RESUMO
BACKGROUND: Bronchogenic cysts (BCs) refer to congenital lesions that result from primitive or abnormal foregut budding, and can be pulmonary or mediastinal. Their occurrence can take place at any point on the tracheobronchial tree, but they are usually localized in the lung parenchyma and mediastinum, and may be symptomatic or asymptomatic. Bronchogenic cyst symptoms can vary, depending on the size and location of the cyst. METHODS: A retrospective review of the charts of five patients with a histopathological diagnosis of bronchogenic cysts was performed between 2014 and 2020. The patients reported in this study were diagnosed and managed at Abha Maternity and Children Hospital, Abha, southwest Saudi Arabia. In addition, demographic information, as well as diagnostic and therapeutic information, was provided for each patient, both at discharge and after discharge. All patients had confirmed congenital bronchogenic cysts with different clinical phenotypes and radiological findings. RESULTS: All patients had histopathologically confirmed bronchogenic cysts with different clinical and radiological presentations. Two patients had mediastinal-located cysts; one had a laryngeal cyst; and the last two patients had infected intrapulmonary bronchogenic cysts. All patients underwent complete excision and did not experience recurrence or other postoperative complications during the follow-up period. The latter two patients required lobectomies of the right middle and upper lobes. CONCLUSIONS: Although bronchogenic cysts are considered a rare congenital pulmonary malformation, they should be considered in the differential diagnosis of pediatric patients with unusual airway and parenchymal lung manifestations, particularly, persistent stridor, feeding difficulty, and complicated pneumonia. Surgical excision of the cyst is the gold-standard therapy for symptomatic bronchogenic cysts and is highly recommended for asymptomatic ones. Long-term follow-up studies will be required to explore any long-term complications of BCs, particularly regarding the malignancy transformation.
RESUMO
OBJECTIVE: To report the finding of transmural rectal involvement, in four children with lesions which seemed to be neoplastic in appearance and progression. METHODS: The case records of four children presenting with rectal involvement by the fungus Basidiobolus ranarum, were retrospectively analyzed for clinical presentation, hematological and radiological investigations, the procurement of histopathological material for diagnosis and the findings thereof, the treatment of these patients and the follow-up. RESULTS: The four children presented with non-specific symptoms of fever, loss of weight and appetite, constipation and bleeding per rectum. One presented with excoriation and ulceration of the perineum and perianal skin. Examination generally unremarkable, however, showed the rectum extensively involved by a lesion which narrowed the lumen. Hematological investigations showed leukocytosis with eosinophilia and raised levels of acute phase reactants like ESR, platelets, and C-reactive proteins. Ultrasonogram (USG) and CT scan confirmed the lesion to be involving all layers of the rectum and compromising the rectal lumen. The right colon was also involved in one patient. One underwent a colonoscopy and biopsy which proved inconclusive. All four underwent a Tru-cut biopsy which was diagnostic. Histopathologically the disease was based in Basidiobolus ranarum, a fungus which is emerging as a cause of visceral abdominal involvement. CONCLUSIONS: Lesions involving the rectum, and appearing to be neoplastic may be caused by the fungus Basidiobolus ranarum. The symptomatology and presentation may be non-specific. High levels of eosinophils in the blood, a raised ESR, and C-reactive protein may be useful pointers to the diagnosis. USG and CT scans localize the lesions and also provide a guide for biopsies. The characteristic histopathological findings are diagnostic and based on these, treatment with Itroconazole / Voriconazole is beneficial.