Torsion of an Accessory Liver Lobe in a Newborn.

Accessory liver lobes are rare. We present the rare case of torsion of an accessory liver lobe in a neonate. A 13-day-old newborn presented with failure to thrive and hematemesis without fever. The initial workup with sonography, magnetic resonance imaging, and upper gastrointestinal study was suspicious of a duplication cyst, most likely in the posterior wall of the stomach. Laboratory and radiological findings were not suggesting a choledochal cyst. We performed a laparotomy with resection of the 3.2 × 2.1 × 1.1 cm mass. Intraoperatively, the cystic formation extended from of the liver bed up to the lesser curvature of the stomach. The mass was attached to the left liver lobe with fibrous bands. Histopathology revealed necrotic liver parenchyma with patent viable biliary ducts, indicative of an accessory liver lobe that underwent torsion in the perinatal period. The postoperative course and follow-up (6 months so far) were uneventful. To our knowledge, this is the youngest described patient in the literature with an accessory liver lobe torsion and the second case report concerning this entity in a neonate. It presents an extremely rare differential diagnosis in symptomatic neonates with a cystic mass in the upper abdomen.

Unique Presentation of Hematometrocolpos Mimicking Cauda Equina Syndrome: Severe Back Pain and Urinary Incontinence in an Adolescent Girl.

BACKGROUND: Imperforate hymen with hematometrocolpos in adolescent females is a rare pediatric condition. Classical presentation includes abdominal pain or a pelvic mass in female patients with primary amenorrhea. Atypical complaints and reluctance among emergency physicians to perform genital examination in the emergency department or the pediatric emergency department (PED) may delay correct diagnosis. CASE REPORT: We report a unique, cauda equina syndrome-like presentation of hematometrocolpos secondary to imperforate hymen in a 13-year old, previously healthy girl with primary amenorrhea. In the PED, the unusual clinical presentation of severe back pain and urinary incontinence initially mimicked cauda equina syndrome and led to delayed correct diagnosis. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: The novelty of this case is a cauda equina-like presentation of imperforate hymen secondary to hematocolpos. This report illustrates the highly variable clinical presentation of this rare gynecological pediatric entity. It underlines the importance of considering this rare condition in the differential diagnosis of severe upper or lower back pain alongside voiding abnormalities including urinary retention and incontinence in adolescent females with primary amenorrhea. Above all, the importance of performing a thorough history and genital examination in this subgroup early in the investigation process in the PED emerges from this case. Essentially, excellent clinical judgment and genital examination by the emergency physician may minimize unnecessary radiological investigations and ultimately, accelerate correct diagnosis and expedite appropriate surgical treatment. However, not only pediatric and adult emergency physicians, but also pediatricians and general practitioners should be aware of this entity and its diverse clinical presentation.

Pre- and postnatal imaging of Pai syndrome with spontaneous intrauterine closure of a frontal cephalocele.

Pai syndrome is a rare congenital disorder characterized by cutaneous polyps of the face, pericallosal lipoma and median cleft lip. We report on a newborn girl with a variant of Pai syndrome presenting with all typical findings except a median cleft. In addition, fetal sonography and MRI showed the unique intrauterine evolution of a cephalocele into an atretic cephalocele.

Actinobaculum schaalii an emerging pediatric pathogen?

BACKGROUND: Actinobaculum schaalii was first described as a causative agent for human infection in 1997. Since then it has mainly been reported causing urinary tract infections (UTI) in elderly individuals with underlying urological diseases. Isolation and identification is challenging and often needs molecular techniques. A. schaalii is increasingly reported as a cause of infection in humans, however data in children is very limited. CASE PRESENTATION: We present the case of an 8-month-old Caucasian boy suffering from myelomeningocele and neurogenic bladder who presented with a UTI. An ultrasound of the urinary tract was unremarkable. Urinalysis and microscopy showed an elevated leukocyte esterase test, pyuria and a high number of bacteria. Empiric treatment with oral co-trimoxazole was started.Growth of small colonies of Gram-positive rods was observed after 48 h. Sequencing of the 16S rRNA gene confirmed an A. schaalii infection 9 days later. Treatment was changed to oral amoxicillin for 14 days. On follow-up urinalysis was normal and urine cultures were negative. CONCLUSIONS: A.schaalii is an emerging pathogen in adults and children. Colonization and subsequent infection seem to be influenced by the age of the patient. In young children with high suspicion of UTI who use diapers or in children who have known abnormalities of their urogenital tract, infection with A. schaalii should be considered and empiric antimicrobial therapy chosen accordingly.

Local resection of metanephric adenoma with kidney preservation.

Metanephric adenoma (MA) is a rare renal neoplasm present at any age. Usually, a total nephrectomy is performed. Some successful partial nephrectomies or selective tumor resections are described in adults but not in children. We present here the case of a 15-month-old boy, the youngest patient yet to be reported with an MA. We performed a local resection of the tumor. Today, 2 1/2 years after surgery, we can document a favorable clinical course and normal ultrasound findings in the follow-up of the operated kidney. This case gives further evidence that organ-sparing resection of MA is feasible in selected cases. A close clinical and ultrasound follow-up is mandatory for this approach.