ﮔﺰارش ﯾﮏ ﻣﻮرد ﻧﻮزاد ﺳﻪ روزه ﺑﺎ ﺳﻨﺪرم ﻫﺮﻟﯿﻦ ورﻧﺮ وﻧﺪرﻟﯿﺶ )OHVIRA( در ﻣﺮﮐﺰ آﻣﻮزﺷﯽ درﻣﺎﻧﯽ ﻃﺎﻟﻘﺎﻧﯽ ﮔﺮﮔﺎن 1393

Case Report of Herlyn-Werner-Wanderlich Syndrome in Three Days Newborn Infant of Taleghani Educational Hospital (Gorgan) 2014

ﻧﺎﻫﻨﺠﺎريﻫﺎي رﺣﻤﯽ در ﺑﺮﮔﯿﺮﻧﺪه ﺑﺴﯿﺎري از ﺑﯿﻤﺎريﻫﺎ اﺳﺖ. از ﺟﻤﻠﻪ آﻧﺎن، ﻧﺎﻫﻨﺠﺎريﻫﺎي ﻣﺮﺑﻮط ﺑﻪ ﻧﺎﺣﯿﻪ ژﻧﯿﺘﺎل ﻣﯽﺑﺎﺷﺪ ﮐﻪ ﺑﺴﯿﺎر ﭘﯿﭽﯿﺪه و ﻧﺎدر ﻫﺴﺘﻨﺪ. اﻏﻠـﺐ اﯾﻦ ﻧﻮع اﺧﺘﻼﻻت ﺑﻪ ﺷﮑﻞ ﻧﺎدرﺳﺖ ﺗﺸﺨﯿﺺ داده ﻣﯽﺷﻮﻧﺪ و درﻣﺎن ﻧﺎﻣﻨﺎﺳﺐ را درﯾﺎﻓﺖ ﻣﯽﻧﻤﺎﯾﻨﺪ. اﯾﻦ ﮔﺰارش ﻣﻮرد ﻣﺮﺑﻮط ﺑﻪ ﻧﻮزاد ﺳﻪ روزه ﺑﺎ ﺗﺸﺨﯿﺺ ﺗﻮده ﺷﮑﻤﯽ ﺑﻮده ﮐﻪ، در ﻣﻌﺎﯾﻨﻪ اوﻟﯿﻪ ﺗﻤﺎﻣﯽ ﻋﻼﺋﻢ ﺣﯿﺎﺗﯽ ﻃﺒﯿﻌﯽ و ﻧﺎﺣﯿﻪ ژﻧﯿﺘﺎل ﻇﺎﻫﺮي ﻃﺒﯿﻌﯽ داﺷﺖ. در ﻟﻤﺲ ﺷﮑﻢ ﺗﻮده ﺑﺰرگ ﺑﺎ ﻗﻮام ﺳﻔﺖ و ﻏﯿﺮ ﻣﺘﺤﺮك در ﻧﺎﺣﯿﻪ ﻫﯿﭙﻮﮔﺎﺳﺘﺮ ﺗﺎ ﻧﺎف ﻗﺎﺑﻞ ﻟﻤﺲ ﺑﻮد. آزﻣﺎﯾﺸﺎت ﺗﺸﺨﯿﺼﯽ ﺳﯿﺴﺘﻮ ﯾﻮرﺗﺮوﮔﺮام ﺣﯿﻦ ادرار و ﺳﯽﺗﯽ اﺳﮑﻦ ﺑﺮاي ﺑﯿﻤﺎر اﻧﺠﺎم ﺷﺪ. ﺑﯿﻤﺎر ﺑﺎ ﺗﺸﺨﯿﺺ واژن ﻣﺘﺴﻊ ﺑﺎ رﺣﻢ دوﮔﺎﻧﻪ ﺗﺤﺖ درﻣﺎن ﺟﺮاﺣﯽ ﻗﺮار ﮔﺮﻓﺖ. درﻧﺎژ ﺗﺮﺷﺤﺎت اﻧﺠﺎم و ﯾﮏ ﻫﻔﺘﻪ ﺑﻌﺪ ﺑﯿﻤﺎر ﺑﺎ وﺿﻌﯿﺖ ﻣﻨﺎﺳﺐ ﻣﺮﺧﺺ ﮔﺮدﯾﺪ. ﺗﺸﺨﯿﺺ ﻧﻬﺎﯾﯽ ﺑﯿﻤﺎر ﺳﻨﺪرم ﻫﺮﻟﯿﻦ ورﻧﺮ وﻧﺪرﻟﯿﺶ ﻣﯽﺑﺎﺷﺪ. اﻏﻠﺐ ﻣﻄﺎﻟﻌﺎت زﻣﺎن ﺑﺮوز ﻋﻼﯾﻢ را ﺣﺪود 12-14 ﺳﺎﻟﮕﯽ ذﮐﺮ ﮐﺮدهاﻧﺪ، وﻟﯽ در اﯾﻦ ﻣﻮرد ﺑﺨﺼﻮص ﺗﺸﺨﯿﺺ و درﻣﺎن زودرس اﺗﻔﺎق اﻓﺘﺎد. ﺑﺮاي اﯾﻨﮑﻪ ﺗﺸﺨﯿﺺ زودرس اﺗﻔﺎق ﺑﯿﻔﺘﺪ، ﻫﻤﮑﺎري ﻫﻤﻪ ﺟﺎﻧﺒﻪ ﭘﺰﺷﮑﺎن ﻣﺘﺨﺼﺺ اﻃﻔﺎل، زﻧﺎن، رادﯾﻮﻟﻮژي و ﺟﺮاح اﻃﻔﺎل ﺿﺮوري ﺑﻪ ﻧﻈﺮ ﻣﯽرﺳﺪ.

Uterine abnormalities including many disease and disorders of the genital area are very complex and rare. These diseases often incorrectly are diagnosed and receive inappropriate treatment. This case report related to three-days neonatal abdominal mass was detected in the initial examination, vital signs and natural appearance to the genital area was normal. Also in touch an abdominal mass was palpable in the hypogastria. VCUG and CTscan were performed and patient with vaginal hydrometrocolpus were taken to operating room and underwent treatment. The catheter was inserted in the vagina to drain but seen in other uterine so drainage was performed then a week later the patient discharged with a good condition. Many studies have noted the signs for about12-14 years, but in this case happened early diagnosis. For early detection cooperation pediatricians, obstetricians, radiologists and pediatric surgeon seem necessary.