Modifications to the WHISTLE Flap Procedure for Correction of Secondary Cleft Lip Deformities.

Background: The whistle deformity, a deficiency of tissue in the central upper lip, is a consequential outcome of primary cleft lip repair. Among multiple described reconstructive options, the wide-hinged island swing transposition labial enhancement (WHISTLE) flap procedure by Grewal et al has been shown to be a reliable technique that restores the continuity of the orbicularis oris muscle and creates a more natural appearing tubercle and central lip element. This article aims to refine the WHISTLE flap procedure with the addition of tailored mucomuscular flaps and an upper lip-lengthening mucosal Z-plasty. Methods: A total of 11 patients with a whistle deformity were examined. All underwent the WHISTLE flap procedure with tailored mucomuscular flaps and a mucosal Z-plasty. The patients were followed for a period of from 6 to 51.5 months. Pre- and postoperative photographs were used for objective outcome comparison. Results: From 2018 to 2023, a total of 11 patients with a whistle deformity were included in the final cohort, comprised four bilateral and seven unilateral cleft lips. Ten cases (90.1%) resulted in satisfactory postoperative cosmetic appearance and did not require further interventions. A single patient with a significant preoperative discrepancy between the upper and lower lip volumes had a postoperative residual deformity. None of the patients demonstrated any functional deficits associated with the procedure. Conclusions: The WHISTLE flap procedure with the proposed individual tailoring of the mucomuscular flap and Z-plasty for mucosal scar lengthening has excellent cosmetic outcomes and can be considered as the primary treatment modality in most patients with a whistle deformity.

Management of Craniosynostosis in Lethal Perinatal Hypophosphatasia.

Although perinatal lethal hypophosphatasia (HPP) was once a disease with a universally poor prognosis, it has now become a rare but treatable condition with the advent of enzyme replacement therapy with asfotase alfa. As a result, a greater population of patients with perinatal HPP are presenting with abnormal head shape and craniosynostosis. The authors present here 3 cases of perinatal lethal HPP, 1 treated with traditional open cranial vault remodeling and 2 treated utilizing distraction osteogenesis techniques. All patients demonstrated outcomes comparable to those previously reported with traditional observation or open cranial vault repair. Thorough consideration and discussion between the surgical team and patient's family is needed to determine a treatment plan that best addresses the goals of patient and family in light of recent advances in medical treatment in this rare patient population in which surgical interventions were previously nearly impossible. This article further supports the safety and efficacy of surgical intervention and explores the utility of distraction osteogenesis to address craniosynostosis in this patient population.

Multidisciplinary Presurgical Education: Clinical Impact on Children With Orofacial Clefts Undergoing Maxillary Distraction via Rigid External Distraction.

OBJECTIVE: Treatment of severe maxillary hypoplasia is commonly addressed via distraction osteogenesis with a rigid external device (RED). While effective, this method can be socially stigmatizing in an already vulnerable patient population. To prepare children and their caregivers for life with a RED and decrease peri-operative anxiety, we instituted a multidisciplinary pre-surgical education session (MPES). This educational team involves our cleft care coordinator, child life specialist, orthodontist and plastic surgeon 2 weeks prior to surgery. We reviewed the impact of this intervention by examining clinical outcomes before and after its implementation. DESIGN: From February 2017 to February 2020, a retrospective chart review was performed to include patients with orofacial clefts and maxillary hypoplasia who underwent maxillary distraction osteogenesis with RED at our center before (28 patients) and after (29 patients) the implementation of MPES. RESULTS: MPES was associated with a significantly shorter length of stay compared to controls who did not receive MPES (3.6 vs 3.1 days, p < 0.03) and significantly decreased usage of inpatient narcotic pain medication compared to controls (16.8 morphine equivalents vs 31.8 morphine equivalents, p < 0.02). Our intervention also demonstrated a trend towards decrease in minor complications but did not achieve statistical significance p = 0.32). CONCLUSIONS: Multidisciplinary presurgical education is a beneficial adjunct in the care of patients with orofacial clefts and maxillary hypoplasia undergoing maxillary advancement with a RED.

Implementation of Telehealth in a Pediatric Plastic Surgery Clinic: A Single Center's Response to COVID-19.

BACKGROUND: With limitation of in-person clinical visits due to the COVID-19 pandemic, telehealth has emerged as an important platform to deliver healthcare. Satisfaction with telehealth has been examined previously in the adult plastic surgery population. However, studies examining its use and satisfaction in the pediatric plastic surgery population are limited. In this study, we explore patient/caregiver satisfaction with the rapid institution of telehealth in our pediatric plastic surgery clinic and factors affecting satisfaction scores. METHODS: From March 2020 to February 2021, families of pediatric patients seen via video telehealth in the pediatric plastic surgery clinic at our center were invited to participate in a posttelehealth session survey. Telehealth visits encompassed both preliminary consultations as well as follow-up visits. After appropriate consent was obtained, patients' families completed a posttelehealth survey conveying demographics and satisfaction with telehealth via a 5-point Likert scale. RESULTS: Seventy eight of 131 eligible families elected to participate. Visit types included the following: initial consultations (23.1%), preoperative visits (10.3%), postoperative visits (29.5%), and follow-up visits (37.1%). A total of 80.3% of the respondents were overall satisfied with their telehealth experience. A total of 85.3% of the respondents agreed that telehealth made it easier for them to see their doctor, and 96.7% of the respondents felt using telehealth saved them time in traveling to the clinic. A total of 89.5% of the respondents felt it important for the state of Utah to have a telehealth program available to pediatric patients. CONCLUSIONS: Video telehealth is a useful adjunct in the care of pediatric plastic surgery patients with high caregiver satisfaction.

Simultaneous Closure of the Cleft Alveolus and Hard Palate with Concomitant Bone Grafting.

Cleft repair has been in constant evolution since its inception. Conventional repair of the cleft hard palate involves closure of nasal and oral mucosa without bony reconstitution. In many instances, this approach is adequate, but, particularly in complete clefts, the lack of bony support can lead to collapse of the maxillary arch, dental crowding, and posterior cross-bite. To address these shortcomings, our institution performs a two-staged palatoplasty with concomitant bone grafting of the alveolus and hard palate in the second stage. A retrospective review of children who underwent a two-staged palatoplasty at our institution was performed. These patients' records and images were reviewed for complications and changes in maxillary morphology. Fourteen patients with complete clefts had a two-staged palatoplasty with bone grafting in the second stage. The mean age at surgery was 37.5 months, and the mean follow-up was 16 months. One patient had resorption of the alveolar bone graft requiring additional bone grafting. The remaining patients were without complications and had good consolidation of the bone graft on follow-up imaging. Our early results support that there is a low complication rate (7% regrafting) in those patients who underwent bone grafting at the time of cleft palate repair with early evidence of bony consolidation on imaging and clinical examination. Wide exposure during the repair allows complete grafting of the maxillary bony deficit, which is not possible with traditional alveolar cleft repair and may alleviate the shortcoming of soft-tissue closure only. Future study is necessary to determine long-term outcomes.

Management of Tessier Type 3 Cleft With a Novel Reverse Palatal Expander.

ABSTRACT: The purpose of this clinical report is to present the novel management of a type Tessier 3 cleft which was treated using a palatal expander in reverse fashion to reapproximate the craniofacial skeleton allowing for closure of the palate and soft tissue of the cleft. Reapproximation of the bony component of the cleft was achieved without osteotomies and allowed for easier and earlier realignment of the bony and soft tissue components of the cleft. To our knowledge, this is the first use of reverse palatal expansion in the treatment of type 3 Tessier cleft. Reverse palatal expansion made management of this cleft more straightforward and should be considered as a useful adjunct in the management of wide facial clefts.

Alveolar Bone Graft Stabilization with Custom Maxillary Splints.

For secondary alveolar bone grafting in cleft patients, the success of bone graft take is dependent upon creating an ideal environment for both bony and soft tissue healing. This is particularly challenging in patients with existing fistulas, wide clefts, and bilateral alveolar clefts, where large soft tissue mobilization is required to get a tensionless repair, and micro-motion around the bone graft is significantly higher. Herein we describe our method for manufacture and placement of a custom postoperative maxillary splint following secondary alveolar bone grafting. Our splint encompasses the palate and alveolus to stabilize the maxillary arch and protect the incision lines during healing. We find our splint to be a useful adjunct to facilitate postoperative healing following secondary alveolar bone grafting.

Layered Closure of Lumbosacral Myelomeningocele Defects with Bilateral Paraspinous Muscle and Composite Fasciocutaneous Flaps.

Robust, reliable, and reproducible closure of lumbosacral myelomeningocele defects remains a challenge. In infants with lumbosacral myelomeningocele defects, multiple methods of soft tissue coverage have been described. These include various cutaneous, fascial, and muscle flaps and grafts. This is done with relative ease when ample soft tissue is present but becomes extremely difficult for large and distally located defects. We present here our closure technique of lumbosacral myelomeningocele defects in newborns, with associated short- and medium-term outcomes. We demonstrate the anatomy of this technique with fresh cadaver dissection and present a review of demographic and outcome data of 12 consecutive patients treated with this method from June 2014 to August 2019. No major intra- or postoperative complications have been encountered, with a mean follow-up of 22.2 months and median follow up of 18 months. After the neurosurgical repair of lumbosacral myelomeningocele, bilateral composite fascial flaps composed of thoracolumbar and gluteus maximus fascia are elevated in continuity. The paraspinous muscle flaps are then elevated, disinserted distally, and medialized to provide complete muscular coverage of the dural repair. The bilateral composite fascial flaps are medialized and closed over the deep paraspinous muscle flap repair. Two patients experienced areas of small, superficial skin necrosis, one of which healed by secondary intention and the other by debridement and full-thickness skin grafting. Use of bilateral paraspinous muscle flaps and bilateral composite fascial flaps composed of thoracolumbar and gluteus maximus fascia provides robust coverage of lumbosacral defects following myelomeningocele repair in infants.

Median Arcuate Ligament Syndrome-Review of This Rare Disease.

IMPORTANCE: Median arcuate ligament (MAL) syndrome is a rare disease resulting from compression of the celiac axis by fibrous attachments of the diaphragmatic crura, the median arcuate ligament. Diagnostic workup and therapeutic intervention can be challenging. OBJECTIVE: To review the literature to define an algorithm for accurate diagnosis and successful treatment for patients with MAL syndrome. EVIDENCE REVIEW: A search of PubMed (1995-September 28, 2015) was conducted, using the key terms median arcuate ligament syndrome and celiac artery compression syndrome. FINDINGS: Typically a diagnosis of exclusion, MAL syndrome involves a vague constellation of symptoms including epigastric pain, postprandial pain, nausea, vomiting, and weight loss. Extrinsic compression of the vasculature and surrounding neural ganglion has been implicated as the cause of these symptoms. Multiple imaging techniques can be used to demonstrate celiac artery compression by the MAL including mesenteric duplex ultrasonography, computed tomography angiography, magnetic resonance angiography, gastric tonometry, and mesenteric arteriography. Surgical intervention involves open, laparoscopic, or robotic ligament release; celiac ganglionectomy; and celiac artery revascularization. There remains a limited role for angioplasty because this intervention does not address the underlying extrinsic compression resulting in symptoms, although angioplasty with stenting may be used in recalcitrant cases. CONCLUSIONS AND RELEVANCE: Median arcuate ligament syndrome is rare, and as a diagnosis of exclusion, diagnosis and treatment paradigms can be unclear. Based on previously published studies, symptom relief can be achieved with a variety of interventions including celiac ganglionectomy as well as open, laparoscopic, or robotic intervention.