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Congenital cystic adenomatoid malformation - dangers of misdiagnosis: a case report.

ABSTRACT: Congenital cystic adenomatoid malformation is a rare pulmonary malformation, but is the most common lung malformation observed in children. In developing countries, such as Morocco, prenatal diagnosis is missing. Congenital cystic adenomatoid malformation may occur after birth in the presence of complications and needs a computed tomography scan for confirmation. However, our lack of awareness of this malformation has been responsible for a late and wrong diagnosis along with therapeutic errors. We report the first case in Morocco where diagnosis is confirmed by histology after death.A 10-month-old Arab boy was prescribed various antibiotics (including anti-staphylococcal) and endured repeated chest drainages, leading to his death just after radiological diagnosis and instant surgery.The goal of this case report is to firmly express the need for both pediatricians and radiologists to enlarge diagnosis investigations, especially of congenital or constitutional entities in children, as soon as recurrence of respiratory distress and pulmonary infections are manifested. We also emphasize this important entity because of its frequency, to avoid the eventual therapeutic errors.

SUBMITTER: El Amraoui W

PROVIDER: S-EPMC5543741 | biostudies-other | 2017 Aug

REPOSITORIES: biostudies-other

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Project description:Introductionand 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.Case presentationWe 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.Clinical discussionRetrorectal 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.ConclusionRCH 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.

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Congenital cystic adenomatoid malformation - dangers of misdiagnosis: a case report.

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