An evaluation of the customized nasal clip protocol for bilateral cleft lip and palate presurgical infant orthopaedics.

OBJECTIVES: Nasoalveolar moulding (NAM) has resulted in profound outcomes in the treatment of bilateral cleft lip and palate patients, including non-surgical columellar lengthening and nasal moulding. We examine an innovative alternative that is less invasive, yet provides similar results. In this study, we describe a novel approach using the Customized Nasal Clip Protocol (CNCP™) and compare the treatment outcomes of a small cohort of infants with bilateral cleft lip ± palate with published results of the Grayson nasoalveolar moulding protocol. MATERIALS AND METHODS: A cohort of six bilateral-cleft-affected patients was evaluated for this study. Standardized frontal and worm's eye view photographs were obtained, and clinical measurements were utilized to garner columellar length measurements and nostril height comparisons. The initial and post-surgical results were statistically compared with a student's t-test (p < .05). Inclusion and exclusion criteria were applied to the cohort, which will be described. RESULTS: The resulting columellar length and nostril height increases of the CNCP™ group were comparable to a published cohort of subjects that have undergone nasoalveolar moulding. The nasal changes were found to be significant with a p-value <.01. The CNCP™ cohort also had fewer clinic visits, no complications that led to complete pauses of active treatment, and the benefit of receiving comprehensive treatment that was initiated at their first clinical presentation, in comparison to traditional NAM patients. CONCLUSION: The increase of columellar length and nostril height that resulted from utilizing the CNCP™ in bilateral cleft patients met the treatment goals of presurgical infant orthopaedics, with results on par with published results of NAM. These results, paired with the reduction in patient, family, and provider burden, further support the continued use and development of the CNCP™ for appropriate patient populations.

Pleomorphic Adenoma of the Palate: A Rare Case in an Adolescent With Cleft lip and Palate.

Benign salivary gland tumors are rarely found in children and adolescents compared with adults. Pleomorphic adenomas (PAs), the most common benign salivary gland tumor, account for only 1% of all head and neck lesions and fewer than 5% of all salivary gland tumors in individuals under the age of 16 years. The data on palatal PA in the first 2 decades of life is confined to published case reports and case series. To date, there has never been a report of palatal PA in a patient with cleft lip and palate. Here we describe an adolescent female with bilateral cleft lip and palate with PA of the hard and soft palate who underwent wide local excision and reconstruction with a buccal fat pad and buccal myo-mucosal flap.

Malignant Granular Cell Tumor of the Lower Extremity in an Adolescent Male.

Granular cell tumors (GCTs) are extremely rare soft tissue tumors, with only 2% of tumors being malignant. Malignant GCTs are more often seen in women between the ages 40 and 60. There has been no case reported of a malignant GCT in a pediatric patient. We present a case of a 14-year-old male who presented with a large mass in his left lower extremity. After being biopsied, the mass was diagnosed as a malignant GCT. The tumor was completely excised with wide margins and close follow-up with the patient continued.

An Alternative Internal Le Fort I Distractor: Early Results With a New Trans-Nasal Device.

PURPOSE: To report the early experience using a new internal trans-nasal Le Fort I distractor in patients with cleft lip and palate. METHODS: Patients with cleft lip and palate and severe maxillary deficiency, who were treated with the trans-nasal Le Fort I distractor, were retrospectively reviewed. Cephalometric images were evaluated preoperatively and at least 6 months postoperatively. Speech outcomes were measured before and at least 6 months after surgery. Patient experience with the device was documented and complications were recorded. RESULTS: Five male patients with bilateral cleft lip and palate (ages 11-19) underwent the maximum advancement allowed by the device (25 mm). Follow-up averaged 2 years. Average SNA changed from 75.5°preoperatively to 84.6°postoperatively. Average ANB angle changed from -2.8° to 7.4°, or a tendency to Class 2 overcorrection. There was an overall increase in upper anterior facial height by 7.5 mm. All patients achieved acceptable postoperative occlusions. Two patients with borderline velopharyngeal function preoperatively developed velopharyngeal insufficiency postoperatively that did not resolve 6 months postoperatively, necessitating further surgery. Families reported ease of turning with minimal discomfort reported by patients. All patients maintained normal mouth opening during and after the distraction phase. Two of the patients developed localized pin site infections after the distraction phase that were treated successfully with oral antibiotics. CONCLUSION: The trans-nasal Le Fort I distractor can be an effective device to advance the deficient maxilla and is well tolerated by patients.

You "Cantu": Multidisciplinary Collaboration Resulting in Successful Orthognathic Surgery.

Cantú syndrome (CS) is a rare autosomal dominant disorder caused by a heterozygous pathogenic variant in the ABCC9 or KCNJ8 gene. The disorder is characterized by congenital generalized hypertrichosis, coarse acromegaloid facial features (broad nasal bridge, epicanthal folds, wide mouth, macroglossia), skeletal abnormalities (calvarial thickening, metaphyseal flares, coxa valga, scoliosis), tortuous vasculature (meningeal arteriovenous malformations), and cardiac abnormalities (patent ductus arteriosus, pericardial effusion). Despite the constellation of craniofacial features, there are currently no documented cases of a patient with CS having orthognathic surgery. The purpose of this report is to highlight the multidisciplinary collaboration, including establishment of a genetic diagnosis, cardiac management, and orthodontic therapy, in performing successful orthognathic surgery in a patient with CS.

A novel nonsense substitution identified in the AMIGO2 gene in an Occulo-Auriculo-Vertebral spectrum patient.

OBJECTIVE: Craniofacial microsmia is the second most common congenital disorder with mostly unilateral defects of ear, temporomandibular joint, mandible, and muscles of facial expression and mastication. The objective of this study was to identify, if there were any, de novo germline or somatic variants in a patient with Occulo-Auriculo-Vertebral Spectrum (OAVS) using whole-exome sequencing. SETTINGS AND SAMPLE POPULATION: Trio/Family-based study of an OAVS proband. MATERIALS AND METHODS: Children's Mercy Hospital Institutional Review Board approved this study and a request-to-rely was procured from the University of Missouri Kansas City IRB. Informed assent/consent was obtained for all family members prior to any research activities. The peripheral blood/affected side tissues from corrective surgery of the proband and peripheral blood samples from unaffected parents were collected. The isolated genomic DNA were enriched for exomes and sequenced on an Illlumina HiSeq 2500 instrument yielding paired-end 125 nucleotide reads (84X coverage). Gapped alignment to reference sequences (GRCh37.p5) was performed with BWA and the GATK and analysis completed using custom-developed software. RESULTS: Analyses revealed that the proband carried a de novo germ line nonsense substitution (c.901C>T) in AMIGO2 gene, and missense substitutions in ZCCHC14 (c.1198C>T), and in SZT2 genes (c.2951C>T). CONCLUSIONS: The nonsense substitution in AMIGO2 gene introduces a premature stop codon possibly rendering the gene non-functional via nonsense-mediated pathway decay-therefore considered a stronger candidate. Further functional studies are required to confirm whether loss-of-function variants in AMIGO2 can cause OAVS.

Reverse Distraction for Treatment of Hydrocephalic Macrocephaly in Late Childhood.

Macrocephaly diminishes quality of life for children whose head size inhibits independent mobility and appropriate interaction with caregivers. Cranial reduction is a method of addressing these issues, historically with a high morbidity due most commonly to bleeding and shunt complications. The authors present a 9-year-old girl with holoprosencephaly and severe macrocephaly from progressive hydrocephalus who underwent cranial reduction via reverse distraction osteogenesis, a method to slowly reduce the skull volume. The patient underwent circumferential occipital temporoparietal frontal craniotomy with placement of 4 cranial distractors, followed approximately 1 month later by removal of the distractors and cranioplasty with resorbable fixation devices. The patient demonstrated significant postoperative improvement in head control and interaction in school activities. This is the oldest patient with macrocephaly treated with reverse distraction in the literature to date. The slow contraction of the cranial vault with limited bony surgery at the time of initial reduction provides an additional safety margin, and should be considered in older children presenting with profound macrocephaly.

Orthognathic Correction in Prader-Willi Syndrome: Occlusion and Sleep Restored.

Children with Prader-Willi Syndrome (PWS) may present with a malocclusion and have a high propensity of developing obstructive sleep apnea (OSA). Obstructive sleep apnea is associated with short- and long-term adverse effects that negatively impact children with PWS. A case of a 15-year-old male with PWS, OSA, and a debilitating malocclusion is presented who underwent a combination of Le Fort 1 osteotomy, genioplasty, and tongue reduction to successfully treat his OSA and malocclusion. In select cases, orthognathic correction and other surgical therapies should be considered in patients with PWS.

Early Surgical Correction of the Nasal Deformity in Laurin-Sandrow Syndrome.

Laurin-Sandrow syndrome (LSS) is a rare autosomal disorder characterized by polysyndactyly of the hands and feet in a mirror fashion, absence of the radius and tibia with duplicated ulna and fibula, and nasal anomalies. Nasal defects are varied, and range from hypoplastic nasal skeleton to redundant nasal tissue, along with abnormalities of nasal subunits. Only 14 patients of LSS have been described in the literature. The authors present a unique case of a newborn with LSS and anterior nasal stenosis, resulting in respiratory failure. Early surgical intervention to relieve the bony and soft tissue overgrowth of the anterior nasal vault was required to allow for successful extubation.

A Postsurgical Obturator After Cleft Lip Repair in Patients With Holoprosencephaly.

Several factors affect healing of lip repair in children with complete cleft lip and palate in the immediate postoperative period. However, children with holoprosencephaly present a unique challenge. Because of their wide midline clefts and premaxillary agenesis they have protrusive positioning of their tongue, which can adversely affect the surgical result. In these cases we have found a postsurgical obturator made with hard-setting acrylic to be especially useful. Such an appliance may be used for the initial healing period (1-2 weeks). Two cases are presented here where such a device was used successfully.

Multifocal lipoblastoma of the face.

Lipoblastoma is a rare benign neoplasm found exclusively in the pediatric population that can occur anywhere in the body, most commonly seen in the extremities but also found in the face. We report an 8-month-old female subject who presented with multifocal soft tissue masses of the face. The diagnosis of lipoblastoma was made in 2 separate locations after surgical resection. Subsequent surgery was performed at the cheek site in an attempt to remove further mass, resulting in discovery of 2 other discrete tumors found to be lipoblastomas. Although the literature reports recurrence rates ranging from 14% to 27%, to our knowledge, aside from a case of Proteus syndrome, there are no known reports of multiple lipoblastomas in the literature. Presentation of the case, review of pertinent literature, and consideration of congenital infiltrative lipomatosis of the face follow.

Comparing plastic surgery and otolaryngology surgical outcomes and cartilage graft preferences in pediatric rhinoplasty: A retrospective cohort study analyzing 1839 patients.

ABSTRACT: Rhinoplasty in children has raised concerns about its safety in the pediatric population. There is scarcity of evidence describing outcomes and surgical techniques performed in pediatric rhinoplasty. We analyzed post-operative complications and cartilage preferences between plastic surgeons and otolaryngologists.Data was collected through the Pediatric National Surgical Improvement Program from 2012 to 2017. Current Procedure Terminology codes were used for data extraction. Patients were grouped according to type of rhinoplasty procedures (primary, secondary, and cleft rhinoplasty). A comparison between plastic surgeons and otolaryngologists was made in each group in terms of postoperative complications. Additionally, a sub-group analysis based on cartilage graft preferences was performed.During the study period, a total of 1839 patients underwent rhinoplasty procedures; plastic surgeons performed 1438 (78.2%) cases and otolaryngologists performed 401 (21.8%) cases. After analyzing each group, no significant differences were noted in terms of wound dehiscence, surgical site infection, readmission, or reoperation. Subgroup analysis revealed that plastic surgeons prefer using rib and ear cartilage, while otolaryngologists prefer septal and ear cartilage.The analysis of 1839 pediatric patients undergoing three types of rhinoplasty procedures showed similar postoperative outcomes, but different cartilage graft utilization between plastic surgeons and otolaryngologists.

Posterior pharyngeal augmentation in the treatment of velopharyngeal insufficiency: a 40-year experience.

BACKGROUND: Velopharyngeal insufficiency (VPI) remains a common problem in the care of patients with cleft palate and other related conditions. It may be successfully corrected with augmentation of the posterior pharynx. The purpose of this study was to review the experience and results of posterior pharyngeal augmentation over a 40-year period at one cleft center in the United States. METHODS: All patients from 1968 to 2008 who underwent posterior pharyngeal augmentation for the treatment of VPI were reviewed retrospectively. Diagnosis, age at the time of operation, type of implant used, duration of follow-up, speech performance both pre- and postoperatively, and complications were analyzed. Speech performance preoperatively was assessed by video fluoroscopy and/or nasoendoscopy. Resonance was assessed by both the surgeon and speech pathologist using a 4 point grading scale. RESULTS: One hundred eleven patients underwent posterior pharyngeal augmentation. Thirteen patients required removal of the implant because of extrusion (n = 12) or postoperative sleep apnea (n = 1). Five patients with failed implants at the primary operation had successful replacement at a second operation, resulting in a retained implant success rate of 93%. Of the 103 patients who were successfully augmented, 15 were lost to follow-up, leaving 88 patients with full postoperative speech evaluations. Of the 88 patients evaluated, 64 exhibited normal or near normal speech (73%), 22 patients had mildly nasal speech (25%), and 2 patients (2%) were not improved. Highest extrusion rates were seen for smooth surface silicone and rolled gortex implants. CONCLUSION: Augmentation of the posterior pharyngeal wall is a safe and effective treatment for patients with VPI. Implants are well tolerated and speech is substantially improved.

The Graf/Biggs flap to increase upper pole projection in breast reductions with free nipple grafts.

BACKGROUND: Macromastia necessitating breast reduction with free nipple grafts often results in a breast shape that lacks upper pole projection. This study aimed to describe and review the experience with use of the Graf/Biggs flap to improve upper pole fullness in patients requiring breast reductions with free nipple grafts. METHODS: A retrospective review evaluated patients treated by the senior author who had breast reductions with free nipple grafts and simultaneous use of the Graf/Biggs flap. Reduction amount, aesthetic result, and complications including hematoma, seroma, infection, fat necrosis, wound breakdown, and nipple hypopigmentation were studied. RESULTS: A total of six patients, with an average follow-up period of 22 months, underwent breast reductions with free nipple grafts and simultaneous use of the Graf/Biggs flap. The average reduction amount was 2,583 g per side. There were no complications except for some early wound breakdowns at the lower T incision of the inframammary fold. These wounds resolved with local care. All the patients exhibited hypopigmentation of the grafted nipples and desirable breast shape, with excellent upper pole projection. All were universally happy with their result. CONCLUSION: The Graf/Biggs flap is a reliable technique for increasing upper pole projection in the patient requiring breast reduction with free nipple grafts.

Techniques for labia minora reduction: an algorithmic approach.

BACKGROUND: Aesthetic reduction of the labia minora has gained popularity, and a number of different techniques have been described. Each procedure has its own set of advantages and disadvantages. However, no algorithm has been defined for pairing the degree of deformity with the optimal surgical procedure. METHODS: Patients were stratified into one of four groups based on labial size. The surgeon chose one of three reduction techniques based on the degree of labial hypertrophy and the patient's aesthetic preferences for labial edge color and contour. Three reduction techniques were used including the edge excision technique, the inferior wedge resection technique, and deepithelialization reduction labioplasty. The success of aesthetic reduction was evaluated, as was symptomatic relief. RESULTS: The 12 procedures performed included five deepithelialization techniques, four edge excision techniques, and three inferior wedge resection techniques. As reported, 92% of the patients were "very satisfied" with their aesthetic and functional results. The complications were minimal, with three patients experiencing minor wound healing difficulties that resolved spontaneously. One patient who underwent the edge excision technique was "not satisfied" and complained of overreduction. CONCLUSION: The authors found all three techniques for labia minora reduction to be useful in different clinical situations. A novel algorithm is described for matching the optimal surgical technique for each patient based on her degree of hypertrophy and aesthetic goals.

Prosthetic temporomandibular joint reconstruction in a cohort of adolescent females with juvenile idiopathic arthritis.

BACKGROUND: Temporomandibular joint (TMJ) arthritis and involvement is commonly seen in Juvenile Idiopathic Arthritis (JIA). Therapy includes conservative measures, but also includes intraarticular corticosteroid injections (IASI) and systemic immunosuppressive therapy. Despite aggressive medical therapy, some patients develop arthritic changes and frank TMJ ankylosis that can result in persistent pain and limitation in range of motion (ROM). A surgical option is prosthetic TMJ replacement with concurrent correction of dentofacial deformities, which can be performed simultaneously. The objective of this study was to evaluate the outcomes of prosthetic TMJ replacement in a cohort of adolescent females with JIA and severe TMJ involvement. METHODS: This is a retrospective case series that took place at one tertiary care center. Patients with a diagnosis of JIA who also underwent alloplastic TMJ replacement were identified through electronic medical record system (EMR) and reviewed. Chart review included analysis of all documents in the EMR, including demographic data, JIA history, surgical complications, ROM of TMJ measured by maximal incisal opening in millimeters (mm) and TMJ pain scores (4-point Likert scale: none, mild, moderate, severe) obtained pre- and postoperatively. RESULTS: Five female patients, ages 15-17 year when TMJ replacement was performed, had nine total joints replaced with a post-operative follow-up period of 12-30 months. All patients had polyarticular, seronegative JIA and were treated with IASI and multiple immunosuppressive therapies without resolution of TMJ symptoms. One patient had bilateral TMJ ankylosis. Three of the five patients demonstrated significant dentofacial deformities, and all underwent simultaneous or staged orthognathic surgery. All patients had improvement in TMJ pain with most (80%) reporting no pain, and all had similar or improved ROM of their TMJ postoperatively. There was one delayed postoperative infection with Cutibacterium Acnes that presented 15 months after surgery and required removal and reimplantation of prosthesis. CONCLUSION: The sequelae of TMJ arthritis and involvement from JIA in the adolescent population can be difficult to treat. Current medical therapy can be successful, however, in select cases that develop chronic changes in the TMJ despite extensive medical therapy, early results show that prosthetic joint replacement maybe a reasonable surgical option. With prosthetic joint replacement pain levels were reduced and range of motion was maintained or improved for all patients.

Reconstruction of full-thickness calvarial defect: a role for artificial dermis.

The reconstruction of scalp defects presents a clinical challenge. Free tissue transfer has played an increasingly important role in the reconstruction of complex scalp defects. In many cases, patient medical comorbidities along with the length of the operative procedures incurs significant patient risk. Artificial dermis, used extensively in burn reconstruction, has emerged as a reconstructive option for the coverage of many complex posttraumatic and postoncologic defects; however, none have described its use for the reconstruction of full-thickness calvarial defects with exposed dura.We report a clinical case of an elderly, medically compromised patient with a full-thickness scalp defect, who underwent successful reconstruction with artificial dermis.The use of artificial dermis and subsequent skin grafting, as was performed in this case, provides a less invasive, less intensive, and satisfactory means of soft tissue reconstruction for full-thickness calvarial defects.

Implant-supported distraction osteogenesis: a technique to advance the deficient maxilla.

We describe a technique in which osseointegrated implants are used as anchors to support a custom-fabricated distraction device to advance the deficient anterior maxilla. The absolute rigidity of osseointegrated implants in bone provides stable anchorage for successful distraction.

Dental implant reconstruction in a patient with ectodermal dysplasia using multiple bone grafting techniques.

Ectodermal dysplasia is a syndrome in which 2 ectodermally derived structures fail to develop. Patients have a reduced number of teeth, and dental implant reconstruction is the preferred method of replacing teeth. We report the use of the tent pole technique in the severely resorbed mandible with sinus lifts and block grafting in the maxilla to restore the maxillofacial complex of a female patient with ectodermal dysplasia. The treatment sequence and techniques are discussed.