Tada Yoichiro, Yamamoto Manabu, Sunaguchi Teppei, Uejima Chihiro, Tanio Akimitsu, Murakami Yuki, Takano Shuichi, Sakamoto Teruhisa, Honjo Soichiro, Ashida Keigo, Saito Hiroaki, Fujiwara Yoshiyuki
Division of Surgical Oncology, Department of Surgery, School of Medicine, Tottori University Faculty of Medicine, 36-1 Nishi-cho, Yonago, 683-8504, Japan.
Surg Case Rep. 2019 May 31;5(1):89. doi: 10.1186/s40792-019-0645-2.
Hunter syndrome is an X-linked disorder caused by a deficit of the lysosomal enzyme iduronate-2-sulfatase and is associated with many disorders. Patients with Hunter syndrome often develop inguinal hernias in early childhood and undergo Potts' method, laparoscopic percutaneous extraperitoneal closure (LPEC), or laparoscopic direct suture.
An 18-year-old male visited our hospital for evaluation of a palpable mass in the right groin hernia. Computed tomography revealed a right indirect inguinal hernia. He had a history of repeated admission to our hospital and pediatric treatments for pneumonia, heart failure, and convulsions after birth. Because he has stopped growing and a wide hernia orifice was present with no apparent hernia on the left side, we performed TAPP repair. During surgery, we noted softness of the abdominal wall, similar to children's abdominal wall, and laparoscopy revealed well-developed veins around the spermatic cord and testicular artery. The softness of the abdominal wall made insertion of the trocars difficult and well-developed veins needed our special care to avoid hemorrhage. After surgery, the patient developed a convulsion due to Hunter syndrome and subsequent aspiration pneumonia; however, he recovered with medical treatments administered in cooperation with specialists and was discharged on postoperative day 9.
This is the first reported patient with Hunter syndrome whose inguinal hernia was treated by TAPP repair. TAPP repair might be a useful procedure even for adolescent patients with Hunter syndrome, although adequate care is needed for symptoms due to Hunter syndrome.
亨特综合征是一种X连锁疾病，由溶酶体酶艾杜糖醛酸-2-硫酸酯酶缺乏引起，与多种疾病相关。亨特综合征患者常在幼儿期发生腹股沟疝，并接受波特氏法、腹腔镜经皮腹膜外修补术（LPEC）或腹腔镜直接缝合术。
一名18岁男性因右侧腹股沟疝可触及肿块前来我院就诊。计算机断层扫描显示为右侧腹股沟斜疝。他有出生后因肺炎、心力衰竭和惊厥多次入住我院及接受儿科治疗的病史。由于他已停止生长，且疝孔较宽，左侧无明显疝，我们进行了经腹腹膜前修补术（TAPP）。手术过程中，我们注意到腹壁柔软，类似于儿童腹壁，腹腔镜检查显示精索和睾丸动脉周围静脉发育良好。腹壁柔软使套管针插入困难，且静脉发育良好需要我们特别小心以避免出血。术后，患者因亨特综合征发生惊厥，随后并发吸入性肺炎；然而，经与专科医生合作进行药物治疗后康复，并于术后第9天出院。
这是首例报道的采用TAPP修补术治疗腹股沟疝的亨特综合征患者。TAPP修补术可能对患有亨特综合征的青少年患者也是一种有用的手术方法，尽管对于亨特综合征引起的症状需要给予充分的护理。
