Colchicine as rescue treatment in two pediatric patients with chronic recurrent multifocal osteomyelitis (CRMO).

Chronic recurrent multifocal osteomyelitis (CRMO) is a rare idiopathic autoinflammatory bone disease characterised by noninfective inflammation of bones. Diagnostic approach is challenging and requires exclusion of other causes such as malignancies or infections. Nonsteroidal anti-inflammatory drugs (NSAIDs) and corticosteroids are usually applied as first-line therapy in CRMO patients; however, some cases require more intensive therapy with second-line agents to control disease activity. We hereby describe the use of colchicine as a nonconventional second-line disease-modifying antirheumatic drug in two pediatric patients with CRMO refractory to NSAIDs and corticosteroids. Our data indicate that colchicine might prove an important area for future research as a potential therapeutic option with easy administration, low cost, and a good safety profile in CRMO patients refractory to first-line therapy.

Gynecological anomalies in patients with anorectal malformations.

PURPOSE: The association of gynecological anomalies in all anorectal malformations (ARM) is firmly established. Our goal is to study this pathology in our patients to focus attention to this important issue. METHODS: Retrospective study of female patients operated for ARM and who underwent magnetic resonance imaging in our center. The type of malformation, the presence and type of vaginal, uterine, tubaric and urological anomalies were studied. RESULTS: 63 patients were included: 34.9% cloaca, 28.6% vestibular and 12.7% perineal. Half of patients had some type of müllerian anomaly; 19 vaginal, most frequent being the longitudinal vaginal septum (66.7%); 30 had uterine alterations, most frequent being the uterus didelphys (60%). Eighty percent of patients with complex ARM (cloaca, exstrophy) presented some type of gynecological malformation compared to 21.8% found in simple ARM (stenosis, perineal, vestibular) (p < 0.001). Vaginal anomalies are associated with a uterine anomaly in 100% of cases. Conversely, patients with uterine anomalies have concurrent vaginal anomaly in 63.3% of cases. CONCLUSION: Screening for gynecological anomalies is indicated in all patients with ARM. We recommend a vaginal examination in any girl with ARM during definitive repair and a subsequent MRI during follow-up. Collaboration with a gynecologist is essential.

Neonatal magnetic resonance imaging in double aortic arch diagnosed prenatally by ultrasound.

Congenital double aortic arch (DAA) is an uncommon vascular anomaly; however, its prenatal detection is associated with congenital heart defects and chromosomal abnormalities, including 22q11 deletion. We present a case of DAA diagnosed prenatally. DAA can be diagnosed by prenatal ultrasound in the transverse three vessel-trachea view, which shows a trident image formed by a complete vascular ring and the ductus arteriosus. Postnatal magnetic resonance images in this view correlate well with prenatal ultrasound images and help in confirmation of diagnosis, evaluation of the risk of airway or esophageal compression, and planning of surgery.

Endourological management in a newborn with incomplete bladder duplication.

We report an unusual case of a newborn with incomplete bladder duplication and a complete sagittal septum. This malformation was associated with dysplasia of the right kidney, right cryptorchidism, single urethra, and no other genital or gastrointestinal anomalies. At birth, we found severe ureterohydronephrosis in a solitary left kidney caused by the collapse of the left bladder when the right bladder was filled. We performed a neonatal puncture of the bladder septum. At 3 months of life, the patient underwent complete resection of the septum by cystoscopy with monopolar electrocautery. There are no previously reported cases of a newborn with obstructed uropathy. This is the first reported case of incomplete bladder duplication with endourological management.