Alhassan Basmah Faris, Alharbi Abdullah Saji, Omar Walid Mokhtar, Zayed Mohammed Ayesh, Abdulla Maha, Bin Traiki Thamer Abdulla
Department of General Surgery, King Khalid University Hospital and College of Medicine, King Saud University, Saudi Arabia.
Department of Anesthesia, King Khalid University Hospital and College of Medicine, King Saud University, Saudi Arabia.
Int J Surg Case Rep. 2019;60:224-229. doi: 10.1016/j.ijscr.2019.05.020. Epub 2019 May 11.
Pseudomyxoma peritonei (PMP) is a feared complication of appendicular mucocele perforation. Although a rare disease, its major sequel mandates recognition and early intervention. Intestinal malrotation is mostly asymptomatic in adults. Its significance arises when it complicates another coinciding condition by confusing the presentation, leading to delay in diagnosis and treatment. PMP and incidental finding of gut malrotation in adults are two rare events, and the chance of both occurring in the same patient is very slim. This can complicate the clinical picture and lead to devastating outcomes.
We present a case of PMP in a patient with gut malrotation, managed with cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC).
Management with CRS/HIPEC has been found to improve outcomes for patients with PMP. However, the extensive disease and abnormal anatomy of the patient in our report proposed unique intraoperative challenges. Preserving part of the colon was possible with an improvised surgical technique that we used which proved to be safe and effective.
Early recognition and consideration of uncommon but serious surgical conditions are essential for improved patient outcomes. To our knowledge, this is the first report in the English literature that describes the use of CRS/HIPEC for PMP in a case of intestinal malrotation. This improvised surgical technique was found to be safe and can provide a surgical solution for preserving part of the colon in selected patients.
腹膜假黏液瘤（PMP）是阑尾黏液囊肿穿孔令人担忧的并发症。尽管是一种罕见疾病，但其主要后遗症需要得到认识和早期干预。成人肠旋转不良大多无症状。当它与另一种同时存在的疾病并发时，其重要性就显现出来，会使临床表现变得复杂，导致诊断和治疗延迟。PMP和成人偶然发现肠旋转不良是两个罕见事件，两者在同一患者中发生的可能性非常小。这会使临床情况复杂化并导致灾难性后果。
我们报告一例患有肠旋转不良的PMP患者，采用细胞减灭术（CRS）和热腹腔内化疗（HIPEC）进行治疗。
已发现采用CRS/HIPEC治疗可改善PMP患者的预后。然而，我们报告中的患者病情广泛且解剖结构异常，带来了独特的术中挑战。我们采用的一种临时手术技术能够保留部分结肠，事实证明该技术安全有效。
早期识别并考虑罕见但严重的外科疾病对于改善患者预后至关重要。据我们所知，这是英文文献中首例描述在肠旋转不良病例中采用CRS/HIPEC治疗PMP的报告。这种临时手术技术被发现是安全的，可为特定患者保留部分结肠提供手术解决方案。
