Síndrome del opérculo torácico en adolescentes: serie de casos / Thoracic outlet syndrome in adolescents: a case series

OBJETIVO: El síndrome del opérculo torácico (SOT) está causado por una compresión del plexo braquial y vasos subclavios en su paso hacia la extremidad superior. Patología típica de mujeres entre 20 y 50 años, que es infrecuente diagnosticar en niños. Presentamos nuestros resultados en el diagnóstico y tratamiento del SOT pediátrico. MATERIAL Y MÉTODOS: Estudio retrospectivo de pacientes diagnosticados de SOT entre diciembre de 2017 y junio de 2018. Se analizaron variables clínicas, radiológicas, quirúrgicas y de evolución. RESULTADOS: Cinco SOT fueron diagnosticados en cuatro pacientes, uno de ellos bilateral. La edad media al diagnóstico fue de 12,5 años (7-15) y hubo una demora en el diagnóstico de 153 días (10-360). SOT venoso (3) y neurológico (2). Presentaron dolor (5/5), edema (4/5), hipoestesia (3/5), disminución de fuerza (3/5) y dolor cervical (2/5). Una paciente presentaba dolor asociado al deporte. El estudio neurofisiológico fue normal en tres casos. Dos pacientes presentaron anomalías óseas por TAC. Se realizaron tres intervenciones quirúrgicas en dos pacientes por abordaje supraclavicular realizando resección de la primera costilla anómala y escalenectomía. Una paciente rechazó la intervención y en otra se mantuvo en una actitud expectante sin reaparición de los síntomas. Seguimiento posoperatorio de 9 meses (6-12) con mejoría progresivas de los síntomas. CONCLUSIÓN: El SOT puede darse en adolescentes, siendo el dolor y edema de la extremidad superior lo más específico. Se recomienda la realización de pruebas de imagen para detectar estructuras anatómicas anómalas. El abordaje supraclavicular se presenta como una técnica segura y eficaz en la descompresión del desfiladero torácico

OBJECTIVE: Thoracic Outlet Syndrome (TOS) is caused by a compression of the brachial plexus and the subclavian vessels in their passage to the upper limb. It mostly occurs in women aged 20-50, so it is infrequent in children. We present our results in the diagnosis and management of pediatric TOS. MATERIALS AND METHODS: Retrospective study of patients diagnosed with TOS between December 2017 and June 2018. Clinical, radiological, surgical, and evolution variables were assessed. RESULTS: Five TOS were diagnosed in 4 patients - one TOS was bilateral. Mean age at diagnosis was 12.5 years (7-15), and there was a delay in diagnosis of 153 days (10-36). TOS was either venous (3) or neurogenic (2). Patients presented with pain (5/5), edema (4/5), hypoesthesia (3/5), decreased strength (3/5), and cervical pain (2/5). One patient presented with sport-related pain. Neurophysiological study was normal in three cases. Two patients presented bone anomalies at CT-scan. Three surgeries were performed in two patients using the supraclavicular approach with resection of the anomalous first rib and scalenectomy. One patient refused surgery, and another patient remained expectant without reappearance of symptoms. Postoperative follow-up was 9 months (6-12), with progressive improvement of symptoms. CONCLUSIONS: TOS may occur in adolescents in the form of upper limb pain and edema. Imaging tests are recommended to detect abnormal anatomical structures. The supraclavicular approach represents a safe and effective technique in decompressing the thoracic outlet

Costilla accesoria cervical como causa de síndrome de apertura torácica superior. Nuestra experiencia de los últimos diez años / Cervical accessory rib, as a cause of thoracic oulet syndrome (tos): our experience of the last ten years

El síndrome de apertura torácica superior por costilla cervical, es una patología no muy frecuente, ocasionada por la persistencia después del nacimiento de la última costilla cervical que debería haberse reabsorbido. La patología que origina, es comprensiva de las estructuras vecinas en especial nerviosas, pero también vasculares. Se manifiesta en pacientes jóvenes después de la adolescencia con una especial incidencia en el sexo femenino. El diagnóstico es sencillo si se piensa en esta causa como la etiológica del síndrome, con la realización de una simple radiografía de base de cuello para evidenciar su presencia, aunque es posible realizar otras complementarias. El tratamiento es la simple extirpación de la costilla que en la mayoría de las ocasiones resuelve el cuadro clínico. Se analiza en la publicación la experiencia de nuestro servicio hospitalario en los últimos diez años, analizando aspectos demográficos, diagnósticos, terapéuticos y los resultados obtenidos

The syndrome of superior thoracic opening due to cervical rib is a not very frequent pathology, caused by the persistence after birth of the last cervical rib that should have been reabsorbed. The pathology that it originates is comprehensive of neighboring structures, especially nervous, but also vascular. It manifests itself in young patients after adolescence with a special incidence in the female sex. The diagnosis is simple if we think of this cause as the etiological cause of the syndrome, with a simple neck base X-ray to show its presence, although it is possible to perform other complementary ones. Treatment is the simple removal of the rib, which in most cases resolves the clinical picture. The experience of our Hospital Vascular Service in the last ten years is analyzed in the publication, analyzing demographic, diagnostic and therapeutic aspects and the results obtained

Síndromes compresivos vasculares: un desafío diagnóstico / Vascular Compressive Syndromes: A Challenge Diagnosis

Resumen Los síndromes de compresión vascular constituyen un grupo de patologías poco frecuentes con prevalencia incierta, no solo por el desconocimiento que se tiene de estos, sino también por su presentación clínica inespecífica. Eso conlleva a que sean infradiagnosticados o detectados en etapas tardías, con secuelas a largo plazo. Su fisiopatología y clínica dependen del tipo de vaso comprometido, el sitio anatómico afectado y la causa subyacente que lo esté produciendo. La anamnesis, el examen físico dirigido y el uso de métodos complementarios como la ultrasonografía Doppler y la angiografía por tomografía computarizada (ATC) o angiografía por resonancia magnética (ARM), son herramientas vitales para su correcto diagnóstico, planificación terapéutica y seguimiento.

Abstract Vascular compression syndromes constitute a group of rare pathologies with uncertain prevalence not only because of their unawareness, but also for its nonspecific clinical presentation. This leads to their underdiagnosis, or detection in late stages with long-term sequelae. Its pathophysiology and presentation depends on the type of vessel involved, the affected anatomical site and the underlying cause producing it. Clinical history, physical examination and use of complementary methods such as Doppler ultrasonography, Computed Tomography Angiography (CTA) or Magnetic Resonance Angiography (MRA), are essential tools for its proper diagnosis, therapeutic planning and follow-up.

Síndrome de la costilla deslizante. Serie de 4 casos clínicos / No disponible

Introducción: El síndrome de costilla deslizante (SCD) es una entidad poco frecuente en niños. Se cree que su causa es una debilidad en los ligamentos costales que permite una hipermovilidad de las costillas. Genera un dolor intermitente en la región baja del tórax o alta del abdomen que puede afectar a las actividades de la vida diaria o generar un dolor crónico. Material y métodos: Revisión retrospectiva de SCD entre octubre de 2012 y diciembre de 2017. Se recogió información acerca de los datos demográficos, síntomas, estudios de imagen, hallazgos intraoperatorios, material fotográfico y seguimiento a largo plazo. Resultados: Durante este periodo, 4 pacientes fueron diagnosticados de SCD. La mediana de edad al diagnóstico fue de 13 años (12-15 años) con una duración previa de los síntomas de 13 meses (12-36 meses). En 2 pacientes se asoció una dismorfia costal (DC). El diagnóstico fue clínico con confirmación ecográfica. Se realizó resección de los cartílagos afectos en 3 pacientes con un seguimiento posterior de 6 meses (3-30 meses). Actualmente se encuentran sin dolor y con un resultado estético satisfactorio. Un paciente rechazó la intervención. Conclusiones: El SCD aparece en pacientes preadolescentes que en algunos casos asocian DC. Una exploración física y ecografía enfocada son las claves para un diagnóstico certero. La resección de cartílagos es efectiva a largo plazo

Aim of the study: The slipping rib syndrome (SRS) is an unknown pathology for the pediatric surgeon due to its low incidence in children. The weakness of the costal ligaments allowing an area of rib hypermobility has been postulated recently as the main etiology. It produces an intermittent pain in the lower thorax or upper abdomen that can affect to the daily activities and can be the origin of unspecific chronic pain. Methods: A retrospective review of patients diagnosed with SRS between october 2012 and march 2017 was performed. Data of demographics, symptoms, imaging studies, surgical findings and long-term follow-up were collected. Results: During this period, 4 patients were diagnosed with SRS. Median age at diagnosis was 13 years (12-15 years) with a mean duration of symptoms of 13 months (12-36 months). In 2 patients the SRS was associated with Costal Dysmorphia (CD). The initial diagnosis was clinical with posterior ultrasound confirmation. Resection of the affected cartilages was performed in 3 patients and after a follow-up of 6 months (3-30 months), they all are painless and refer a good cosmetic result. One patient refused the intervention. Conclusions: The SRS is an infrequent cause of thoracic pain with an etiology not well understood. The awareness of this disease and its typical presentation can avoid unnecessary studies. The resection of the affected cartilages is a safe and effective treatment

Modified Interscalene Approach for Resection of Symptomatic Cervical Rib: Anatomic Review and Clinical Study.

BACKGROUND: Cervical ribs have been reported to be present in about 0.5% of the general population, 10% of patients with cervical rib who are symptomatic usually have neurogenic symptoms, but some have arterial symptoms. In 1861, Coote was the first to excise a cervical rib through a supraclavicular approach and relieved the symptoms of thoracic outlet syndrome. OBJECTIVE: In this study, we address the efficacy and safety of a modification to the supraclavicular approach for resection of symptomatic cervical ribs. PATIENTS AND METHODS: The surgical team in collaboration with an anatomist performed cadaveric dissections of the posterior triangle of the neck in the Department of Anatomy, Ain Shams University. A prospective study was performed of 25 patients with moderate to severe neck or upper limb pain; this pain was resistant to medical treatment for at least 6 months. Preoperative cervical radiography showed cervical ribs. Pain was assessed by using the visual analog scale. Electrophysiologic tests were performed to confirm the diagnosis. In this study, we performed a modified supraclavicular interscalene approach with resection of the symptomatic rib and without resecting either of the scalene muscles or the first thoracic rib. RESULTS: A total of 25 patients were included in this study; the mean age was 36 years (± 12 standard deviation), and the mean follow-up period was 12.3 months. All patients had moderate (28%) to severe (72%) preoperative pain. Motor deficits were present in 6 cases (24%); Sensory manifestations were present in 80%. All patients had a relief of severe pain at the first postoperative visit in the first week. There were improvements in the motor power in 5 of the 6 patients who had preoperative motor deficit. CONCLUSIONS: A modified supraclavicular interscalene approach for resection of symptomatic cervical ribs has been shown to be effective in the treatment of neuralgic pain. Compared with other approaches, it proved to be less invasive, with small transverse incision and without resection of scalenus anterior muscle.

The prevalence of cervical ribs in Enugu, Nigeria.

BACKGROUND: Cervical rib is an important cause of nontraumatic thoracic outlet neurovascular compression. This study was undertaken as there is no known documented report on its prevalence in the study environment. AIMS: To evaluate the prevalence of cervical ribs and its variation with sex and age among patients presenting at radiological facilities for a chest radiograph. SETTINGS AND DESIGN: Retrospective evaluation of plain posterior-anterior view chest radiographs done in radiological facilities in Enugu, Southeast, Nigeria. SUBJECTS AND METHODS: Evaluated in this study were all 6571 chest radiographs consecutively obtained between 2009 and 2012 in three randomly selected radiological facilities in Enugu. STATISTICAL ANALYSIS USED: SPSS version 17 software was used in data analysis. Chi-square and student t-tests were used to test for the significance of findings at 95% confidence level. RESULTS: This study reports the overall prevalence of cervical ribs as 48 (0.7%) with a significantly higher rate in females 43 (1.1%) when compared to males 5 (0.2%) (P = 0.000). In 27 (0.4%) cases, the cervical ribs were bilateral; whereas in 21 (0.3%) cases, they were unilateral with 8 (0.1%) on the left and 13 (0.2%) on the right. There was no age-related variability with prevalence (P = -0.813). CONCLUSIONS: The prevalence of cervical rib in the study population is low and within the known range as seen in other geographical regions of Nigeria.

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.

[Thoracic outlet syndrome in a child: a rare pediatric diagnosis]. / Syndrome du défilé thoraco-brachial par côte surnuméraire : un diagnostic rare en pédiatrie.

Thoracic outlet syndrome (TOS) is a rare pediatric syndrome and few cases have been reported in the literature. The authors report the case of a 10-year-old boy with generalized arm pain and neck stiffness. The angiocomputed tomography revealed the presence of a TOS caused by an additional cervical rib and stenosis of the sub-clavian artery. The resection of this rib relieved the patient of his pain during a full year following surgery. This case reminds us that children can also be affected by TOS. When a cervical rib causes TOS, a resection is often necessary.

Cervical rib with stroke as the initial presentation.

Cervical ribs rarely become symptomatic. Cerebral ischemia or infarct due to cervical rib is extremely rare and, invariably, these patients have a history of upper limb symptoms before presenting with stroke. We report a young boy with cervical rib who presented with stroke. A right sided cervical rib was noted during angiogram, causing mild stenosis and post stenotic dilatation of right subclavian artery distal to the rib. An abduction angiogram showed complete occlusion of the right subclavian artery and visualization of collaterals. Right carotid angiogram also showed evidence of thromboembolic episodes in the right middle cerebral artery territory.

[Falconer-Weddel's costoclavicular syndrome].

Analysed herein are the findings of examination and outcomes of surgical management of fifty-eight patients (25 men and 33 women) presenting with Falconer-Weddel's costoclavicular syndrome in which the subclavian artery and vein and the brachial plexus at the thoracic outlet appear to be compressed. Of the 58 patients,five subjects were found to have a rudimentary cervical rib and three more (5.3%) patients had trophic disorders on their digits fingers: dystrophy of the nail plates, their fragility, periodically opening trophic ulcers at the digital tips and one patient presented with gangrene of the inguinal phalanx of the middle finger. Three patients had hyperemia of the face. A further four patients had roughening, hyperkeratosis of the skin of the hands, cracks. The presence of the supernumerary ribs was determined roentgenologically. Haemodynamics was studied using Doppler ultrasonography making it possible to reveal disordered blood flow in the upper-limb arteries in the physiological position assumed, in the Adson test as well as with Raynaud syndrome, which was observed in 39 patients. The function of the nerves was studied using electroneuromyography (ENMG). All patients were operated on under endotracheal anaesthesia. Decompression-medical operations were carried outperformed in all 58 patients, with the following four types of interventions being performed: transaxillary resection of the first rib combined with sympathectomy carried out in 23 patients, resection of the first rib without sympathectomy in eighteen patients, resection of the first rib via a surpraclavicular approach in four patients, scalenotomy and selective cervicothoracic sympathectomy in 13 patients.

Thoracic outlet syndrome associated with a large cervical rib.

This case illustrates the anatomic impact of a large cervical rib as an etiology for thoracic outlet syndrome. Current management remains predicated on astute diagnosis, multidisciplinary therapy including, physical therapy, scalene muscle blockade, and surgical decompression in appropriate cases.

A "hard" neck lump.

We discuss a case of an asymptomatic cervical rib presenting as a hard neck lump in a 56-year-old woman. Cervical rib is a common condition associated with thoracic outlet syndrome. The diagnosis, investigation and management of this condition is an important differential diagnosis that an ENT surgeon should know in a patient presenting with a neck mass.

The prevalence of cervical ribs in a London population.

Cervical ribs are an important cause of neurovascular compression at the thoracic outlet. Previous studies have shown the prevalence of cervical ribs to be between 0.05 and 3%, depending on the sex and race of the population studied. We examined 1,352 chest radiographs to determine the prevalence of cervical ribs in a London population of mixed sex and ethnicity. Our study found that the overall prevalence of cervical ribs was 0.74% with a higher rate in females compared with males (1.09 and 0.42%, respectively). Of the 10 individuals with a cervical rib, five were on the left, three were on the right and two were bilateral. The presence of elongated C7 transverse processes (transverse apophysomegaly) was also noted. We found a total of 30 elongated transverse processes with an overall prevalence of 2.21%. They were also more common in females (3.43%) than males (1.13%).

[The use of intravascular nitinol stents in the treatment of subclavian vein compression for thoracic outlet syndrome]. / Zastosowanie samorozprezalnych nitynolowych stentów naczyniowych w powiklaniach ucisku tylnego w przebiegu zespolu uciskowego górnego otworu klatki piersiowej.

AIM: The purpose of this study is to evaluate the multimodal treatment (thrombolysis, anticoagulation and surgical decompression) of subclavian-axillary vein thrombosis (Paget-Schroetter syndrome) and possibility of shortening of time of therapy. MATERIAL AND METHODS: In this paper 23 patients with Paget-Schroetter syndrome in Department of Vascular, General and Transplantological Surgery is presented. Thrombolysis with rt-PA, anticoagulation with heparin and intra venous angioplasty with the use intravascular stent, and operation with resection of the first rib in the treatment of subclavian vein thrombosis was used. During the follow up period the patients were divided into two groups: group I (13 patients) with traditional surgical decompression after 3-4 months (potential risk of hemorage) and group II (10 patients) with early surgical treatment (median 8 days). Long-term follow-up was obtained by chart review and asking patients to complete the DASH (Disabilities of the Arm, Shoulder and Hand) questionnaire that was developed by the American Academy of Orthopedic Surgeons. RESULTS: Complete revascularization with venous thrombolysis was achived in all patients. Decompresion with transaxillary resection of first rib and venous revascularization were performed in the same procedure in all patients. Statistical difference were not found in both groups. Most patients report no disability of upper limb at work and sport activity. CONCLUSIONS: Multimodal treatment of Paget-Schroetter syndrome (thrombolysis, the use of intravascular stents and early thoracic outlet decompression) can be used as a optimal of therapeutic method to subclavian vein thrombosis. The advantages of immediate surgical treatment are a promotion of rapid resumption of athletic activities.

Bilateral neurogenic thoracic outlet syndrome.

We report a case of bilateral neurogenic thoracic outlet syndrome (TOS). Electrophysiological examination suggested the presence of bilateral lower brachial plexus neuropathy. Radiography showed rudimentary bilateral cervical ribs. In the cases reported in the literature to date, the clinical findings are typically unilateral despite the common presence of bilateral bony abnormalities. Neurogenic TOS should be considered in young women, even if they present with bilateral symptoms, when they have occupations requiring strenuous activity of the upper limbs.