Unilateral thoracic outlet syndrome in a case with bilateral cervical ribs, does it always produce compression?

BACKGROUND: The cervical rib can be a rare finding in asymptomatic subjects. When it produces compression, it affects variably the vasculo-nervous structures of the brachial plexus; the clinical history is progressive. The imaging studies indicate the site of osteo-neuro-vascular conflict and electrophysiological studies indicate the degree of neural involvement. CASE REPORT: We present a patient with an eight-month history of paresis and right dysesthesias, with an image of bilateral cervical ribs with different anatomical characteristics. This case discusses how a variant of the anatomy produces pathology and when it is not related to compression.

ANTECEDENTES: La costilla cervical puede ser un raro hallazgo en sujetos asintomáticos. Cuando produce compresión, afecta variablemente a las estructuras vasculonerviosas del plexo braquial. El cuadro clínico es progresivo. Los estudios de imagen indican el sitio de conflicto osteoneurovascular, y los electrofisiológicos, el grado de afección neural. CASO CLÍNICO: Presentamos un paciente con historia de 8 meses de paresia y disestesias derechas, con imagen de costillas cervicales bilaterales con diferentes características anatómicas. Con este caso se discute cómo una variante de la anatomía produce patología y cuando no está relacionada a compresión.

Cervical rib which resembles original thoracic rib presenting with thoracic outlet syndrome (TOS): literature review with a case report / Costilla cervical que se asemeja a una costilla torácica presentándose con síndrome de salida torácica (SST): revisión de la literatura a partir de un caso clínico

The cervical rib (CR) is a rare skeletal anomaly, which generally articulated with the transverse process of the 7th cervical vertebra, and commonly lead to compression of neurovascular structures in the region of the thoracic outlet. CRs are divided into 2 classes as complete and incomplete forms. A clarifying description of the so-called complete CR form has not been found with sufficient information in the literature. We aimed to present a novel case of an anomalous, supernumerary, extra, or additional rib which arises from the seventh cervical vertebra. We present the case of a 23-year-old female who presented with a mass described as slowgrowing since her childhood in the supraclavicular region. The patient complained of pain, numbness, weakness, and difficulty in lifting her right arm, which increased gradually over in the last 6 months. Physical examination revealed findings of thoracic outlet syndrome (TOS). Radiographic analysis demonstrated a huge cervical rib, which resembles the size of a real thoracic rib. The cervical rib was completely resected through the supraclavicular approach. There is not enough data in theliterature about different morphologic properties of CRs. It is presented with 3-D CT images before and after surgical resection. The final version of the transformation of C7 transverse process to an original Thoracic Rib is shown. As a result, the following question presented, can it be called a Zeroth Rib?.

La costilla cervical (CC) es una anomalía esquelética rara, que generalmente se articula con el proceso transverso de la séptima vértebra cervical y generalmente conduce a la compresión de estructuras neurovasculares en la región de salida torácica. Las CC se dividen en 2 clases, como formas completas e incompletas. No se ha encontrado una descripción aclaratoria de la forma completa de CC, con información insuficiente en la literatura. El objetivo de este trabajo fue presentar un nuevo caso de costilla anómala, supernumeraria, extra o adicional que surge de la séptima vértebra cervical. Exponemos el caso de una mujer de 23 años que presentó una masa descrita como de crecimiento lento desde su infancia en la región supraclavicular. La paciente relató dolor, entumecimiento, debilidad y dificultad para levantar el miembro superior derecho, con un aumento gradual de sus síntomas en los últimos 6 meses. El examen físico reveló hallazgos del síndrome de salida torácica (SST). El análisis radiográfico demostró una costilla cervical de tamaño importante, que se asemejaba al tamaño de una costilla torácica real. La costilla cervical fue resecada completamente a través de un abordaje supraclavicular. No hay suficientes datos en la literatura sobre las diferentes características morfológicas de las CC. Se presentan imágenes tridimensionales de tomogracía computarizada, antes y después de la resección quirúrgica. Se muestra la versión final de la transformación del proceso transverso de C7 a una costilla torácica original. Como resultado, se plantea la siguiente pregunta, ¿se puede denominar a esta costilla como "costilla cero"?.

Clinical and cytogenetic features of a Brazilian sample of patients with phenotype of oculo-auriculo-vertebral spectrum: a cross-sectional study.

CONTEXT AND OBJECTIVE: Oculo-auriculo-vertebral spectrum (OAVS) is considered to be a defect of embryogenesis involving structures originating from the first branchial arches. Our objective was to describe the clinical and cytogenetic findings from a sample of patients with the phenotype of OAVS. DESIGN AND SETTING: Cross-sectional study in a referral hospital in southern Brazil. METHODS: The sample consisted of 23 patients who presented clinical findings in at least two of these four areas: orocraniofacial, ocular, auricular and vertebral. The patients underwent a clinical protocol and cytogenetic evaluation through high-resolution karyotyping, fluorescence in situ hybridization for 5p and 22q11 microdeletions and investigation of chromosomal instability for Fanconi anemia. RESULTS: Cytogenetic abnormalities were observed in three cases (13%) and consisted of: 47,XX,+mar; mos 47,XX,+mar/46,XX; and 46,XX,t(6;10)(q13; q24). We observed cases of OAVS with histories of gestational exposition to fluoxetine, retinoic acid and crack. One of our patients was a discordant monozygotic twin who had shown asymmetrical growth restriction during pregnancy. Our patients with OAVS were characterized by a broad clinical spectrum and some presented atypical findings such as lower-limb reduction defect and a tumor in the right arm, suggestive of hemangioma/lymphangioma. CONCLUSIONS: We found a wide range of clinical characteristics among the patients with OAVS. Different chromosomal abnormalities and gestational expositions were also observed. Thus, our findings highlight the heterogeneity of the etiology of OAVS and the importance of these factors in the clinical and cytogenetic evaluation of these patients.

Clinical presentation of cervical ribs in the pediatric population.

Cervical ribs may cause thoracic outlet syndrome in adults, but symptoms are poorly described in children. In our series, 88.8% of the 322 children were asymptomatic. The most common symptoms were neck mass and pain. Useful diagnostic tools were cervical spine and chest radiographs. Differential diagnosis of a supraclavicular mass includes cervical ribs.