Driscoll Children's Hospital

Mycotic pseudoaneurysms can be fatal due to spontaneous rupture and therefore early diagnosis may prevent such outcome. However, this proves to be a challenge due to non-specific symptoms such as back pain or respiratory distress. This case report aims to highlight mycotic pseudoaneurysms as a differential diagnosis given that it may be a fatal complication of MRSA bacteremia. A 9-year-old female with a history of MRSA bacteremia three months ago presented to an outside hospital due to respiratory distress with concerns for pneumonia versus atelectasis. She was transferred to our hospital for escalation of care. On arrival, she had severe left-sided back pain and shortness of breath. Chest x-ray showed complete atelectasis of the left lung (Image 1). Chest CT scan revealed occlusion of the left mainstem bronchus with left mediastinal shift. Echocardiogram was normal. Atelectasis did not improve despite pulmonary clearance regimen (chest physiotherapy, albuterol, hypertonic saline). Reassessment via Chest CT scan revealed a large mycotic pseudoaneurysm that appeared to have a thrombus located in the descending aorta, compressing the left main bronchus and causing atelectasis on the left lung with mediastinal shift (Image 2). She underwent cardiac catherization, which revealed a large mycotic aneurysm on the descending aorta measuring 18 mm x 15 mm with thrombus around the periphery. The lesion produced a mass effect, compressing the left main bronchus, leading to atelectasis. The pseudoaneurysm was treated via complete coil occlusion to prevent any residual flow to the outpouching sac (Image 3). Following recovery, a repeat chest CT showed improvement of aeration over the left lung field and bronchus. This is the first report regarding the development of a mycotic pseudoaneurysm in the descending aorta among the pediatric population. Manifestations center around the size and the resultant mass effect around the surrounding structures. Sudden, severe back pain, shortness of breath and cough place mycotic pseudoaneurysm higher on the differential diagnosis in a patient with a history of systemic MRSA infection. Hematogenous seeding can occur from previous infective endocarditis or septic foci resulting in direct injury to the lining of the descending aorta and subsequent mycotic pseudoaneurysm. Spontaneous rupture at any moment may prove fatal. CT angiography is the diagnostic gold standard and surgical or endovascular intervention is the definitive treatment.

Disclosure: R. Solomon: None. N. Mitre: None. R. Senguttuvan: None. F.J. Al Muhaisen: None.Introduction: Costello Syndrome (CS) is a rare genetic disorder with an estimated number of affected people 300 worldwide. It is caused by a heterozygous variant mutation in the HRAS gene. Clinical features of CS include dysmorphic facial features, developmental delay, cardiac abnormalities, and increased malignancy risk. Suboptimal growth and short stature have been reported in patients with CS. Growth hormone (GH) therapy is often considered to improve height and metabolic outcomes, though its efficacy and safety in syndromic short stature remain debated. This report highlights the role of GH therapy in CS, evaluates clinical response and growth outcomes, and contributes to evidence-guiding management. Case Presentation: We report a 16 year-year-old male referred to the endocrine clinic for severe short stature. His height was reported to be 147.2 cm (Z score -3.27). Weight was 35.9 kg (Z score -3.93). He was Tanner Stage III male. Physical exam findings included coarse facial features, micrognathia, hypertelorism, frontal bossing, and low-set ears. Initial lab work revealed normal total Testosterone at 601ng/dL (300-900). Further endocrine evaluation was within normal limits, including TSH, FT4, LH, FSH, celiac screening, 17OHP, and DHEAS. Bone age X-ray was read at 13.5 years at a chronological age of 16 and 1 month. GH stimulation testing was obtained, and the patient passed the test with a GH level of 21.3 ng/mL (>10). Given his abnormal physical exam findings, a Symptom Driven Exome sequence revealed a heterozygous pathogenic HRAS mutation, consistent with Costello syndrome. Given his severe short stature (Z score -3.27), GH therapy was initiated at the age of 16 years and 7 months. His GH dose was between 0.30-0.32 mg/kg/week in the last 2 years. His growth velocity improved with maximum GV at 10.3 cm/year, with a total increase of 13 cm over two years. His last bone age x-ray was consistent with 15 years at a chronological age of 18, 5 months. His current height is 164.1cm (Z score -1.71). Discussion: Costello Syndrome belongs to the RASopathy family, affecting growth. While the precise mechanisms remain unclear, GH insensitivity and impaired IGF-1 production have been implicated. Although this patient passed GH stimulation testing, GH therapy was beneficial, resulting in improved growth velocity. This suggests that even without classic GH deficiency, therapy may enhance growth outcomes in CS. Current literature highlights mixed results regarding GH efficacy, with concerns about potential risks such as hypertrophic cardiomyopathy and malignancy. No adverse effects were observed in this case. Endocrinologic evaluation in CS is complex, necessitating individualized management. This case underscores the importance of early genetic screening in short stature to avoid delayed diagnoses. It suggests that GH therapy can benefit even when conventional diagnostic criteria are unmet.Presentation: Saturday, July 12, 2025

LUMBAR syndrome is the association of segmental infantile hemangiomas that affect the Lower part of the body with Urogenital anomalies, hemangioma Ulceration, spinal cord Malformations, Bony deformities, Anorectal malformations, Arterial anomalies and/or Renal anomalies. The etiology is not known but is suspected to be multifactorial, involving genetic and environmental factors. We retrospectively reviewed a large database of 109 published reports of LUMBAR syndrome to study potential associated clinical risk factors, the first such effort. LUMBAR is significantly more common in full-term, normal birth weight, singleton girls. We found no statistically significant differences in disease severity between affected girls and boys. There were no reports in twins or other multiple births, no reports of familial recurrence, and no repeated maternal illnesses, exposures, or other prenatal risk factors. Prospective studies in LUMBAR syndrome are needed to further evaluate maternal risk factors for prenatal hypoxia, gene-environment interactions, and genetic susceptibility variants.

To address the unmet need for a smaller pacemaker for babies, a specially modified implantable pulse generator was developed containing a Medtronic Micra subassembly in a polymer header connecting to a bipolar epicardial lead. The aim of this study was to report midterm follow-up data and outcomes of patients who underwent implantation of this device. Deidentified data were collected from 12 of 15 sites in the United States implanting the pediatric implantable pulse generator between March 2022 and February 2024. All 29 patients at these 12 sites within this timeframe were included in the analysis. The median age at implant was 15 days (range, 0 days to 3 years, including 1 outlier). The median weight was 2.3 kg (range, 1.3-11.4 kg). Gestational age was 28.5 weeks to term, with 23 (79%) patients born prematurely. Of those with anatomic information, 25% had congenital heart disease. The average duration of implant was 325 days (73-808 days). The most recent lead impedance mean was 612 ohms (450-840 ohms), ventricular capture threshold mean was 1 V @ 0.4 ms (range, 0.38-2.75 V), and R-wave sensing mean was 12.5 mV (3.6-20 mV). There were 7 generator explants (24%), removed at 6.5 to 31 months of age. The pediatric implantable pulse generator can be safely implanted in neonates and infants. This multicenter report demonstrates that the devices remain stable, with effective pacing, normal electrical parameters, and battery longevity aligned with projections. This novel pediatric pacemaker provides a viable alternative to standard-size generators and addresses a vital unmet need for these small patients.

To improve continuity of care, some PICUs assign a continuity attending (CA) physician for children with prolonged hospitalizations. Little is known about how this intervention impacts familial caregivers' experiences. The objective of this study was to provide in-depth descriptions of family perspectives about continuity of care during prolonged PICU hospitalizations for children with and without a PICU CA. Qualitative semi-structured interviews. Single center cohort, from October 2021 to December 2022, at an academic PICU in the United States. Familial caregivers (n = 39) of critically ill children hospitalized in the PICU for greater than or equal to 14 days were purposively sampled, stratified by group: 18 with a PICU CA and 21 without a PICU CA. None. Semi-structured interviews were audio recorded, transcribed, coded, and analyzed in the context of the continuity of care model using a realist thematic approach. Familial caregivers described six themes related to relational, informational, and management continuity: 1) familiar providers who demonstrate empathy for the child improve family members' comfort and trust (Relational); 2) providers who know and use a child's baseline health status to inform clinical decision-making alleviate family members' stress (Relational, Management); 3) information loss during care team transitions frustrates families (Informational, Management); 4) known providers enhance caregiver communication (Informational); 5) familiar providers who value a family's expertise about their child's care (Relational, Management); and 6) take responsibility for the child's long-term care plan (Management) decrease parental stress. As PICU patient medical complexity and length of stay increase, familial caregivers' needs transition from understanding day-to-day management to navigating care team transitions and partnering with providers to develop long-term care plans. Targeted interventions to increase provider continuity that consider relational, informational, and management continuity are needed to optimize patient outcomes and family experiences.

The mirid bugs Lygus hesperus (Knight) and L. elisus (van Duzee) are key pests of forage, fiber, and fruit crops. Our goals were to identify pheromone components produced by females of both species and to develop practical pheromone dispensers for use in monitoring these pests. Volatiles collected from virgin female L. elisus contained (E)-2-hexenyl butyrate (E2HB) as the major component with lesser amounts of hexyl butyrate (HB) and (E)-4-oxo-2-hexenal (E4OH) (ratio 117.2:100:17.1, respectively), whereas volatiles and solvent extracts from L. hesperus contained HB and E4OH as major components, with only small amounts of E2HB (100:23.6:3.4, respectively in volatiles). Dispensers fabricated from pipette tips released the components at ~10 µg/d in a ratio similar to the loading ratio. These lures were used to optimize the pheromone blends in field studies from 2012 to 2017. Blends of E2HB and E4OH attracted L. elisus, and a 100:60 blend was optimal. Blends of HB and E4OH attracted L. hesperus, and a 100:60 blend was adopted as a base blend. The additions of possible minor components such as (Z)-3-hexenyl butyrate, (E)-2-hexenal, or 1-hexanol did not improve the attraction of L. hesperus. In trials in alfalfa and strawberry, traps baited with blends of HB:E4OH (100:60) were equally or more effective for monitoring L. hesperus than sweep or vacuum samples, with pipette tip dispensers lasting 2–3 weeks under field conditions. The numbers of L. hesperus captured were lower than expected as compared with reports of pheromone trapping for other Lygus spp. Some possible reasons were investigated.

The imbalance in anesthesia workforce supply and demand has been exacerbated post-COVID due to a surge in demand for anesthesia care, especially in non-operating room anesthetizing sites, at a faster rate than the increase in anesthesia clinicians. The consequences of this imbalance or labor shortage compromise healthcare facilities, adversely affect the cost of care, worsen anesthesia workforce burnout, disrupt procedural and surgical schedules, and threaten academic missions and the ability to educate future anesthesiologists. In developing possible solutions, one must examine emerging trends that are affecting the anesthesia workforce, new technologies that will transform anesthesia care and the workforce, and financial considerations, including governmental payment policies. Possible practice solutions to this imbalance will require both short- and long-term multifactorial approaches that include increasing training positions and retention policies, improving capacity through innovations, leveraging technology, and addressing financial constraints.

Abstract Craniofacial dermoid cysts are congenital anomalies that rarely have intracranial extension and can be associated with other anomalies. Common sites of these lesions are the lateral brow and parietal scalp. Presentation of the dermoid cyst in the occipital region with intracranial extension is extremely rare. We report a 2-year-old female with a presentation of an occipital dermoid cyst with intracranial extension and secondary cerebellar abscess. This case highlights the rarity of the presentation of an occipital dermoid cyst with intracranial extension and secondary infection and the importance of early imaging for suspected dermoid cysts in the occipital region for identification of intracranial extension.