y otra del lado izquierdo, las dos presentaron procesos facetarios para las articulaciones costoclavicular y coracoclavicular simultáneamente, lo cual es un fenómeno raro.

Pseudoartrose congênita da clavícula / Congenital pseudartrosis of the clavicle

A pseudoartrose congênita da clavícula é uma doença rara de etiologia desconhecida. Trata-se de uma patologia não familiar, em geral unilateral, com alta predominância pelo lado direito e pelo sexo masculino. Foi descrita inicialmente por Fitzwillians em 1910. Existem até o momento aproximadamente 200 relatos de caso na literatura. O diagnóstico diferencial inclui a pseudoartrose pós-traumática (tocotraumatismo), a disostose cleidocranial, a osteogênese imperfeita e a neurofibromatose. Os autores apresentam um paciente, masculino, de 28 dias de vida, com uma massa indolor situada no terço médio da clavícula direita.

Congenital pseudartrosis of the clavicle is a rare disease of unknown etiology. It is a non-familial disorder which is usually unilateral, with a high prevalence for the right side and males. It was first reported by Fitzwillians in 1910. There are up to now about 200 case reports in the literature. The differential diagnosis includes post-traumatic pseudartrosis (birth trauma), cleidocranial disostosis, imperfect osteogenesis, and neurofibromatosis. The authors report the case of a 28-day old male patient with a painless mass at the medium third of the right clavicle.

Pseudoartrosis congénita de la clavícula: reporte de un caso / Congenital pseudarthrosis of the clavicle: a case report

La pseudoartrosis congénita de la clavícula (PCC) es una rara anormalidad de la cintura escapular de etiología desconocida. Afecta principalmente a niñas, es más frecuente en la clavícula derecha, generalmente es asintomática y suele ser un hallazgo casual cuando se solicita una radiografía del tórax. El tratamiento es por lo común no quirúrgico a menos de que exista compromiso funcional o estético del hombro afectado.

Mandibuloacral dysplasia in an Iranian girl

Mandibuloacral dysplasia [MAD] is a rare autosomal recessive syndrome. Less than 25 families have been reported, most of which are Italian. Here, we describe a new patient of Iranian origin, born to consanguineous parents

Cleidocranial dysplasia syndrome in a newborn

Cleidocranial dysplasia is a rare congenital bone formation inherited as an autosomal dominant trait. The incidence is one per 200000 live births. Major symptoms include delayed ossification of the skull, excessively large fontanels and clavicular hypoplasia or aplasia, allowing the shoulders in front of the chest. Delayed eruption of teeth, high arched palate and wide pelvic joint may also be present. The case was a newborn boy who had admitted in neonatal ward of Ekbatan hospital because of respiratory distress. Anterior and posterior fontanels were completely large and connected. His shoulders were a little depressed. Chest X ray revealed bilateral clavicular agenesia and skull X-ray failed to demonstrat vortex bone

Free fibular graft in treatment of congenital pseudarthrosis of the clavicle [case report]

Congenital pseudarthrosis of the clavicle is a rare entity, a little more than one hundred cases have been reported in the English literature [1, 2, 3]. This article describe a 7 year-old boy presented with a painless lump in the right clavicular region. Clinical, radiological and histological studies proved to be congenital pseudarthrosis of the clavicle. A well- developed right cervical rib was observed. The case was treated by excision of the pseudarthrosis, a free fibular graft and fixation with a controured 1/3 tubular. AO plate. Solid union was achieved at 5 months postoperatively. Histological examination of the excised ends of the pseudarthrosis showed enchondral ossWcation which supports the hyposthesis that the pseudarthrosis resultsfromfailedfusion of two ossification centers. The final result was satisfactory from the functional and cosmetic points of view after five years of follow-up and there were no signs of complications

Displasia mandíbulo acral en un paciente pediátrico mexicano / Mandibulo acral dysplasia in a Mexican children

Introducción. La displasia mandíbulo acral (DMA) es una rara enfermedad autosómica recesiva, originalmente descrita en 1917. A la fecha se han descrito aproximadamente 20 casos, de los cuales la mayoría son de origen italiano. Caso clínico. Se presenta al primer paciente pediátrico mexicano comparando los hallazgos clínicos y radiológicos de este caso con los encontrados en la literatura y con las entidades que deben considerarse en el diagnóstico diferencial tales como: progeria, acrogeria, displasia cleidocraneal, picnodisostosis, Hallerman-Streiff, Hadju-Cheney síndrome de Werner. Conclusiones. Aunque la DMA tiene una frecuencia baja, en la práctica debe considerarse esta entidad en aquellos pacientes con hipoplasia mandibular o alteraciones dermatológicas como hiperpigmentación moteada

Incidence of an articular facet on conoid tubercle of adult Pakistani clavicles

Presence of an articular facet on the conoid tubercle of clavicles the existence of coracoclavicular joint. Among 450 Pakistani adult clavicles examined, facet was found present in 52 [11.5%] male and in only 1 [8.3%] female clavicle comparable to the previous observations in Asian 10.1% in males and 8.3% in females clavicles. The most common shape of the facet noticed was oval. Double circular facets were seen in only one left clavicle. The average size of facet measured was 10 x 6 millimeters

Tuberculosis osteocutánea de la región esternoclavicular: presentación de un caso y revisión de la literatura / Sterno clavicular region osteocutaneous tuberculosis: Report of a case and revision of the literature

Una paciente de 61 años de edad presentó una tumoración fluctuante sobre la región esternoclavicular izquierda, pérdida de peso y sensación febril. En los meses previos había desarrollado una adenitis en la región axilar izquierda que se fistulizó. El estudio radiológico demostró lesiones destructivas de la clavícula y secuelas de TBC pulmonar; y, la biopsia de un ganglio suparaclavicular reveló un infiltrado inflamatorio granulomatoso crónico con necrosis caseosa y células de Langhans. Hubo respuesta favorable al tratamiento antituberculoso. Esta forma clínica es muy infrecuente y se revisa la literatura al respecto.