Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche. Endometriosis is a prevalent complication. Magnetic resonance imaging (MRI) helps in diagnosing OHVIRA syndrome and endometriosis due to its high contrast resolution and objectivity. We reported a 13- year-old girl who was evaluated for cyclic pelvic pain after her menarche at 12 years of age. Magnetic resonance imaging (MRI) revealed two separate uterine cavities, services and vaginae, indicating didelphys. The left uterine cavity is filled with fluid, and the left hemivagina is dilated and filled with hyperintense and hypointense fluid on T1 and T2, respectively, indicating blood products. Left hemivagina dilatation implicated the presence of an obstructing vaginal septum. A single left adnexal cyst lesion with blood products was suggestive of an endometriotic cyst. Additionally, the left kidney was absent. A uterine didelphys with left hemivagina obstruction, hematometra, hematocolpos, and the ipsilateral ovarian endometriotic cyst was diagnosed. A final diagnosis of OHVIRA syndrome or HWWS was made, considering that she had no left kidney.MRI is a suitable diagnostic tool for precise anatomical delineation of the uterus, cervix, and vagina in uterovaginal disorders such as OHVIRA syndrome. MRI can also properly evaluate endometriosis and adhesion.
Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive paediatric brain tumour and nowadays has not had satisfactory result, with most patients do not survive within 1 year of diagnosis. Due to its proximity to critical organs, surgery is avoided, and radiation is the mainstay of treatment. In this case report, we present a case of DIPG treated with radiation and concurrent temozolomide. A 7- year-old child was admitted with complaints of weakness in the eyelid, upper and lower limbs 2 months ago. Physical examination showed tetra paresis and bilateral cranial nerve palsy. Magnetic resonance imaging (MRI) scan showed intracranial tumour consistent with DIPG. Diagnosis was made based on imaging as surgery or biopsy can lead to further morbidity. The patient underwent radiotherapy with concurrent chemotherapy of temozolomide. Radiation was given by dose of 54 Gy/30 fractions (30 × 1.8 Gy) with volumetric arc therapy (VMAT). Due to technical issue after the first five irradiations resulting in 2 weeks delay, boosting of dose by 5 × 1.8 Gy was then given, hence, the total dose was 63 Gy. The booster only targeted the gross tumour volume. Following radiation, the patient felt clinical improvement. Eyelid and limb movement improved since the 15th fraction. At the last fraction, the patient's condition improved symptomatically, but experienced complaints related to post radiation oedema including dizziness and nausea. These complaints were improved upon steroids administration. The MRI evaluation will be done after 8 to 12 weeks of radiation, considering the effects of acute radiation could still occur at this period. In conclusion, a combination of radiotherapy and temozolomide could be an option for DIPG management, with tolerable acute toxicity and possible clinical improvements.
Juvenile nasopharyngeal angiofibroma (JNA) is a rare paediatric tumour known for its local destructiveness and high recurrence rate. Surgery is the primary treatment modality for JNA, though other options, such as hormonal therapy, embolisation and radiotherapy, exist for inoperable cases. The location of the tumour makes surgical intervention challenging. A 14-year-old male presented with epistaxis and headaches as the chief complaints and was diagnosed with nasopharynx angiofibroma by computed tomography (CT) scan in 2018. Pre-operative embolisation was performed and followed by surgical removal of a 4 cm tumour in January 2019. Pathological examination revealed CD34 positivity, S100 negativity and Ki-67 positivity (5 to 10%), confirming angiofibroma. In October 2019, a 3.6 cm recurrent tumour was treated with embolisation and a second surgery. Pathological findings again confirmed JNA. The patient underwent four surgeries in total, but epistaxis persisted. In 2021, local radiotherapy was administered using intensity-modulated radiation therapy (IMRT) at a dose of 60 Gy in 25 fractions. Serial magnetic resonance imaging (MRI) post-radiotherapy showed a decreasing tumour size, with no further epistaxis and no observed radiation side effects 2 years post-treatment. Radiation therapy remains a strong alternative for managing recurrent JNA.