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.

A Comparison of Postoperative Outcomes between Unilateral and Bilateral Palatoplasty: Analysis of 2015-2020 Pediatric NSQIP Data.

OBJECTIVE: We sought to identify differences in 30-day medical and surgical complications in unilateral versus bilateral palatoplasty. DESIGN: The NSQIP-P 2015-2020 database was queried to identify cleft palate repairs using CPT codes. Cases were stratified as unilateral (Veau III) and bilateral (Veau IV) using ICD-9 and -10 codes. SETTING: A nationally representative random sample. PATIENTS/PARTICIPANTS: A total of 3791 cases were identified with 2608 undergoing unilateral repair and 1183 undergoing bilateral repair. MAIN OUTCOMES/MEASURES: The postoperative outcomes of interest included surgical complications (surgical site infections, wound dehiscence), medical complications (pneumonia, urinary tract infection, seizure, cardiac arrest, bleeding/transfusions, systemic sepsis, unplanned intubation), readmission, and reoperation. RESULTS: The bilateral cohort was older (696 days versus 619 days, P < .001) and had longer operative times (157.3 min versus 144.5 min, P < .001). The unilateral cohort had more comorbidities including developmental delay, structural CNS abnormalities, need for nutritional support, and bleeding disorders. The bilateral cohort had statistically significant higher occurrences of wound dehiscence (2.1% versus. 1.2%, P = .03) and readmission (3.2% versus 1.7%, P = .01). On multivariate analysis, bilateral cleft repair (OR: 1.83, CI: 1.176-2.840, P = .007) and ASA class 4 (OR: 13.1, CI 2.288- 62.586, P = .002) were associated with greater odds of readmission. CONCLUSION: Patients who underwent bilateral cleft repair had a higher proportion of 30-day postoperative complications and a two-fold increased odds of readmission. While palatoplasty is generally regarded as a safe procedure in the pediatric population, identifying factors related to an increased risk of early postoperative complications can help surgical teams better manage high-risk individuals.

Perioperative Comparison Between Open Cranial Vault Remodeling and Distraction Osteogenesis for Unilateral Lambdoid Craniosynostosis.

There are multiple treatment options for unilateral lambdoid craniosynostosis (ULS) including open posterior cranial vault remodeling (OCVR) and distraction osteogenesis (DO). There is a paucity of data comparing these techniques in the treatment of ULS. This study compared the perioperative characteristics of these interventions for patients with ULS. An IRB-approved chart review was performed from January 1999 to November 2018 at a single institution. Inclusion criteria included the diagnosis of ULS, treatment with either OCVR or DO using a posterior rotational flap technique, and a minimum 1-year follow-up. Seventeen patients met the inclusion criteria (12 OCVR and 5 DO). Patients in each cohort were found to have a similar distribution in sex, age at the time of surgery, synostosis laterality, weight, and length of follow-up. There was no significant difference in mean estimated blood loss/kg, surgical time, or transfusion requirements between cohorts. Distraction osteogenesis patients had a longer mean hospital length of stay (3.4 +/- 0.6 d versus 2.0 +/- 0.6 d, P = 0.0004). All patients were admitted to the surgical ward postoperatively. In the OCVR cohort, complications included 1 dural tear, 1 surgical site infection, and 2 reoperations. In the DO cohort, 1 patient had a distraction site infection, treated with antibiotics. There was no significant difference in estimated blood loss, volume of blood transfusion, or surgical time between OCVR and DO. Patients who underwent OCVR had a higher incidence of postoperative complications and the need for reoperation. This data provides insight into the perioperative differences between OCVR and DO in patients with ULS.

Psychosocial Burden of Pediatric and Adult Patients With Congenital Versus Traumatic Facial Differences: Assessment of Psychiatric Distress and Healthcare Utilization in the United States From 2004 to 2012.

BACKGROUND: Psychiatric distress and its effects on healthcare utilization in pediatric patients with congenital and traumatic facial differences remain poorly understood. This study analyzes the psychosocial burden along with mental health and reconstructive surgery services utilization of this patient population in comparison with adult patients with such facial differences. METHODS: The 2004-2012 Medical Expenditures Panel Survey was queried for all patients with facial differences. Socioeconomic variables, Patient Health Questionnaire 2 and Kessler 6 scores, responses from validated screening surveys, and utilization of mental health and reconstructive surgery (ie, plastic surgery and otolaryngology) services were compared between pediatric and adult patients with congenital and traumatic facial differences. RESULTS: Children ages 5 to 12 years were more likely to be affected by facial trauma, whereas adolescents aged 13 to 17 years were more affected by congenital facial conditions. Pediatric patients with congenital facial conditions had higher rates of medical care, education, and special therapy utilization ( P < 0.0001), although their facial trauma counterparts used mental health services more often ( P < 0.0001). In adults, more facial trauma patients reported poorer perceived mental health status ( P = 0.01). Among patients with any facial difference, distressed adult patients were less likely to see a reconstructive surgeon even when controlling for socioeconomic variables (0.55 [0.31-0.97], P = 0.04). CONCLUSIONS: In the pediatric population, psychosocial considerations should include both age and etiology of facial differences to best optimize care. Among adults with facial trauma, poor mental health may contribute to lower rates of surgical follow-up, highlighting a potential benefit for provision of mental health services earlier for these patient populations.

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.

Frontofacial Reconstruction Technique Modification With Preservation of Blood Supply to the Monobloc Segment.

ABSTRACT: Craniosynostosis syndromes, including Apert Syndrome, Pfeiffer Syndrome, and Crouzon Syndrome, share similar phenotypes, including bicoronal craniosynostosis, midface hypoplasia, hypertelorism, and exorbitism. The standard surgical treatment for these craniofacial abnormalities is monobloc osteotomy with distraction osteogenesis. Complications of this technique include the failure of osteogenesis or resorption of the frontal bone. The authors propose an alternative surgical technique with a frontal arch in continuity with the midface segment to ensure vascularization to anterior and posterior borders of distraction. A case report of an 8-year-old female patient with Apert Syndrome is reported using our technique. Our frontal arch monobloc distraction procedure preserves blood supply to a cranial component of the monobloc segment site that becomes the anterior portion of distraction rather than with the traditional devascularized frontal bone flap. This technique modification should improve osteogenesis outcomes by preventing resorption or failure of bone formation.

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.

Maxillary Distraction Osteogenesis in a Patient With Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is characterized by brittle bones, premature hearing loss, blue sclera, dental abnormalities, and short stature. Maxillofacial pathology is marked in many OI patients and includes a high incidence of class III malocclusion secondary to a retrusive maxilla relative to both the mandible and cranial base.Review of literature shows that most of the orthognathic surgeries performed in the setting of OI are double jaw surgeries, in the form of maxillary advancement and mandibular setback. However, severe maxillary hypoplasia is usually not correctable with single-stage maxillary advancement. Distraction osteogenesis (DO) is a technique that relies on the normal healing process that occurs between controlled, surgically osteotomized bone segments and it is a relatively widely used technique in modern management of craniofacial conditions.Distraction osteogenesis has been reported in only several patients with OI. There is only 1 previously documented case of maxillary distraction in the craniofacial literature. The authors present here the successful management of a patient with OI and severe class III malocclusion using LeFort I osteotomy and DO with an external rigid distractor.At 12 months follow-up, the patient had no complications and maintained stable maxillary position with normal occlusion, improvement of facial appearance, obstructive airway symptoms, speech, and chewing.This case serves to reinforce the safety and efficacy of DO in patients with OI. The authors did not significantly change our distraction protocol and did not have any complications, therefore the authors believe that DO should be the preferable treatment technique for severe malocclusion in OI patient population.

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.

Venous cystic adventitial disease of the common femoral vein.

Venous adventitial cystic disease is a rare condition with few reports previously described. We report the case of a 39-year-old female who presented with left lower extremity edema, acutely exacerbated by exercise. Imaging revealed a hypoechoic mass posterior to her left common femoral vein. Open surgical excision and evacuation was performed with resolution of symptoms within 24 hours. A review of the literature is discussed, along with presentation, diagnosis, treatment, and pathology of this intriguing condition.