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.

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.

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.

Serial Posterior Cranial Vault Distraction for the Treatment of Complex Craniosynostosis.

ABSTRACT: Posterior cranial vault distraction is an important modality in the management of craniosynostosis. This surgical technique increases intracranial volume and improves cranial aesthetics. A single procedure is often inadequate in patients with complex multisuture craniosynostosis, as some will go on to develop intracranial hypertension despite the operation. Considering the negative effects of intracranial hypertension, some patients may warrant 2 planned distractions to prevent this scenario from ever occurring. Three patients with complex multiple-suture synostosis and severe intracranial volume restriction (occipital frontal head circumferences [OFCs] <1st percentile) were treated with 2 planned serial posterior cranial vault distractions at the institution between 2013 and 2018. Demographics, intraoperative data, and postoperative distraction data were collected. The OFC was recorded pre- and postdistraction, at 3- and 6-month follow-up appointments. Patients had a corrected average age of 18 weeks at the time of their initial procedure. There was an average of 38 weeks between the end of consolidation and the time for their 2nd distraction procedure. There was an average age of 79 weeks at the time of the 2nd procedure. All patients had a substantial increase in OFC and improvement of the posterior calvarium shape. The average increase in OFC was 5.2 cm after first distraction and 4.3 cm after 2nd distraction. No postoperative complications were encountered. Planned serial posterior cranial vault distraction is a safe and effective strategy for increasing intracranial volume, improving aesthetic appearance, and preventing the consequences of intracranial hypertension in patients with multisuture craniosynostosis and severe intracranial volume restriction.

Improvements in Cleft Lip Aesthetics with the Fisher Repair Compared to the Mohler Repair.

BACKGROUND: The extended Mohler rotation-advancement repair and the Fisher anatomic subunit repair are commonly used for the surgical correction of unilateral cleft lip. The rotation-advancement repair was the initial technique of choice by the senior surgeon. However, due to recurring suboptimal aesthetic results, the senior surgeon transitioned to the anatomic subunit repair. This study was performed to compare the outcomes of the rotation-advancement repair and the anatomic subunit repair. METHODS: A retrospective study of all consecutive patients undergoing unilateral cleft lip repair by the senior author between 2009 and 2016 was conducted. Demographic data, the presence of scar shortening/contraction, hypertrophy, widening, and revision rates were recorded. RESULTS: There were 68 patients identified for inclusion. Thirty-four patients had a rotation-advancement repair and 35 had an anatomic subunit repair. Twelve patients (36%) with the rotation-advancement repair and 1 patient (2.9%) with the subunit repair required anterior lip revision (P < 0.001). Conversely, 2 patients (6.1%) with the rotation-advancement repair and 13 patients (37.1%) with the subunit repair required minor debulking of excess red vermilion fullness (P < 0.005). CONCLUSIONS: Transitioning from the rotation-advancement repair to the anatomic subunit repair has resulted in improved lip aesthetics with decreased incidence of scar contracture, hypertrophy, and widening as evidenced by a decrease in the revision rate for these suboptimal scars. However, the rate of debulking procedures of the red vermilion did increase early in the adoption of the anatomic subunit repair, requiring minor modifications in the technique.

Congenital Amniotic Constriction Band of the Pelvis: First Reported Case and Proposed Classification Criterion.

Congenital amniotic band syndrome (ABS) is a complex disorder characterized by a spectrum of rare congenital anomalies, with constriction bands as their hallmark manifestation. Constriction bands about the thorax, abdomen, or pelvis are rare. There is no consensus in the classification criterion for constriction bands of the thorax, abdomen, or pelvis in the literature. We present the first true pelvic constriction band in a newborn female with ABS. This case adds valuable information to the literature on ABS. The authors propose a classification criterion to further classify constriction bands of the thorax, abdomen, and pelvis using accepted and reliable anatomical landmarks.

Anatomical Nasal Lining Flaps for Closure of the Nasal Floor in Unilateral and Bilateral Cleft Lip Repairs Reduce Fistulas at the Alveolus.

BACKGROUND: Techniques vary for addressing the nasal floor during cleft lip repair in patients with a cleft lip and palate. Sometimes, no closure is performed, leaving a symptomatic alveolar fistula until the time of alveolar bone grafting. Often, medial and lateral skin flaps are used, but these are often thin and unreliable. Anatomical nasal lining flaps are used to improve closure with robust, well-vascularized flaps that anatomically close the nasal floor. METHODS: A retrospective chart review was performed to identify patients with a unilateral or bilateral cleft lip and palate who underwent primary cleft lip repair with nasal lining flaps or with medial and lateral flaps. The primary outcome was presence of a symptomatic and/or visible oronasal fistula. RESULTS: Sixty-four patients were included. Thirty-seven underwent closure with nasal lining flaps, whereas 27 underwent closure using Millard medial and lateral flaps. The rate of symptomatic/visible fistulas after cleft palate repair was 19 percent (seven of 37) for patients with nasal lining flaps and 44 percent (12 of 27) for patients with medial and lateral flaps (p = 0.0509, Fisher's exact test). The alveolar fistula rate was 3 percent (one of 37) for patients with nasal lining flaps and 30 percent (eight of 27) for patients with medial and lateral flaps (p = 0.0032, Fisher's exact test). CONCLUSIONS: Nasal lining flaps at the time of cleft lip repair effectively close the anterior nasal floor in patients with a unilateral or bilateral cleft lip and palate. Decreasing the presence of alveolar fistulas after cleft palate repair improves the quality of life for patients with cleft deformities. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Osseointegrated Prosthetic Ear Reconstruction in Cases of Skin Malignancy: Technique, Outcomes, and Patient Satisfaction.

BACKGROUND: Ear reconstruction with osseointegrated prosthetic implants is a well-established method of reconstruction after resection of skin malignancies on the external ear. There is limited literature reporting technique, outcomes, and patient satisfaction. METHODS: We evaluated our outcomes over a 5-year period looking at osseointegrated prosthetic reconstruction after auriculectomy for external ear skin malignancies. We report demographics, disease characteristics, technique, and complications. The patients were surveyed looking at 6 domains: satisfaction, stability, comfort, ease of use, level of self-consciousness, and preoperative education. RESULTS: Of the 21 patients included in the study, 14 (67%) were treated for invasive melanoma (Breslow depth, >0.8mm), 4 (19%) for squamous cell carcinoma, 2 (10%) for basal cell carcinoma, and 1 (5%) for an atypical fibroxanthoma. Complications rates were low. There were no cases of infection, hematoma, or bleeding. In 2 patients (9.5%), 1 of the 3 implants failed to osseointegrate and was removed, but the prosthesis was able to be secured with the remaining 2 posts. There were 3 cases (14%) of delayed healing and 1 with excessive granulation tissue growth. Survey results showed high satisfaction in all measured domains. CONCLUSIONS: In cases of skin malignancy requiring total or subtotal auriculectomy, prosthetic ear reconstruction with osseointegrated implants is a good alternative to reconstruction with autologous tissue. Our experience demonstrates good outcomes and with low complication rates and high patient satisfaction.

Virtual Surgical Planning Decreases Operative Time for Isolated Single Suture and Multi-suture Craniosynostosis Repair.

BACKGROUND: Cranial vault reconstruction is a complex procedure due to the need for precise 3-dimensional outcomes. Traditionally, the process involves manual bending of calvarial bone and plates. With the advent of virtual surgical planning (VSP), this procedure can be streamlined. Despite the advantages documented in the literature, there have been no case-control studies comparing VSP to traditional open cranial vault reconstruction. METHODS: Data were retrospectively collected on patients who underwent craniosynostosis repair during a 7-year period. Information was collected on patient demographics, intraoperative and postoperative factors, and intraoperative surgical time. High-resolution computed tomography scans were used for preoperative planning with engineers when designing osteotomies, bone flaps, and final positioning guides. RESULTS: A total of 66 patients underwent open craniosynostosis reconstruction between 2010 and 2017. There were 35 control (non-VSP) and 28 VSP cases. No difference in age, gender ratios, or number of prior operations was found. Blood loss was similar between the 2 groups. The VSP group had more screws and an increased length of postoperative hospital stay. The length of the operation was shorter in the VSP group for single suture and for multiple suture operations. Operative time decreased as the attending surgeon increased familiarity with the technique. CONCLUSIONS: VSP is a valuable tool for craniosynostosis repair. We found VSP decreases surgical time and allows for improved preoperative planning. Although there have been studies on VSP, this is the first large case-control study to be performed on its use in cranial vault remodeling.

Breast Cancer Risk Reduction Decisions of the BRCA-Positive Patient: An Observational Study at a Single Institution.

BACKGROUND: BRCA1 and BRCA2 gene mutations carry with them a 50%-80% risk of developing breast cancer. The best choice for managing breast cancer risk in patients with a BRCA1/2 mutation is a highly personal decision. Options for risk management include surveillance with multiple modalities or prophylactic surgical intervention. The goal of this study was to gain a better understanding of contributing factors affecting the decision for managing breast cancer risk made by patients who are BRCA mutation positive and cancer free. METHODS: A retrospective chart review of patients, who tested positive for BRCA1/2 gene mutation between 2003 and 2013 without history of breast cancer, was performed. A logistic regression model was used to assess the association between preselected risk factors and the decision of the patient to undergo prophylactic mastectomy versus surveillance. RESULTS: Of the 106 patients who were cancer free and BRCA positive, seventy (66%) opted for surveillance, whereas 36 (34%) chose prophylactic mastectomy. Three preselected risk factors were found to significantly affect a patient's likelihood of choosing surgery: having a family member with breast cancer before the age of 50 (OR: 4.67 [95% CI: 1.86-11.68]), p = 0.001), cancer-related death of a relative younger than 50 years old (OR: 2.26 [95% CI: 0.92-5.55], p = 0.07), and prophylactic oophorectomy before planned mastectomy (OR: 3.72 [95% CI: 1.49-9.31], p = 0.005). Patient age less than 30 was found to be significantly associated with the decision to proceed with surveillance (OR: 0.2 [95% CI: 0.05-0.75], p = 0.02). CONCLUSION: Risk-reducing strategy is a very personal decision for the patient with positive BRCA mutation, and many factors go into choosing which options are best for each individual. We found that age of patient, relative with breast cancer or death of a relative before age 50, and prophylactic oophorectomy all play significant roles in which risk reduction strategy is chosen.

Early Posterior Vault Distraction Osteogenesis for the Treatment of Syndromic Craniosynostosis.

Posterior cranial vault distraction (PCVD) has become an important modality in the management of complex craniosynostosis to increase intracranial volume and improve the cranial vault appearance. This technique can safely be performed as early as 3 months of age for the initial management of patients with complex craniosynostosis. A retrospective review was performed of all the patients with syndromic, multiple-suture synostosis treated with PCVD at Primary Children's Hospital in Salt Lake City, Utah, between 2012 and 2014. During this time period, a total of nine children with an average corrected age of 21.4 weeks were treated. Demographics, intraoperative data, and postoperative distraction data were collected. Occipital frontal head circumferences (OFCs) were recorded pre- and postdistraction, and at 3- and 6-month follow-up examinations. All patients had a substantial increase in head circumference with subjective improvement of the posterior calvarium shape. The average increase in OFC from preoperative to postdistraction was 4.9 cm. Only two significant postoperative complications were encountered, one hardware infection and one cerebrospinal fluid leak. PCVD is an effective procedure in the management of complex craniosynostosis and can be safely initiated very early in life.

Neonatal Ear Molding: Timing and Technique.

The incidence of auricular deformities is believed to be ∼11.5 per 10,000 births, excluding children with microtia. Although not life-threatening, auricular deformities can cause undue distress for patients and their families. Although surgical procedures have traditionally been used to reconstruct congenital auricular deformities, ear molding has been gaining acceptance as an efficacious, noninvasive alternative for the treatment of newborns with ear deformations. We present the successful correction of bilateral Stahl's ear deformity in a newborn through a straightforward, nonsurgical method implemented on the first day of life. The aim of this report is to make pediatric practitioners aware of an effective and simple molding technique appropriate for correction of congenital auricular anomalies. In addition, it stresses the importance of very early initiation of ear cartilage molding for achieving the desired outcome.

Distraction Osteogenesis Technique for the Treatment of Nonsyndromic Sagittal Synostosis.

BACKGROUND: Historically, surgical treatment of children with a delayed presentation of cranial synostosis required complex cranial vault reconstruction. Recently, less invasive options for surgical correction, such as internal distraction osteogenesis, have been explored. In this study, we describe the successful management of delayed presentation of sagittal synostosis using distraction osteogenesis. METHODS: A bicoronal incision was made and 2 large rectangular osteotomies were performed bilaterally, involving the frontal, parietal, temporal and occipital bones. A 2 cm strut of bone over the sagittal sinus was preserved, creating bilateral free-floating bone segments. Two pairs of distractors were placed transversely, along the midline strut of bone, providing lateral distraction of these segments. This placement allowed maximum displacement at the apex of the cranial vault. Distraction was performed differentially at 1 mm per day anteriorly and 2 mm per day posteriorly, for a total of 17 days, allowing for a greater degree of expansion of the posterior vault. The consolidation phase lasted for 10 weeks. The distractors were removed via the same bicoronal incision and cranioplasty was performed to smooth prominent ridging at the margins of the distracted segments. RESULTS: The child's cranial index improved from 0.67 preoperatively to 0.76 postoperatively. The overall vault contour was smooth with no bony defects. There was a significant cosmetic improvement of the child's head shape. No major complications requiring reoperation or rehospitalization were encountered. CONCLUSION: The use of distraction osteogenesis to laterally expand the cranial vault is a useful alternative in the treatment of delayed presentation, nonsyndromic, sagittal synostosis.

Importance of Postoperative Hydration and Lower Extremity Elevation in Preventing Deep Venous Thrombosis in Full Abdominoplasty: A Report on 450 Consecutive Cases Over a 37-Year Period.

BACKGROUND: Abdominoplasty is a common plastic surgery procedure and has a higher rate of postoperative deep venous thrombosis (DVT) than any other such surgery. DVT prevention remains an important topic in the plastic surgery community. Many surgeons avoid DVT chemoprophylaxis, because they are concerned about the risk of postoperative bleeding. OBJECTIVE: This study was performed in order to evaluate postoperative hydration and lower extremity elevation as two alternative methods of aiding DVT prevention after abdominoplasty. METHODS: Preoperative, intraoperative, and postoperative measures were taken to monitor patient hydration via urine output and to ensure adequate hydration by providing intravenous fluids. In addition, measures were taken throughout these three phases to ensure adequate lower extremity elevation. RESULTS: In a group of 450 consecutive full abdominoplasties from 1976 to 2013, the overall rate of patient DVT at 30 days following surgery was 0.22%. CONCLUSIONS: Hydration and lower extremity elevation are innocuous and risk-free adjuncts that improve venous return from the legs and lessen the chance of postoperative DVT.

Pancraniosynostosis following endoscopic-assisted strip craniectomy for sagittal suture craniosynostosis in the setting of poor compliance with follow-up: a case report.

INTRODUCTION: There is limited craniofacial literature on the complications of helmet therapy and controversy regarding the effects of inadequate orthotic helmet therapy. The authors present a case of inadvertently prolonged orthotic helmet therapy after endoscopic strip craniectomy for isolated sagittal synostosis. CASE PRESENTATION: A two-month-old Caucasian baby underwent uncomplicated endoscopic-assisted strip craniectomy to treat synostosis of the sagittal suture and was fitted for an orthotic helmet two weeks postoperatively. He presented to the craniofacial clinic eight weeks postoperatively with occipital flattening and increased posterior vault height, so the helmet was refitted. During the next 18 months, the helmet was used inconsistently without follow-up. Upon re-presentation, the patient had developed pansynostosis, requiring a subsequent open total cranial vault reconstruction for correction for this secondary deformity. CONCLUSIONS: Although it remains unclear whether postoperative development of pansynostosis is the result of prolonged helmeting or the consequence of progressive synostotic disease, this report highlights the importance of parent education and judicious scheduled follow-up for the avoidance of potential helmet therapy complications.