宫、腹腔镜联合诊治性腺发育不全６例报告

【摘要】  　　目的：探讨宫、腹腔镜联合诊治性腺发育不全的作用及性腺发育不全的镜下特点。方法：对６例性腺发育不全患者进行宫、腹腔镜检查及腹腔镜下切除的性腺病例资料进行分析。结果：６例均为女性外阴，含有Y染色体，性激素低，５例骨龄明显低于同龄，３例为xo/xy性腺发育不全，３例为xy单纯性性腺发育不全，性腺均位于腹腔内，５例宫腔镜下见有宫颈，２例合并腹股沟斜疝，１例因Ｂ超未见子宫外院诊为ＣＡＩＳ，１例xo/xy性腺发育不全者性腺合并性腺母细胞瘤（１６.６７％）。结论：宫、腹腔镜是鉴别性腺发育不全抑或性激素及功能异常较好的诊断手段，且腹腔镜下观察清晰，在镜下切除性腺组织简单易行，可防止性腺发生病变。

【关键词】  性腺发育不全；宫腔镜检查；腹腔镜术；性激素类；病例报告

　　Clinical analysis of gonadal dysgenesis and treatment with hysteroscopy combined with laparoscopy:with a report of 6 cases

    【Abstract】Ｏbjective:To explore the role of hysteroscopy combined with laparoscopy in diagnosis and treatment for gonadal dysgenesis,and analyzed the clinical feature of the disease observed during the operation.Methods:Six cases of gonadal dysgenesis underwent hysteroscopy combined with laparoscopy were collected and analyzed.Results:All of the 6 cases' vulva appeared female,contained Y chromosome,had lower sex homone level,and 5 cases bone ages were lower than the normal people of the same age,3 cases were xo/xy gonadal dysgenesis,the 3 cases were xy pure gonadal dysgenesis.All the gonads sited in abdomen.5 cases were found cervix through hysteroscopy.2 cases werer found indirect inguinal hernia,1 case was diagnosed CAIS in other hospital because the didn't find cervix through B ultrasound,1 case of xo/xy gonadal dysgenesis had gynand rab lastoma.Conclusions:Hysteroscopy combined with laparoscopy is an effective diagnostic tool for gonadal dysgenesis,and dysfunction of sex homones,and also can well observed the feature of gonadal dysgenesis.Gonadectomy can be performed easily by laparoscopy for the prevention of neoplasia.

    【Key words】Gonadal dysgenesis;Hysteroscopy;Laparoscopy;Sex hormones;Case report

    性腺发育不全所致疾病临床上较罕见，以往临床上区别性腺发育不全抑或性激素及功能异常（如ＣＡＩＳ）多靠临床表现、性激素及Ｂ超检查有无子宫等，发育不良时子宫易造成漏诊，且性激素测定各地及个体差异较大，不易分辨，１年来我们对６例性腺发育不全患者行宫、腹腔镜检查并在腹腔镜下切除了性腺，现报道如下。

　　1  资料与方法

    ２００６年１月至２００７年５月在我院经宫、腹腔镜诊治性腺发育不全患者６例，13～19岁，平均15.33岁。均含Ｙ染色体，其中３例为xy单纯发育不全，３例为xo/xy性腺发育不全（即混合性性腺发育不全，染色体为４５，xo/46,xy)。２例xo/xy性腺发育不全者合并有腹股沟斜疝，１例xo/xy性腺发育不全合并双侧性腺母细胞瘤。6例外阴均为幼稚型，4例B超示始基子宫，2例未探及子宫。

　　２  结  果

    ６例宫腔镜检查示５例有宫颈，１例宫腔镜未见宫颈者腹腔镜下亦未见始基子宫，染色体为４５，xo/46,xy。６例均经腹腔镜顺利切除了双侧性腺组织，２例行腹股沟斜疝修补术。腹腔镜下见发育不全的性腺有２种形态：（１）有始基子宫者，其始基子宫位于中央，子宫大者１.５cm×1.0cm×0.8cm，小者为一纤肌性结节，细的输卵管伞端结构不典型，下方灰白色，肾形不典型卵巢样组织；（２）无始基子宫者，双侧有较典型的伞端，伞端之间凸出于腹膜条索样组织，未见明显卵巢组织。６例出血量２～１０ml。合并性腺母细胞瘤１例有宫颈及宫体，宫体１.５cm×1.0cm×0.8cm大，家属强烈要求保留始基子宫（染色体为４５，xo/46,xy）。均行快速冰冻切片检查。