Late Presenting Multi-Suture Craniosynostosis.

ABSTRACT: The authors provide the case of a 6-year-old male who presented late with multi-suture craniosynostosis and chronically elevated intracranial pressures (ICPs). He was surgically managed with frontal orbital advancement. This particular case illustrates the significant bleeding and unique bony pathology that can occur in patients with high ICP with concomitant venous collateralization. At 1-month follow-up, he demonstrated significant improvement with maintained expansion and no signs of elevated ICP despite delayed intervention. Frontal orbital advancement serves as an effective method for cranial vault expansion and correction of frontal deformities caused by craniosynostosis.

Family satisfaction with telemedicine follow-up after pediatric plastic surgery.

OBJECTIVE: One issue faced at institutions that serve a vast area is patients' ability to travel for perioperative care. Telemedicine is an innovative way of providing care while removing the inconvenience of travel or the hindrance of cost associated with travel. We initiated telemedicine as an option for certain postoperative encounters and assessed patient family satisfaction with this novel approach. METHODS: Our practice offers telemedicine visits to patients who have had simple surgical procedures, identified by a fixed list of CPT codes. Visits are scheduled 7 to 14 days after surgery. Families completed a satisfaction survey after their encounter. RESULTS: A pilot program was initiated from January 2019 to March 2020 using this method of postoperative follow-up. The initial response from families (N = 60) was extremely positive. CONCLUSION: We anticipate the option for telemedicine visits will make postoperative follow-ups more amendable to families, increase adherence rates, and increase access to care.

Multidisciplinary management of a previously unreported presentation of severe aplasia cutis congenita.

Aplasia cutis congenita (ACC) is characterized by the complete or partial absence of skin at birth, with 85% of cases of ACC involving the scalp vertex. The etiology of ACC is unclear and appears to be multifactorial. We present the case of a 3-month-old boy who presented with a diagnosis of non-scalp ACC affecting approximately 80% of his total body surface area at birth. This case adds to the literature due to the patient's survival beyond the first day of life and his unique and severe distribution of defects, which led to respiratory compromise and required multidisciplinary management.

Two-Stage Treatment of a Large Premaxillary Defect.

ABSTRACT: Bilateral cleft lip and palate (CLP) patients commonly require surgical management to treat maxillary hypoplasia following the primary repair. Rarer is the CLP patient who also presents with a missing premaxillary segment. Here the authors present the case of a 19-year-old female with a history of bilateral CLP who demonstrated significant maxillary hypoplasia in addition to a large premaxillary defect. To correct this deformity, LeFort I advancement with fibular reconstruction of the maxilla and dental implant placement was performed as a single surgical procedure. The authors discuss the advantages of undergoing this single-staged operation. By utilizing virtual surgical planning (VSP) and incorporating a multidisciplinary team within the operating room, the patient was able to successfully undergo maxillary reconstruction and dental implant placement in a single operation.

Cleft Care Packages and the Impact on Postoperative Care Following a Primary Cleft Lip Repair.

OBJECTIVE: To provide caregivers with all the resources needed to care for a surgical site following a primary cleft lip repair and evaluate its efficacy on postoperative care. SETTING/PARTICIPANTS: Caregivers of infants ages 3 to 6 months with a cleft lip and/or palate undergoing a primary repair at the Texas Children's Hospital. METHODS: Packages were given to caregivers at discharge following repair. Packages included instructions and supplies needed for surgical site care. At discharge an advanced practice provider obtained informed consent and a questionnaire that established baseline knowledge of surgical site care. Following the questionnaire, the advanced practice provider demonstrated how to care for the site using the package provided. Assessment of scar healing, nasal stent compliance, and ease of care was evaluated at postoperative follow up. RESULTS: Thirty-two families were enrolled in this study. Our data supports that caregivers who are provided resources to care for the site had increased comfort level, preparedness, and compliance rates following a primary cleft lip repair. Eighty-four percent of respondents strongly agreed that the package provided aided in preparedness for site care with 100% of respondents recommending the resources to future families undergoing a cleft lip repair. CONCLUSIONS: Caregivers feel comfortable and equipped with their ability to care for their child's repaired cleft lip when given the appropriate instructions and supplies. In addition, they would recommend the packages to future families following a repair. Empowering families to be proactive in postoperative care will potentially lead to better outcomes in cleft care.

Pediatric Ventriculoperitoneal Shunt Infection: The Role of Shunt Tract Debridement in Mycobacterium abscessus Eradication.

Ventricular shunt infections caused by nontuberculous mycobacteria are uncommon, and those caused by Mycobacterium abscessus are particularly rare. This mycobacterium is intrinsically resistant to first-line anti-tuberculous drugs and is considered the most pathogenic of the atypical, rapidly growing mycobacteria. Given the paucity of reported M. abscessus ventricular shunt infections, the appropriate surgical treatment for these cases, especially in the pediatric setting, has yet to be described. The authors present a 4-year-old male with history of intraventricular hemorrhage resulting in hydrocephalus who presented with an M. abscessus ventricular shunt infection that disseminated to the skin and soft tissue of the entire shunt tract. Despite aggressive antimicrobial therapy, several shunt exchanges, and numerous incisions and debridements of separate infected tract areas, the patient's clinical course was prolonged by multiple relapses and re-admissions. Only after opening and debriding the entire length of the infected tract, which measured 100 cm and extended from the scalp to the groin, and months of intrathecal antibiotics did CSF and tissue culture results become negative, and the entire tract was able to be closed. This article describes the management of the second-encountered pediatric M. abscessus shunt infection along with the management of the 4 previously reported cases. In addition, it highlights the vital role of early, aggressive surgical debridement to achieve infection eradication.

Foreign Body Reaction to Poly-D-L-Lactic Acid Resorbable Plates Used in Craniofacial Surgery.

Resorbable surgical materials are often used in the pediatric population to provide fixation in the growing skeleton. Although foreign body reactions to poly-D-L-lactic acid (PDLLA) plates and screws have been previously reported in other fields, to date PDLLA polymers have been well-tolerated in the setting of craniofacial surgery. The authors report a case of a previously healthy 4-month-old patient with Crouzon syndrome who underwent a frontal-orbital advancement with resorbable PDLLA plates and screws and subsequently experienced extensive foreign body reactions and wound healing complications.

Quantifying Free Fibula Flap Growth After Pediatric Mandibular Reconstruction.

INTRODUCTION: The free fibula flap (FFF) is a preferred option for adult mandibular reconstruction. Due to skeletal immaturity, its routine use in pediatric patients remains in question. Inconsistencies regarding the ability of the FFF to grow in concordance with the patients' natural growth currently exist in the literature. The purpose of this report is to quantify mandibular growth in a young patient undergoing partial hemi-mandibular reconstruction with a FFF utilizing advanced three-dimensional software. METHODS: A 2-year old underwent left hemi-mandibular reconstruction with a FFF following resection of a desmoid tumor. The condyle was preserved. Using 3D software, changes in mandibular growth and morphology were evaluated based on preoperative (2.1 years old) and postoperative (2.5 years and 5.2 years old) computed tomography imaging. RESULTS: Mandibular growth occurred throughout the mandible in both postoperative evaluations. Greatest growth was seen in the ramus height. Fibula growth was also seen when comparing measurements to the virtual surgical planning guide. A novel parts comparison analysis revealed the greatest growth potential occurred at the condyle. CONCLUSION: Providing an objective evaluation using 3D software, we have demonstrated growth throughout the reconstructed mandible, with greatest growth occurring at the preserved condyle. Despite scientific limitations of our study, the potential for mandibular growth appears to remain after FFF reconstruction, offering successful functional and cosmetic outcomes.

Midline Intranasal Dermoid Cyst With Intracranial Extension: Technical Details of a Multidisciplinary Approach to a Rare Condition.

Dermoid cysts are rare, benign neoplasms that develop when ectodermal tissue is ectopically included during neural tube closure. Only 0.7% to 1.8% of dermoid cysts occur intracranially, and these make up only 0.3% of all intracranial tumors. Definitive management of intracranial dermoid cysts is achieved with surgical excision, with the primary goal being removal of the lesion before rupture or infection occurs. The authors report the multidisciplinary management of a 6-month-old patient with a midline intranasal dermoid cyst with intracranial involvement successfully treated with surgical resection. The surgical approach included Neurosurgical access with a bifrontal approach to remove the intracranial portion, then Plastic Surgery accessed and removed the intranasal portion through a transcolumellar and infracartilaginous dissection. The entire cyst was successfully removed without rupture. The patient's postoperative course was without complication.Timely preoperative imaging and diagnosis were crucial to the success of this operation. When considering the possibility of a midline dermoid cyst with intracranial extension, it is essential to obtain proper imaging to distinguish between lesions on the differential diagnosis and to define extent of extension. Once diagnosed, surgical planning requires a multidisciplinary approach. Considerations for the optimal surgical approach to intracranial, intranasal dermoids include maximizing surgical exposure and ensuring an aesthetically pleasing reconstructive outcome.

Elevated Intracranial Pressure After Primary Surgical Correction of Sagittal Suture Craniosynostosis.

Study Design: A Case Report. Objective: Craniosynostosis is a craniofacial condition defined by premature fusion of at least one cranial suture. Resynostosis or secondary craniosynostosis of a previously patent adjacent suture following primary repair is a relatively common complication. While studies have assessed the rates of secondary craniosynostosis and subsequent reoperation, extremely limited data regarding reoperation techniques is available. Methods: We present a unique case of a pediatric patient with sagittal craniosynostosis who previously underwent a modified pi procedure and later developed resynostosis of the sagittal suture and secondary synostosis of the bicoronal sutures. We subsequently performed total cranial vault reconstruction with virtual surgical planning (VSP). Results: At his 31-month postoperative follow-up, he displayed normal head shape and denied any clinical signs of elevated intracranial pressures with a normal ophthalmological exam. Conclusions: The reoperation was successful with no significant postoperative complications noted. Performing geometric expansion with VSP to manage fusion of a previously open suture following primary treatment of sagittal synostosis should be considered within the armamentarium of operative options.

Sharing the Operating Room: A Descriptive Study of Combined and Collaborative Plastic Surgery Cases.

BACKGROUND: Plastic surgeons are often asked for intraoperative assistance by other surgical services. Improvement of a plastic surgery service has been shown to improve patient outcomes, decrease length of stay, and increase hospital revenue, yet plastic surgery's contribution to a hospital tends to be undervalued. The purpose of this study was to quantify the multidisciplinary role the plastic surgery service plays within a single, large pediatric institution. METHODS: Surgical cases involving both plastic surgery and at least one other team were identified from 2016 to 2019. Each case was categorized as either "combined" or "collaborative" based on whether the two teams worked separately on separate problems or together on the same problem, respectively. Data points collected included combined and collaborative cases, operating room hours, and total hospital charges billed. RESULTS: Of the 7564 total plastic surgery cases performed, multidisciplinary cases made up a minority of total cases (16 percent) but required 32 percent of the operating room hours and provided 49 percent of the total charges billed. Collaborative cases alone accounted for 20 percent of the service's operating room hours and 39 percent of total charges billed, while making up only 8 percent of total cases. CONCLUSIONS: Relative to cases where plastic surgery operates alone, combined and collaborative cases account for a disproportionately high number of operating room hours and provide a disproportionately high amount of charges billed.

"It's a Living Experience": Bereavement by Suicide in Later Life.

Bereavement by suicide for people in later life is significantly under-researched. Research on ageing and suicide has yet to address the experiences of those bereaved by suicide and how such a devastating loss affects the ageing experience. Objectives: We explored the substantive issues involved in bereavement by suicide and its impact on later life. Methods: This was a co-produced qualitative study. Peer researchers with lived experience conducted in-depth interviews with twenty-four people aged 60-92 years. A phenomenological approach informed the data analysis. Main Findings: Themes described included (1) moral injury and trauma; (2) the rippling effect on wider family and networks; (3) transitions and adaptations of bereaved people and how their 'living experience' impacted on ageing. Conclusions: It is important to understand how individual experiences of suicide intersect with ageing and the significance of targeted assessment and intervention for those bereaved by suicide in ageing policies and support.

Beta-Catenin Mutation with Complex Chromosomal Changes in Desmoid Tumor of the Scalp: A Case Report.

Gain-of-function mutations in the beta-catenin gene ( CTNNB1 ) drive genomic instability within different cancers. However, it is unclear whether alterations in beta-catenin signaling can still lead to chromosomal rearrangements in neoplasms without metastatic potential. Here, we report a unique case, whereby a desmoid tumor of the scalp contains a missense mutation in CTNNB1 . This mutation is located at the T41 phosphorylation site-previously reported to be necessary for proper beta-catenin degradation. Online database analysis then revealed that our mutation is likely causative of many different cancers and also absent in the healthy public. Karyotyping of the desmoid tumor cells then showed complex chromosomal changes in 16 out of 20 cells examined. To treat this patient, we surgically removed both the neoplasm and underlying calvarium and then successfully reconstructed the skull and scalp. Taken together, our data suggest that increased beta-catenin signaling can lead to genomic instability in the absence of metastatic potential.