El Yamine Othmane, Fatine Amine, Boufettal Rachid, Errguibi Driss, Hajri Amal, Rifki El Jay Saad, Chehab Farid
Surgical Department of Cancerology and Liver Transplantation Universitary Hospital Center Casablanca morocco, Morocco.
Ann Med Surg (Lond). 2021 Apr 28;66:102362. doi: 10.1016/j.amsu.2021.102362. eCollection 2021 Jun.
and importance: Retrorectal cystic hamartoma (RCH) is a rare congenital lesion of the presacral space, which is part of the vestigial cystic tumors often benign and predominantly in women. Generally asymptomatic, the appearance of symptoms such as pain or neurological disorders should raise suspicion of degeneration.
We report an unusual observation of a 62-year-old patient admitted for perineal pain evolving for 2 months associated with tenesma and chronic constipation. The digital rectal examination found a posterior bulge at 4 cm from the anal margin, without intraluminal lesion. Rectosigmoidoscopy had noted posterior extrinsic compression but no rectal tumor. Pelvic CT and MRI had shown a solidocystic formation of the retro-rectal and presacral spaces, related to an enteric cyst. The operation was performed by abdominal approach and the surgical exploration had found a bilobed cystic formation. The cystic mass was removed and the anatomopathological examination concluded that it was a cystic hamartoma with no sign of malignancy.
Retrorectal tumors develop in the space bounded anteriorly by the propria fascia of the rectum and posteriorly by the presacral fascia overlying the sacrum. Common in children and then often malignant, inversely, in adults, they are rare and most often benign tumors. They are generally asymptomatic with a predominance of females, unlike our observation where the patient was male with a symptomatology dominated by perineal pain and constipation. The discovery is incidental in the majority of cases, however, in some cases, these cysts may be revealed by complications. The lesion can be explored by transrectal or suprapubic ultrasound, MRI and CT scan. Rectoscopy and fistulography may complete the exploration in case of diagnostic doubt. The resection must be thorough and in monobloc because of the risk of recurrence and the approach depends on the location and the size of the lesion.
RCH is a rare benign lesion whose morphological characteristics seem quite stereotyped. A detailed postoperative anatomopathological examination allows the diagnosis to be made and, above all, to look for a site of malignant transformation. This is why a complete surgical removal is necessary to prevent recurrence.
及重要性:直肠后囊性错构瘤(RCH)是骶前间隙的一种罕见先天性病变,属于常为良性的残留囊性肿瘤,主要发生于女性。通常无症状,出现疼痛或神经功能障碍等症状应怀疑发生了退变。
我们报告了一例不寻常的病例,一名62岁患者因会阴部疼痛入院,疼痛持续2个月,并伴有里急后重和慢性便秘。直肠指检发现距肛缘4厘米处有一个后凸,腔内无病变。直肠乙状结肠镜检查发现有外在性后压,但无直肠肿瘤。盆腔CT和MRI显示直肠后和骶前间隙有一个实性囊性结构,与肠囊肿有关。手术采用腹部入路,手术探查发现一个分叶状囊性结构。切除了囊性肿物,解剖病理学检查结论为这是一个无恶性迹象的囊性错构瘤。
直肠后肿瘤发生于前方由直肠固有筋膜、后方由覆盖骶骨的骶前筋膜界定的间隙内。在儿童中常见,且常为恶性,相反,在成人中则罕见,且大多为良性肿瘤。它们通常无症状,女性居多,与我们的病例不同,该患者为男性,症状以会阴部疼痛和便秘为主。大多数情况下发现是偶然的,然而,在某些情况下,这些囊肿可能因并发症而被发现。可通过经直肠或耻骨上超声、MRI和CT扫描对病变进行检查。在诊断存疑的情况下,直肠镜检查和瘘管造影可完善检查。由于存在复发风险,切除必须彻底且整块切除,手术入路取决于病变的位置和大小。
RCH是一种罕见的良性病变,其形态特征似乎相当固定。详细的术后解剖病理学检查有助于做出诊断,最重要的是,可查找恶性转化部位。这就是为什么需要进行完整的手术切除以防止复发。