Introduction: Posterior fossa anomalies, hemangioma, arterial anomalies, cardiac anomalies, eye anomalies, and sternal anomalies (PHACES) syndrome is a rare, multi-system disorder. Case Report: We illustrate a case of a 1-month-old female who presented emergently with an enlarging, large, left-sided hemangioma segmentally distributed over the ophthalmic branch of the trigeminal nerve (V1) region prompting clinical concern for PHACES syndrome. Further workup was recommended to evaluate for extracutaneous involvement, which subsequently revealed coarctation of the aorta and multiple arterial abnormalities. Conclusion: The vast array of potential, devastating complications linked to PHACES syndrome highlights the importance of prompt identification when confronted with hemangiomas, especially in the pediatric population. We further present a photo series demonstrating the evolution of our patient’s infantile hemangioma from its subtle initial stages through its rapid evolution leading to her emergent presentation, emphasizing the importance of early clinical suspicion and intervention.

Introduction: Physicians often treat patients in the urgent and non-urgent settings who present with various types of foreign bodies lodged in the external auditory canal (EAC). This can cause significant distress to the patient, as well as the caregiver in pediatric cases. Typically, these objects are inanimate and include beads, rocks, toys, craft supplies, food particles, and jewelry. However, sometimes the patient will present with biotic organisms in the ear that seem to cause considerably more distress and damage, including otalgia and otorrhea from abrasions to the EAC, or damage to the tympanic membrane. These creatures can present alive or dead in the EAC. The culprits can include crawling and flying species. However, even rarer, the larval stages have been reported. When an ear is infested by Diptera larvae, the juvenile stage of a fly, the parasitosis is known as aural myiasis. Case Report: In this case, a 7-year-old boy in acute distress presented to the emergency department with left ear otalgia and pruritis. His ear canal was covered in dried blood, and he was found to have 15–20 maggots belonging to the Sarcophagidae family in the left EAC that were removed via lidocaine application and normal saline irrigation. Conclusion: The patient and his caregivers were educated on hygienic practices and advised on outpatient follow-up. Further research is indicated for best practices treating aural myiasis and establishing environmental risk factors.

Introduction: Secondary syphilis, also termed the great mimicker due to its vast array of clinical presentations, is associated with skin manifestations including rupioid, macular, psoriasiform, and condylomatous lesions. On clinical examination alone, it can be difficult to differentiate between rupioid syphilis and rupioid psoriasis, especially among cases of human immunodeficiency virus (HIV) and syphilis co-infection; however, histologic examination can aid in their differentiation. Additional testing such as serum rapid plasma reagin (RPR), anti-HIV antibody, rheumatoid factor, and fungal cultures should be performed to exclude other causes of rupioid lesions. Case Report: A 19-year-old HIV-positive male developed numerous hyperkeratotic skin lesions involving the head, trunk, and extremities over a 4-week period. His RPR was positive (1:64), suggesting a diagnosis of rupioid syphilis. The patient was treated with doxycycline, given a reported history of penicillin allergy. Rapid plasma reagin titers decreased to 1:2 following treatment; however, the skin eruption did not improve. A biopsy of a lesion demonstrated psoriasiform acanthosis with confluent hyperkeratosis, clusters of intracorneal neutrophils, and negative spirochete immunostaining. Based on histopathology and clinical presentation, a diagnosis of rupioid psoriasis was favored. The patient was started on targeted anti-psoriatic therapy with apremilast and maintains close follow-up with his dermatologist every three months. Conclusion: Although the patient’s presentation was initially presumed to be secondary to syphilis, his lack of response to treatment prompted further workup to assess the etiology of the patient’s skin findings more accurately. In doing so, a diagnosis of rupioid psoriasis was favored based on the histologic findings observed.

Introduction: Osteosarcoma is the most common malignant bone tumor in children, most often occurring in the lower extremity of the femur, and is known to be particularly aggressive, with a high risk of metastasis. The most frequent secondary localizations are pulmonary and bone metastases; peritoneal metastases are extremely rare and have a poor prognosis. Case Report: We report the case of a 14-year-old child with a history of a treated right femoral osteosarcoma, which presented with peritoneal and pulmonary metastases. Conclusion: Imaging plays a very important role in the management of patients with osteosarcoma, it allows an early detection of metastases through a rigorous clinicoradiological surveillance, metastases usually have the same radiological appearance as the primary tumor and the diagnosis of certainty is essentially based on anatomopathological study.

Introduction: The Paenibacillus genus consists of saprophytic organisms that are commonly associated with soil, water, plants, feces, and diseased insect larvae. Human infection is rare. This disease typically occurs in immunocompromised hosts, adults with a history of intravenous drug use, and hosts with prosthetic medical devices. There are a limited number of case reports describing Paenibacillus infections in neonates. This is the second published instance of pediatric meningoencephalitis caused by Paenibacillus thiaminolyticus in a preterm infant with intrauterine drug exposure. Case Report: A 37-day-old male infant with a history of prematurity of 33 weeks completed gestation presented to the Emergency Department for acute onset poor feeding, poor color, and unresponsiveness at home. Examination revealed cyanosis, apnea, and hypotonia. Vital signs were significant for hypotension and hypothermia. Initial labs revealed a metabolic acidosis, elevated C-reactive protein, normal complete white blood cell count, and a negative viral respiratory pathogen panel. Aerobic blood culture and cerebrospinal fluid (CSF) culture were positive for P. thiaminolyticus within 24 hours. Cranial ultrasound and magnetic resonance imaging revealed changes concerning for liquefactive meningoencephalitis. The infant was admitted to the neonatal intensive care unit (NICU) and ultimately discharged home on a “do not resuscitate/do not intubate” status and later died at 11 months of age. Conclusion: Paenibacillus species are common environmental organisms but can cause devastating disease in neonates. This is the second reported case of a preterm infant with P. thiaminolyticus infection and inutero drug exposure (IUDE), supporting that prematurity and IUDE may be risk factors for severe disease.

Introduction: Chronic kidney disease (CKD) among children is on the rise, both locally and globally. The burden of managing CKD in children from resource-poor centers and the developing nations as a whole is quite challenging. Pediatric CKD has remained a death sentence in many poor nations of the world, including Nigeria. Chronic kidney disease is asymptomatic in its earliest stages, although urinalysis findings or blood pressure may be abnormal. As CKD progresses to more advanced stages, signs and symptoms greatly increase, making the diagnosis of CKD more obvious. Case Report: We hereby report a rare presentation of only headache, sudden blindness, hypertension, and retinal detachment as the only pointer to end stage kidney disease (ESKD) in a 15-year-old girl. Conclusion: There is need for nephro-prevention in children, and a higher index of suspicion for CKD, with the intention of reducing or halting disease progression at earlier stages of the disease.

Introduction: Meconium peritonitis is a rare disease which can be potentially fatal. Case Report: We present the history of a full-term male infant born at 2945 g via lower segment cesarean section (LSCS). There was an antenatal diagnosis of an intra-abdominal cyst. At 27+6 weeks gestation, the ultrasound scan noted a thick-walled cystic mass superior to the bladder. Postnatally, an X-ray showed a paucity of gas in the right lower quadrant and an ultrasound scan confirmed a large central avascular cystic lesion, with posterior sediments noted. Laparotomy revealed extensive adhesions surrounding a meconium filled cyst. This was excised and a primary anastomosis performed. However, he failed to progress with feeds, having intermittent abdominal distension, and after a contrast study on day 18 post op which showed failure of contrast to pass the dilated proximal jejunum, he was taken back to the operating room and was found to have significant adhesions. Adhesiolysis was done and the anastomosis was patent and well healed. However, these episodes of abdominal distension continued, and he never progressed with feeds. On day 39 of life, there was severe metabolic acidosis refractory to resuscitation, leading to cardiac arrest. Conclusion: There can be subtle presentations of meconium peritonitis in the newborn and it should be considered in any neonate with an intra-abdominal cyst.

Introduction: Hemophagocytic lymphohistiocytosis is a rare condition with dysregulated multi-organ inflammation that may cause acute liver failure. It often presents with non-specific clinical features and can be difficult to diagnose. Case Report: We present the case of a 2-year-old girl with lethargy, encephalopathy, poor oral intake, vomiting, and jaundice. Her labs indicated acute liver failure with additional findings of bicytopenia and elevated ferritin. Given high concern for hemophagocytic lymphohistiocytosis, we promptly consulted the hematology/oncology, transplant hepatology, and pharmacy services. Bone marrow biopsy initially showed no evidence of hemophagocytosis. Given our high clinical index of suspicion for hemophagocytic lymphohistiocytosis and its high mortality and morbidity, we initiated treatment with high dose dexamethasone, etoposide, and the recently approved biologic drug emapalumab, a monoclonal antibody against interferon gamma, despite not meeting full HLH-2004 diagnostic criteria. One day after treatment initiation, the final review of the bone marrow biopsy showed evidence of hemophagocytosis. Ultimately, with implementation of multidisciplinary rounds, close neurologic examinations, aggressive management of evolving hyperammonemia and cerebral edema, and early initiation of treatment, our patient achieved full liver recovery. Conclusion: This patient’s presentation emphasizes the importance of having a broad differential when a patient presents with liver failure and cytopenias. Our management of this patient showcases the importance of expedient, multidisciplinary management for a complex critically ill pediatric patient. The patient’s survival and complete liver recovery with the treatment protocol given suggests emapalumab should be studied in future clinical trials as an important adjunctive treatment for patients with hemophagocytic lymphohistiocytosis with acute liver failure.