Congenital Hepatic Fibrosis in a 2-Year-Old Child Presenting with Fever of Unknown Origin.

Congenital hepatic fibrosis is a rare, autosomal recessive, fibro-polycystic disease resulting from ductal plate malformation, leading to proliferation and fibrosis of bile ducts. Progressive hepatic fibrosis leads to portal hypertension and varices which can present with life threatening gastrointestinal hemorrhage. We report a case of congenital hepatic fibrosis in a 2-year-old child who presented with 8 days of fever without any significant medical history or physical examination findings.

Ophthalmoplegia and cranial nerve deficits in an adolescent with headache.

Tolosa-Hunt syndrome is an idiopathic, inflammatory condition involving the cavernous sinus and is characterized by unilateral, painful ophthalmoparesis. The condition often begins with retro-orbital pain followed by select cranial nerve involvement. We report the case of a 17-year-old female whose presentation with progressive left-sided headache and ophthalmoparesis culminated in the diagnosis of Tolosa-Hunt syndrome. While many of her signs and symptoms have been previously reported in the rare pediatric cases of Tolosa-Hunt syndrome described in the literature, this case illustrates a unique presentation involving cranial nerves V and VII in addition to the more commonly reported cranial nerve III, IV, and VI palsies.

Use of Copeptin Levels to Predict the Resolution of Transient Postoperative SIADH.

BACKGROUND: Copeptin levels reflect vasopressin activity and help classify osmoregulatory disorders. There is limited pediatric experience using copeptin to diagnose and manage diabetes insipidus, syndrome of inappropriate antidiuretic hormone secretion (SIADH), and bi- or tri-phasic postsurgical osmoregulatory disorders. In this report, we describe serial copeptin levels in an infant who developed transient SIADH after neurosurgery. CASE DESCRIPTION: A 4-month-old infant with no prior pituitary dysfunction underwent endoscopic fenestration of a large arachnoid cyst (3.5 × 4.7 × 3.8 cm). He developed SIADH on postoperative day 4 with seizures, hyponatremia (sodium 121 mmol/L), and concentrated urine (535 mOsm/kg). His initial copeptin level was inappropriately high in the context of his hyponatremia. Copeptin levels decreased as his SIADH resolved. Serial copeptin levels correlated to the infant's increased ability to dilute urine. CONCLUSION: Copeptin levels in this infant are consistent with levels described in adults and older children. Obtaining copeptin levels may improve providers' ability to quickly diagnose and manage SIADH amongst other heterogeneous causes of hyponatremia. Lastly, trending copeptin levels improved providers' ability to monitor SIADH progression, and may allow preemptive fluid titration for children with bi- or tri-phasic shifts in osmoregulation after neurological procedures.