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[肋骨畸形的不寻常表现]

[Unusual presentation of rib malformation].

作者信息

Cosson M-A, Breton S, Aprahamian A, Grevent D, Cheron G

机构信息

Service des urgences pédiatriques, université Paris Descartes, hôpital Necker Enfants-Malades, 149, rue de Sèvres, 75743 Paris cedex 15, France.

出版信息

Arch Pediatr. 2012 Nov;19(11):1208-11. doi: 10.1016/j.arcped.2012.08.023. Epub 2012 Oct 1.

Abstract

Rib malformation and anatomical variations are not well known and are still often underdiagnosed. Usually, rib malformations are fortuitously discovered. We describe here the case of a girl, 4 years and 4 months old, who presented at the emergency unit for fever and an anterior tumefaction of the ribcage, without any other symptoms. She was eupneic with a normal pulmonary auscultation and viral tonsillitis with a negative streptococcus test. The thoracic tumefaction was parasternal, painless, and fixed and measured approximately 2.5 × 2cm. Ultrasound findings consisted of a duplicated and hypoechogenic hypertrophy of the sterno-costal cartilage of the 4th left rib. Magnetic resonance imaging (MRI) confirmed the diagnosis of chondral bifidity of the sterno-costal junction of the 4th left rib. Fever, due to the viral tonsillitis, disappeared after 4 days. Rib malformations are rare, often anterior, unilateral, and preferentially located on the 3rd or the 4th rib. The main malformative rib lesions are bifid ribs, rib spurs, and widened ribs. Very rarely, they can be associated with Gorlin-Goltz syndrome or with other malformations such as VATER complex. The main differential diagnoses of these rib malformations are traumatic, tumoral, and infectious etiologies. In case of tumoral diseases, the topography of the lesion focuses the etiologic diagnosis: whereas an anterior and cartilaginous lesion is always benign, a lateral or posterior lesion can be an Ewing sarcoma. Rib malformation investigation consists in meticulous questioning, a complete clinical examination looking for any associated anomaly, completed by basic imaging explorations such as plain thoracic radiography focused on the ribcage and ultrasound. Finally, complementary computerized tomography or preferably MRI, depending on the anatomic location of the lesion, confirms the final diagnosis, as presented in our case report, and removes any uncertainty.

摘要

肋骨畸形和解剖变异尚不为人熟知,且常常漏诊。通常,肋骨畸形是偶然发现的。我们在此描述一名4岁4个月女童的病例,她因发热和胸廓前部肿胀到急诊就诊,无其他症状。她呼吸正常,肺部听诊正常,患有病毒性扁桃体炎,链球菌检测呈阴性。胸廓肿物位于胸骨旁,无痛,固定,大小约为2.5×2厘米。超声检查发现左侧第4肋胸骨肋软骨重复且回声减低性肥大。磁共振成像(MRI)证实左侧第4肋胸骨肋关节软骨二分畸形的诊断。由病毒性扁桃体炎引起的发热在4天后消退。肋骨畸形很少见,多位于前部、单侧,且优先发生于第3或第4肋。主要的肋骨畸形病变有肋骨二分、肋骨骨刺和肋骨增宽。极少数情况下,它们可能与戈林-戈尔茨综合征或其他畸形如VATER综合征相关。这些肋骨畸形的主要鉴别诊断包括创伤性、肿瘤性和感染性病因。对于肿瘤性疾病,病变的部位有助于病因诊断:前部软骨病变通常为良性,而外侧或后部病变可能是尤因肉瘤。肋骨畸形的检查包括详细询问病史、全面的临床检查以寻找任何相关异常,并通过胸部X线平片和超声等基本影像学检查加以补充。最后,根据病变的解剖位置,采用计算机断层扫描或更优选的MRI进行补充检查,可如我们的病例报告所示确诊最终诊断,并消除任何不确定性。

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