Hospital Variation and Resource Use for Infants with Craniosynostosis Undergoing Open, Endoscopic, and Distraction Osteogenesis Surgical Techniques.

BACKGROUND: Craniosynostosis is treated with endoscopic, open, and/or distraction surgical techniques. We assessed institutional variation in the use these techniques for craniosynostosis and compared hospital resource use. METHODS: Retrospective analysis of 5249 infants age <18 months old undergoing surgical procedures for all types of craniosynostosis in 2016-2020 in 39 freestanding children's hospitals in the Pediatric Health Information System (PHIS) database. Endoscopic vs. open cranial vault surgery (with and without distraction osteogenesis) was identified using ICD-10-CM codes. Inpatient cost and length of stay (LOS) were compared by surgery type with Wilcoxon Rank Sum. RESULTS: There was significant (p < .001) variation in the percentage of infants who underwent endoscopic repair across hospitals [median 23.6% (interquartile range (IQR): 7.6%-37.5%), range: 0% to 80.4%] and across regions [range: 22.1% (southeast) to 42.5% (northeast)]. For endoscopic procedures, median LOS and inpatient cost were lower (p < .001) without vs. with distraction [1 day (IQR 1-1) vs. 2 days (IQR 2-2); $14,617 (IQR 11,823-22178) vs. $33,599 (IQR 22,800-38,619)]. For open interventions, median LOS and inpatient cost were also lower (p < .001) without vs. with distraction [3 days (IQR 2-4) vs. 5 days (IQR 4-6) and $37,251 (IQR 27,114-50.320) vs. $62,247 (IQR 42,124-91,620)]. CONCLUSIONS: Substantial variation in the surgical approach for craniosynostosis exists across hospitals and regions. Endoscopic repair without distraction had the lowest hospital resource use, while open repair with distraction had the highest hospital resource. Subsequent analysis of short- and long-term outcomes as well as patient-and-family costs is necessary to assess the true cost-effectiveness of each approach.

Orbital and Eyelid Characteristics, Strabismus, and Intracranial Pressure Control in Apert Children Treated by Endoscopic Strip Craniectomy versus Fronto-Orbital Advancement.

Apert syndrome is characterized by eyelid dysmorphology, V-pattern strabismus, extraocular muscle excyclorotation, and elevated intracranial pressure (ICP). We compare eyelid characteristics, severity of V-pattern strabismus, rectus muscle excyclorotation, and ICP control in Apert syndrome patients initially treated by endoscopic strip craniectomy (ESC) at about 4 months of age versus fronto-orbital advancement (FOA) performed about 1 year of age. Methods: Twenty-five patients treated at Boston Children's Hospital met inclusion criteria for this retrospective cohort study. Primary outcomes were magnitude of palpebral fissure downslanting at 1, 3, and 5 years of age, severity of V-pattern strabismus, rectus muscle excyclorotation, and interventions to control ICP. Results: Before craniofacial repair and through 1 year of age, none of the studied parameters differed for FOA versus ESC treated patients. Palpebral fissure downslanting became statistically greater for those treated by FOA by 3 (P < 0.001) and 5 years of age (P = 0.001). Likewise, severity of palpebral fissure downslanting correlated with severity of V-pattern strabismus at 3 (P = 0.004) and 5 (P = 0.002) years of age. Palpebral fissure downslanting and rectus muscle excyclorotation were typically coexistent (P = 0.053). Secondary interventions to control ICP were required in four of 14 patients treated by ESC (primarily FOA) and in two of 11 patients initially treated by FOA (primarily third ventriculostomy) (P = 0.661). Conclusions: Apert patients initially treated by ESC had less severe palpebral fissure downslanting and V-pattern strabismus, normalizing their appearance. Thirty percent initially treated by ESC required secondary FOA to control ICP.

Normal Neonatal Sleep Defined: Refining Patient Selection and Interpreting Sleep Outcomes for Mandibular Distraction.

BACKGROUND: Although polysomnography is paramount when evaluating neonatal airway obstruction, "normal" published references do not exist. We present normative polysomnography data for newborns age 0-1 month. We compare this reference to pre and postoperative sleep data from infants undergoing mandibular distraction osteogenesis (MDO) at this same age. METHODS: Following IRB approval, normative subjects were recruited from our neonatal intensive care unit to undergo nap polysomnography. One blinded sleep physician read all studies. From 2016 to 2019, we prospectively collected sleep data for newborns undergoing MDO. RESULTS: In total, 22 neonates without airway obstruction provided normative sleep data. Median total apnea-hypopnea index (AHI), obstructive apnea-hypopnea index (OAHI), and central apnea index (CAI) were 7.3, 4.9, and 0.7 events/hour. Median O2 nadir was 91%. Polysomnography for 13 neonates before MDO and during consolidation showed median preoperative AHI was 38.3, OAHI was 37.0, CAI was 1.9, and median O2 nadir was 83%. Following MDO, median AHI was 6.1, OAHI was 4.0, CAI was 1.3, and median O2 nadir was 92.5%. Paired t-tests confirmed significant improvements in all indices; when comparing the postoperative group with the normative group, there was no difference in oxygenation nor any respiratory index. CONCLUSIONS: "Normal" neonates have more obstructive events and lower oxygenation nadirs than previously appreciated. We provide normative nap polysomnography values for this age group and encourage centers with multidisciplinary MDO teams to utilize this data to calibrate patient selection algorithms, inform treatment discussions, and better understand surgical outcomes. Limitations include a small sample size and single institution study.

Pediatric Mandible Reconstruction for Osteomyelitis During Largest Reported Mycobacterium Abscessus Outbreak.

In 2016, water lines at a children's dental clinic in Orange County, California were contaminated with Mycobacterium abscessus (MA), a non-tuberculosis rapidly-growing mycobacterium, leading to the largest MA outbreak ever reported. Mandatory reporting and active case finding directed by the Public Health Department was conducted in collaboration with community Pediatric Infectious Disease physicians for patients who underwent dental pulpotomies at the contaminated Dental Clinic from January 1 to September 6, 2016. Seventy-one cases (22 confirmed and 49 probable) were identified. One case that required extensive debridement and reconstruction of the mandible is presented in detail. CT maxillofacial demonstrated osteomyelitis extending from the right mandibular angle to the left ramus with multifocal periapical lucencies. CT chest and neck revealed numerous pulmonary nodules and bilateral cervical lymphadenopathy. Extraction of several involved teeth, bilateral selective neck dissection, and extensive mandibular debridement was performed, followed by mandibular stabilization with a custom pre-bent 2.0-mm locking plate. CT images 1-year post-operative showed clearance of infection and sufficient bony stability. Subsequent removal of hardware and bone grafting was performed and the patient is doing well. In the event of a future odontogenic mycobacterium outbreak, the experience at our institution can inform multidisciplinary treatment approaches. Prophylactic extraction of primary teeth that received pulpotomies with contaminated water should be performed. Early and thorough debridement of affected bone, including enucleation of secondary teeth, should be performed if necessary for early source control.

Evaluation of Ventilation Tube Placement and Long-term Audiologic Outcome in Children With Cleft Palate.

OBJECTIVE: The purpose of this study was to assess the effect of ventilation tube (VT) placement on long-term hearing outcomes in children with cleft palate. STUDY DESIGN: Case series with chart review. SETTING: Genetic and dysmorphology database at Rady Children's Hospital-San Diego (RCHSD). PATIENTS: Children with cleft palate diagnosis who underwent surgery at RCHSD between 1995 and 2002. MAIN OUTCOME MEASURE: The primary outcome studied was hearing acuity at 10 years of age. Independent variables studied included gender, age at palate repair and first VT placement, total number of VTs, number of complications, and presence of tympanic membrane perforation. RESULTS: An increased number of tubes was associated with a greater incidence of hearing loss at age 10, even after adjusting for total number of otologic complications. The timing of initial tube placement did not have a significant effect on long-term hearing outcome in this study. CONCLUSIONS: While children with worse middle ear disease are more likely to receive more tubes and have long-term conductive hearing loss as a result of ear disease, the results of this study suggest that multiple tube placements may not contribute to improved long-term hearing outcomes. Further research focusing on long-term outcomes is needed to establish patient-centered criteria guiding decision making for ventilation tube placement in children with cleft palate.

Undescended parathyroid adenoma.

Undescended parathyroid adenomas are rare, representing 0.08% of all parathyroid adenomas; however, they make up 7% of the underlying cause of failed cervical exploration in patients with persistent primary hyperparathyroidism. A 43-year-old woman with no significant medical or family history presented with fatigue and was diagnosed with primary hyperparathyroidism; however, preoperative imaging including sestamibi scan and ultrasound was unable to identify the hyperfunctioning gland. She underwent a neck exploration and hemithyroidectomy and partial parathyroidectomy with failure of resolution of her disease. Subsequent work up including a CT of the neck demonstrated a 1.9 cm mass adjacent to the left submandibular gland. This was removed with postoperative normalisation of the patient's serum calcium and parathyroid hormone levels.