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.

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.

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.

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.

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.

Journey to chew: a case of maxillary duplication and bony syngnathia.

Tessier no 3 cleft, bony syngnathia, and maxillary duplication are rare as independent anomalies and have never been reported together in a single case. Here we present a patient with congenital bony syngnathia, maxillary duplication, and a Tessier no. 3 nasal cleft. Other abnormalities included situs inversus, dextrocardia, coarctation of the aorta, left choanal stenosis, left coloboma, and hypertelorbitism. Given the unique presentation, we present our early surgical management to this complex problem.

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.

Defining the role for submental intubation.

Submental intubation, a less invasive alternative to tracheostomy, was first described for acute airway management of maxillofacial trauma patients, where nasoendotracheal intubation was contraindicated and oral intubation was not possible due to the desire to establish dental relationships perioperatively. While submental intubation is used most commonly in trauma patients, the range of indications for its use has broadened to include many orthognathic and skull base surgical procedures. Submental intubation is a safe, effective technique for many maxillofacial procedures, requiring the cooperation of both anesthesiologists and maxillofacial surgeons.

rhBMP-2 with a demineralized bone matrix scaffold versus autologous iliac crest bone graft for alveolar cleft reconstruction.

BACKGROUND: Secondary alveolar cleft reconstruction using autologous iliac crest bone graft is currently the standard treatment for alveolar clefts. Although effective, harvesting autologous bone may result in considerable donor-site morbidity, most commonly pain and the potential for long-term sensory disturbances. In an effort to decrease patient morbidity, a novel technique using recombinant human bone morphogenetic protein (rhBMP)-2 encased in a demineralized bone matrix scaffold was developed as an alternative to autografting for secondary alveolar cleft reconstruction. METHODS: A chart review was conducted for the 55 patients who underwent secondary alveolar cleft reconstruction over a 2-year period with a mean follow-up of 21 months. Of these, 36 patients received rhBMP-2/demineralized bone matrix scaffold (including 10 patients with previously failed repairs using iliac crest bone grafting) and 19 patients underwent iliac crest bone grafting. Postoperatively, bone stock was evaluated using occlusal radiographs rated according to the Bergland and Chelsea scales. RESULTS: Alveolar clefts repaired using rhBMP-2/demineralized bone matrix scaffold were 97.2 percent successful compared with 84.2 percent with iliac crest bone grafting. Radiographically, initial repairs with rhBMP-2/demineralized bone matrix scaffold were superior to iliac crest bone grafting according to both Bergland and Chelsea scales, and significantly more patients in the rhBMP-2/demineralized bone matrix scaffold group had coronal bridging. The postoperative intraoral infection rate following iliac crest bone grafting was significantly greater than for rhBMP-2/demineralized bone matrix scaffold. The cost of rhBMP-2/demineralized bone matrix scaffold products was offset by cost savings associated with a reduction in operative time averaging 102 minutes. CONCLUSIONS: rhBMP-2 encased in a demineralized bone matrix scaffold appears to be a viable alternative for secondary alveolar cleft repair. Patients are spared donor-site morbidity and achieve excellent results, decreasing operative time, and increasing operating room use. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

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.

Dentoalveolar infections.

Dentoalveolar infections represent a wide spectrum of conditions, from simple localized abscesses to deep neck space infections. The initial assessment of the patient with a dentoalveolar infection requires considerable clinical skill and experience, and determines the need for further airway management or emergent surgical therapy. Knowledge of head and neck fascial space anatomy is essential in diagnosing, understanding spread, and surgically managing these infections. Oral and maxillofacial surgeons must make use of their wide spectrum of clinical skill and knowledge to effectively evaluate and treat patients with dentoalveolar infections.