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.

A Survey on Enhanced Recovery After Surgery (ERAS) Elements in Cleft Palate Repair.

This study aims to characterize current use, knowledge, and attitude toward ERAS protocols by academic craniofacial surgeons.Craniofacial surgeons were provided with electronic surveys.Electronic survey; Institutional tertiary surgeons.102 cleft palate surgeons surveyed and 31 completed the survey (30.4%).None.Respondents rated their knowledge, use, and willingness to implement perioperative interventions modeled after adult ERAS protocols.Majority (67.7%) rated they were knowledgeable about ERAS. However, 61.3% "never use" a standardized protocol for cleft palate surgery. Only 3 ERAS elements are currently implemented by a majority of cleft surgeons: avoiding prolonged perioperative fasting (67.7%), using hypothermia prevention measures (74.2%), and minimizing use of opioids (62.5%). A large majority of respondents noted they never administer bolus (71.0%) or infusion (80.6%) dosing of tranexamic acid; most of these surgeons also indicated that administering tranexamic acid "would not be a valuable addition" (67.7% and 71.0%, respectively). Short-acting sedatives are used by 12.9% and by 16.1% of surgeons in all patients during extubation and postoperative recovery, respectively. By contrast, 22.6% never use such agents during extubation and 48.4% never use it during postoperative recovery. Overall, 67.7% of respondents replied that they would be willing to implement an ERAS protocol for cleft palate repair.Many respondents report using interventions compatible with an ERAS approach and the majority are willing to implement an ERAS protocol for cleft palate repair.

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.

Ultrasound Evaluation of Primary Alveolar Grafting in Cleft Lip/Palate Treatment: Development of a Novel Sonographic Grading System.

OBJECTIVE: To explore ultrasonographic evaluation of primary alveolar repair in cleft lip/palate patients and develop a grading system to assess outcomes of graft success. DESIGN: Sixteen patients with an average age of 2 years 1 month had sonograms performed at various points in their treatment to determine the feasibility of ultrasound in visualizing alveolar bone defects and changes over time postgrafting. A total of 23 sonograms were performed: 21 at an average of 12 months postoperatively and 2 at an average of 1 month preoperatively. MAIN OUTCOME MEASURES: A 10-point grading system was developed assessing three categories: locations of lateral bone bridging across the cleft, quantification of residual defects with air or fluid channels, and locations of calcification. Three operators graded 10 sonograms to assess interobserver reliability, and the scores were also validated against dental radiographs in patients old enough for radiographic imaging. RESULTS: Linear weighted kappa statistics revealed substantial interobserver agreement for total scores, with an average kappa value of .708. In limited patients with radiographs, a total score of 9/10 correlated with a Chelsea score of 6.5/8 and category A. CONCLUSIONS: Sonographic evaluation, coupled with this novel grading system, shows potential for early assessment of outcomes of graft success when evaluating new techniques of primary alveolar grafting.

Congenital-infantile fibrosarcoma presenting as a hemangioma: a case report.

INTRODUCTION: Congenital-infantile fibrosarcoma (CIFS) is a rare pediatric soft tissue tumor that on initial clinical presentation can mimic an infantile hemangioma. Management of these conditions differs drastically, and delay in diagnosis and treatment may affect prognosis. METHODS: A 6-month-old male infant originally presented to dermatology for evaluation of a suspected right buttock hemangioma, present at birth as a small red mass just lateral to the gluteal cleft. The lesion rapidly grew over 2 weeks to a size of 4×4.5×4.5 cm, with violaceous color change and central ulceration. The patient received a course of cephalexin; however, the mass became painful with several episodes of bleeding and did not show clinical improvement. Magnetic resonance imaging and ultrasound supported the diagnosis of atypical hemangioma. RESULTS: Plastic Surgery was consulted for excision due to recurrent bleeding. An excisional biopsy was undertaken and pathologic finding showed CIFS. The case was discussed with Hematology/Oncology and Pediatric Surgery, and the patient underwent re-excision and obtained clear margins. Initial metastatic workup was negative; however, patient had evidence of recurrent disease as well as pulmonary metastasis on his most recent surveillance imaging. He underwent re-excision of the gluteal mass and is currently receiving adjuvant chemotherapy. CONCLUSIONS: This case demonstrates a rare malignancy initially misdiagnosed as a hemangioma. Consideration of CIFS is crucial in cases of complicated hemangiomas not responding to standard treatment. Treatment calls for excision with wide margins and/or adjuvant chemotherapy if excision is not possible. Classic pathologic findings, immunohistochemistry, and/or reverse transcription-polymerase chain reaction can confirm the diagnosis. A correct and rapid diagnosis of CIFS is critical to optimizing treatment and subsequent prognosis.

Reverse Distraction Cranioplasty for Treatment of Hydrocephalic Megalocephaly.

Macrocephaly resulting from untreated hydrocephalus is a rare but difficult condition to treat. The patient presented is a 6-year-old boy who had progressively increasing head size since birth secondary to untreated hydrocephalus with associated developmental delay. His initial head circumference was 69 cm, and computed tomography scan showed evidence of obstructive hydrocephalus. For the first stage of the procedure, bicoronal and circumferential strip craniectomies were performed, 5 fully open 3.5 mm midface distractors were placed to facilitate vault reduction, and absorbable plates were placed in the frontoorbital region. Neurosurgery also performed an endoscopic third ventriculostomy and placed an external ventricular drain. Reverse distraction was completed over 5 days and tolerated well by the patient. Three months after the first procedure, the distractors were removed and frontoorbital reduction and additional cranial vault reduction and reshaping were performed. At 1 month following this operation he was noted to have excellent forehead contour and dramatic improvement of his macrocephaly with some remaining mid-vault asymmetry. His head circumference was measured at 61.5 cm with bicoronal distance of 39.5 cm. He demonstrated improvement in head control and ability to sit. This patient demonstrates a new technique for reduction cranioplasty involving the use of reverse distraction to facilitate gradual and controlled reduction prior to extensive cranial vault remodeling. The challenges associated with managing hydrocephalus in cranial vault reduction patients are also addressed.

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.

Mucor Osteomyelitis of the Distal Radius Necessitating Ulnocarpal Fusion.

This is a case report of a 60-year-old female who developed distal radius osteomyelitis secondary to Mucor infection from likely hematogenous spread that was managed with ulnocarpal wrist fusion. Following serial debridement and systemic antifungal therapy, ulnocarpal wrist fusion offered functional limb salvage rather than amputation in this patient with significant operative risk and comorbidities.

Predictors of Digit Survival following Replantation: Quantitative Review and Meta-Analysis.

Introduction Microsurgical replantation following digital amputation has variable success rates. Sociodemographic factors and surgery-related variables have been shown to influence survival rates; however, few studies have evaluated these data systematically across a combined dataset. Therefore, the objective of this study was to analyze the current literature to identify the predictors of replant survival. Materials and Methods A literature review was performed using the PubMed/Medline database focused on complete digit amputation/replantation studies. Studies were evaluated for patient and surgery-related variables and their respective effects on survival. Statistical analysis was conducted to identify predictors of survival and derive pooled estimates from the combined dataset. Results Thirty-two studies representing more than 6,000 digit amputation/replantation cases met inclusion/exclusion criteria. Statistical analysis revealed the number of venous anastomosis (0 vs. 1 vs. 2), the number of arterial anastomosis (0 vs. 1 vs. 2), and the mechanism of injury (sharp cut versus blunt cut versus avulsion versus crush) to influence replant survival ( p < 0.05). The authors failed to find a significant association between survival and the following variables: age, sex, zone of injury, digit number, tobacco use, ischemia time, method of preservation, and use of vein graft. Conclusion Patient- and surgery-related variables affect digit survival following replantation. The etiology of injury can help risk-stratify patients and assist in an informed decision making process, whereas surgery-related factors can guide surgeon practice to improve clinical outcomes following replantation.

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.