儿童会阴型中缝囊肿7例

目的：探讨儿童会阴型中缝囊肿的临床特征和诊治经验。方法对2009年10月至2013年3月收治的7例会阴型中缝囊肿患儿的临床及随访资料进行回顾性分析。结果7例患儿均为男性,平均年龄6岁（3～13岁）,均表现为会阴中缝上条索状或管状囊肿,呈淡黄色半透明状,边界较清,触之质软,囊肿多呈进行性增大,无疼痛等不适,无炎症表现。7例均予手术完整切除病变。术后病理检查证实为（会阴型）中缝囊肿。平均随访33个月无复发。结论中缝囊肿为良性病变,会阴型较为少见。手术完整切除病变后预后良好。     

肝间叶性错构瘤临床及病理分析

<正>肝间叶性错构瘤(mesenchymal hamartoma of liver,MHL)为罕见的肝脏良性肿瘤,发病年龄常为2周岁以内,偶尔可发生于成人。由于此病罕见,容易误诊,现结合我院2004～2010年收治的6例病例并复习国内外相关文献报道,探讨其临床及病理特征、鉴别诊断及发病机制等。     

膀胱血管瘤的诊断及治疗

目的 探讨膀胱血管瘤的诊断及治疗。方法 对我院收治的3例膀胱血管瘤的诊治过程进行同顾性分析(1例行膀胱镜检、膀胱部分切除术,2例行膀胱镜活检、电灼疗法),并对国内外已报道的62例病例进行文献复习。结果 3例治疗效果良好,术后随访6个月～5年,未见复发。结论 膀胱血管瘤是一种少见的先天性脉管发育畸形,常在追查肉眼血尿和／或排尿异常症状时偶然发现此病。由于膀胱镜下所见是非特异的,故全面认识此病,提高警惕是防止误诊的关键。此病预后良好。     

小儿肠重复畸形的临床及影像学诊断

肠重复畸形又称肠源性囊肿或肠内囊肿,是少见的先天性消化道畸形,男女之比为1.21.0.此病常发生于小儿,病理类型不同,临床表现各异,术前诊断困难,误诊率高.现就小儿肠重复畸形的基础和临床表现及影像学诊断,特别是核医学现象的特点和相关的知识进行介绍.     

先天性颈部肺疝一例

先天性颈部肺疝(congenital cervical lung hernia)临床上十分罕见,目前国内外文献关于本病多为个案报道.本文就笔者诊断一病例,结合文献复习,对该病的临床及影像学表现加以讨论,以提高对本病诊断的准确性.     

先天性结核病

先天性结核病比较少见,且不易早期诊断,故预后不良.目前国外文献关于先天性结核病已有报道.1952年,美国文献中先后报道了24名先天性结核病.日本1908～1978年共报道12例,全部死亡.国内有关此方面的文献尚少,为了使我们进一步认识先天性结核病,以达到临床上早期诊断、早期治疗的目的,本文收集了近10余年来国内外有关文章,作一综述.     

小儿阴性阑尾切除现状的分析与探讨

多年以来,急性阑尾炎仍是小儿外科常见急腹症之一,约占小儿外科急腹症总数的25％。依靠典型的症状及体征,临床上诊断阑尾炎并不困难,但此病如延误诊治可导致阑尾穿孔、腹膜炎、败血症等严重并发症。为避免此类情况的发生,患儿一旦疑诊为急性阑尾炎,应尽快予以手术治疗。由于不同年龄患儿解剖生理特点及诊断的误差,国内外文献报道仍有2％～30％的阴性阑尾切除率［1-3］。阴性阑尾切除是指术前诊断为急性阑尾炎,经术中探查及术后病理检查诊断为非急性阑尾炎的病例。本文综述近年来国内外文献关于小儿阴性阑尾切除的资料,分析其误诊误切的原因、并发症及其他负面影响,旨在为临床提高诊断率、降低阴性阑尾切除率提供依据。     

小儿肠系膜乳糜囊肿3例报告

小儿肠系膜乳糜囊肿较罕见,诊断困难,现将我院收治并经手术及病理检查确诊的3例小儿肠系膜乳糜囊肿报道如下.     

先天性肠系膜裂孔疝的临床特征及治疗

先天性肠系膜裂孔疝国内外文献报道较少，此病发展迅速，常合并绞窄性肠梗阻及中毒性休克，是小儿外科严重的急腹症之一。自１９７３年以来，在手术治疗小儿各类机械性肠梗阻９８４例中，肠系膜裂孔迹引起急性肠梗阻７例，占０．７１９％，现报道如下。一、临床资料：本组...     

伴皮质下囊肿的巨脑性白质脑病

伴皮质下囊肿的巨脑性白质脑病(megalencephalic leukoencephalopathy with subcortical cysts,MLC)是一种常染色体隐性遗传疾病.1995年荷兰儿科医生vall der Knaap 等最先报道此病,故又称为van der Knaap病,2000年Topcu 等川将此病的相关基因定位于22q13.33,此后Leegwater等[2] 从4个候选基因中筛选出KIAA0027为该病基因,并于2001年将其重命名为MLC1基因.     

PHACES syndrome with congenital hypothyroidism

The acronym PHACE syndrome stands for Posterior fossa malformations, Hemangiomas, Arterial anomalies, Coarctation of the aorta and other cardiac defects, Eye abnormalities, Sternal clefting and/or a supraumbilical raphe. We report a 20 day old neonate who presented with a large facial hemangioma and Dandy-Walker cyst. This case is unusual because the cyst was detected antenatally and there was associated congenital hypothyroidism, which has been described very rarely with this condition.     

'Air-bubble' signs in the CT diagnosis of perforated pulmonary hydatid cyst: three case reports

Computed tomography is an important imaging modality in the evaluation of hydatid cyst. However, diagnosis of perforated hydatid cyst is very difficult and for this reason some radiological findings have been defined. An important finding is the 'air bubble' sign. As a consequence of cyst rupture, secondary bacterial infections like bronchopneumonia or lung abscess may develop which complicate the clinical picture further. In the literature, the 'air bubble' sign which is a relatively newly discovered radiological sign is reported to be very sensitive in establishing diagnosis. For this reason, in suspected cases of hydatid cyst, the air bubble sign may aid physicians in establishing diagnosis.     

Abdominal lymphangioma in childhood

The symptoms of abdominal lymphangioma are unspecific and depend on site and size of the tumor. The most frequent symptoms consist of pain up to the clinical picture of a pseudo-appendicitis, digestive problems, palpable or even exterior visible tumors such as the pseudo-ascites. Often the diagnosis cannot be found by clinical examination or further investigation. As a differential diagnosis all mesenterial cysts as well as other cystic tumors which originate in an abdominal organ must be taken in account. In most cases possible complications make surgical intervention necessary through which an exact diagnosis of the actually asymptomatic tumor can be found. Lymphangioma can lead to obstruction of the intestine, the ureter or the bile-duct. Inflammations of the cyst wall, bleeding into the cyst, torsion of the cyst pedicle and rupture of the cyst occur. The only curative therapy is the total surgical removal. Prognosis is good and relapse seldom occurs. We report on a 4.5-year-old boy with a cystic lymphangioma of the mesosigma. In addition, we discuss cases from the literature of the past 15 years.     

Unusual presentation of cervical spinal intramedullary arachnoid cyst in childhood: case report and review of the literature

The authors report a 7-year-old girl who presented with progressive quadriparesis which had started a month before admission. Magnetic resonance imaging of the spine revealed an intramedullary cystic lesion extending from C(2) to C(4). After performing a C(2-5) laminectomy, the cyst was drained and anatomically fenestrated with the subarachnoid space by a 1-cm vertical median myelotomy without using a stent. The histopathological findings revealed arachnoid cyst. In the first month after operation, the neurological deficits disappeared gradually, except for a slight left upper paresis. Cervical spinal intramedullary arachnoid cyst is extremely rare and should be considered in the differential diagnosis of intramedullary cystic lesions in childhood. Recovery is possible after subtotal excision and fenestration of the cyst to allow communication with the subarachnoid space even if neurological deficits are present for a long time.     

Hydatid cyst

A 3-year-old girl who presented with clinical features suggesting a choledochal cyst was found to have a retroperitoneal hydatid cyst. The correct diagnosis was made at operation. Details regarding the history, results of investigations, and operative findings are presented.     

Craniocervical neurenteric cyst without associated abnormalities

Spinal neurenteric (NE) cyst is an uncommon congenital cyst and frequently found in the cervical region. The clinical symptoms associated with this entity depend on the site of the lesion and are not typical for all such cysts. A definitive diagnosis can only be made by biopsy and histological examination. MRI can confirm these cystic masses and is the method of choice for their imaging investigation. They are often connected by a fibrous tract, fistula or cleft to structures derived from the primitive gut in the thoracic or abdominal cavities and are commonly associated with anterior spina bifida or other vertebral anomalies. We report a case of craniocervical NE cyst without associated abnormalities and discuss the implications for clinical diagnosis and management by a thorough review of the literature.     

Giant Meckel's diverticulum presenting as tumor

The authors report a case of giant Meckel's diverticulum, presenting as a subumbilical abdominal mass, associated with an infectious picture and anemia in a 16 month-old boy. Epidemiologic, pathologic and clinical features of this rare disorder are reviewed as are the diagnostic means. The differential diagnosis with other cystic masses of the umbilical area (omphalo-mesenteric duct cyst, urachal cyst, mesenteric cyst, duplication) is discussed.     

Huge arachnoid cyst of the posterior fossa with cerebellar tentorium dysplasia associated with juvenile polyposis

We report an infant with a huge arachnoid cyst of the posterior fossa with dysplasia of the cerebellar tentorium and meningeal sinus and associated juvenile polyposis. Neuroimaging studies disclosed a huge median cystic lesion extending posterosuperiorly over the cerebellum. The cerebellar tentorium was raised to the parietal area; the vermis was normoplastic. Cystography showed no direct communication with the 4th ventricle or subarachnoid space. We discuss the differential diagnosis of median cysts of the posterior fossa and the association of juvenile polyposis.