Spectrum of Disease in Hospitalized Newborns with Congenital Micrognathia: A Cohort of 3,236 Infants at North American Tertiary-Care Intensive Care Units.

OBJECTIVE: To describe the spectrum of disease and burden of care in infants with congenital micrognathia from a multicenter cohort hospitalized at tertiary care centers. STUDY DESIGN: The Children's Hospitals Neonatal Database was queried from 2010 through 2020 for infants diagnosed with micrognathia. Demographics, presence of genetic syndromes, and cleft status were summarized. Outcomes included death, length of hospitalization, neonatal surgery, and feeding and respiratory support at discharge. RESULTS: Analysis included 3,236 infants with congenital micrognathia. Cleft palate was identified in 1266 (39.1%). A genetic syndrome associated with micrognathia was diagnosed during the neonatal hospitalization in 256 (7.9%). Median (IQR) length of hospitalization was 35 (16, 63) days. Death during the hospitalization (n = 228, 6.8%) was associated with absence of cleft palate (4.4%, P < .001) and maternal Black race (11.6%, P < .001). During the neonatal hospitalization, 1289 (39.7%) underwent surgery to correct airway obstruction and 1059 (32.7%) underwent gastrostomy tube placement. At the time of discharge, 1035 (40.3%) were exclusively feeding orally. There was significant variability between centers related to length of stay and presence of a feeding tube at discharge (P < .001 for both). CONCLUSIONS: Infants hospitalized with congenital micrognathia have a significant burden of disease, commonly receive surgical intervention, and most often require tube feedings at hospital discharge. We identified disparities based on race and among centers. Development of evidence-based guidelines could improve neonatal care.

Cadaveric Bone Allografting for Severe Mandibular Hypoplasia With Alleviation of Upper Airway Obstruction in Goldenhar Syndrome.

ABSTRACT: Severe congenital mandibular hypoplasia may cause significant upper airway obstruction, often necessitating immediate tracheostomy. Recent surgical advances have demonstrated early intervention with mandibular distraction osteogenesis over tracheostomy may yield desirable aesthetic and functional outcomes. Here we present a case of bilateral mandibular hypoplasia, severe on the right and mild on the left, secondary to Goldenhar syndrome yielding significant tongue-based upper airway obstruction that was surgically corrected with mandibular bone allograft reconstruction of the right condyle, ramus, and posterior half of the mandibular body. Postoperative polysomnography revealed significantly improved obstructive apnea and computed tomography demonstrated adequate placement of the bone allograft to overcorrect the affected hemi-mandible. To the authors' knowledge, this is the first report of immediate mandibular allografting alleviating tongue-based upper airway obstruction secondary to mandibular hypoplasia in an infant. Mandibular bone allografting may be a favorable alternative to immediate tracheostomy with delayed surgical intervention, though long-term follow up is needed to assess graft durability and maintenance of airway patency.

Congenital Midline Cervical Cleft: A Variant of Tessier Number 30 Cleft Causing Micrognathia.

Congenital midline cervical cleft is a rare and generally isolated malformation of the ventral neck region with no clear etiology established. Mandibular deformities, such as micrognathia, could be considered as a consequence of a cleft cervical contracture. Complete surgical excision of the subcutaneous fibrous cord at an early age is the primary treatment modality, minimizing growth development problems on surrounding affected tissue. The aim of this study is to describe the clinical, surgical, and histological findings in a female child with congenital midline cervical cleft along with a relevant literature review. Three years follow-up after surgery exhibited satisfactory functional and cosmetic results.

"Ortoglossopelveplastia" e o algoritmo de sua utilização na sequência de Pierre-Robin / "Orthoglossopelveplasty" and the algorithm for its use in the Pierre Robin sequence

Introdução: Muitos pacientes portadores de sequência de Pierre Robin (micrognatia, glossoptose e obstrução de via aérea) apresentam o músculo genioglosso alterado, encurtado e retrátil, que impede a protração lingual, mantendo a parte anterior da língua verticalizada e seu volume deslocado em direção posterior. Isso pode corroborar para obstrução supraglótica, dificuldade alimentar e inversão das forças de estímulo do crescimento do corpo mandibular. Métodos: Estudo retrospectivo de pacientes com Pierre Robin tratados entre 2012 e 2017 pela equipe, com descrição da "ortoglossopelveplastia", que propõe uma modificação na glossopexia, soltando o genioglosso anômalo da sua inserção, liberando a língua para elevar seu terço anterior e avançar o volume de sua base, sendo auxiliada por ponto de tração da base lingual à sínfise mandibular. Apresentamos um algoritmo de tratamento proposto que prioriza a necessidade desta cirurgia, associada ou não à distração mandibular, de acordo com a gravidade da dificuldade respiratória e/ou alimentar. Resultados: São apresentados 12 casos de obstrução da orofaringe atendidos de 2012 a 2017, discutem-se suas prioridades, a ortoglossopelveplastia e se aplica o algoritmo proposto. Conclusão: A reorganização anatômica da musculatura em uma posição anteriorizada correta proporciona protração e funcionalidade à língua, com desobstrução da via aérea na orofaringe, melhora da função alimentar e do desenvolvimento mandibular, com baixa morbidade cirúrgica e poucas complicações.

Introduction: Several patients with the Pierre Robin sequence (micrognathia, glossoptosis, and airway obstruction) have an altered, shortened, and retractable genioglossus muscle that prevents protraction of the tongue and keeps the anterior part of the tongue vertical and its volume posteriorly displaced. This can contribute to supraglottic obstruction, feeding difficulty, and inversion of the growth stimulation forces of the mandibular body. Methods: A retrospective study of patients with the Pierre Robin sequence treated between 2012 and 2017 with "orthoglossopelveplasty," which includes modification of glossopexy, releasing the anomalous genioglossus of its insertion and releasing the tongue to raise its anterior third and advance the volume of its base using a traction suture of the tongue base to the mandible symphysis. We present a treatment algorithm that prioritizes the need for surgery associated, or not, with mandibular distraction in accordance with respiratory and/or feeding difficulty severity. Results: Twelve cases of oropharyngeal obstruction treated from 2012 to 2017 are presented, their priorities and orthoglossopleoplasty are discussed, and the proposed algorithm is applied. Conclusion: Anatomical reorganization of the musculature in a correct anterior position provides protraction and functionality to the tongue, clears the airway in the oropharynx, and improves the feeding function and mandibular development, with low surgical morbidity rates and few complications.

Cephalometric Improvement of Severe Microretrognathia With an Anterior Open Bite Using Curvilinear Mandibular Distraction Osteogenesis.

Curvilinear mandibular distraction osteogenesis (MDO) provides for multidimensional augmentation and rotation in mandibles with complex, abnormal morphology. This study aims to demonstrate cephalometric changes after curvilinear MDO.A prospectively maintained craniofacial database was queried for patients undergoing curvilinear MDO from 2009 to 2015. Demographic and operative data were collected. Cephalometric measurements were obtained from preoperative and postdistraction imaging, including sella-nasion-B point angle (SNB), mandibular plane angle (MPA), occlusal plane angle (OPA), and gonial angle (GA). Measurements were compared using a paired t test.Nine patients exhibiting microretrognathia and an anterior open-bite deformity had a mean age of 8.4 ±â€Š6.0 years and a mean follow-up of 23.2 ±â€Š25.3 months. Mean distance distracted was 32.6 ±â€Š7.2 mm, with augmentation in length and height, and rotatory closure of anterior open bites. Preoperative SNB versus postdistraction was 66.9 ±â€Š4.2 versus 78 ±â€Š9.1° (P = 0.0029). MPA, OPA, and GA all decreased (61.0 ±â€Š10.7 vs. 45.7 ±â€Š11.1°, P = 0.0066, 37.3 ±â€Š8.9 vs. 25.7 ±â€Š6.0°, P = 0.0025, 140.3 ±â€Š16.1 vs. 127.3 ±â€Š13.2°, P = 0.0019, MPA, OPA, and GA, respectively), trending toward normalization. Airway diameter increased by 7.1 ±â€Š2.8 mm. Anterior bite improved by 7.2 ±â€Š1.9 mm. Complications arose in 2 subjects including device malfunction and wound infection.Curvilinear MDO results in dramatic cephalometric improvement in patients with severe microretrognathia and anterior open-bite. The procedure is associated with a high rate of complications, and long-term stability of movement has yet to be documented.

Fisura palatina en la secuencia de Pierre Robin / Palatal cleft in Pierre Robin sequence

Entre los niños que nacen con falta de fusión de la bóveda palatina, un número determinado corresponde a recién nacidos con síndrome de Pierre Robin. Aquí se abre un abanico de pacientes con un grado variable de complejidades y anomalías. Algunos que portan otros síndromes aún más complejos, que padecen la entidad por hipoplasia mandibular, hasta otros que solo poseen una modesta micrognatia que permitió el desarrollo de la secuencia. Si bien la fi sura del paladar es una arista más dentro de anomalías que pueden ser más complejas, debe corregirse a tiempo y de manera efi caz para permitir una adecuada fonación. En este trabajo se presentan, sobre 126 fi surados tratados durante 10 años en un hospital de atención pediátrica privado en Buenos Aires, 17 casos de síndrome de Pierre Robin, así como sus características, edad, sexo, tratamiento, resultados quirúrgicos y foniátricos, y complicaciones.

Between children born with cleft palate, a number of them are newborn with Pierre Robin Syndrome. These have a variety and diff erent degrees of complexity and abnormalities. Added complex syndromes may be found aff ecting them with hypoplasia and others with a slight micrognathia that allowed the development of the sequence. Cleft palate is only one between other abnormalities, but needs and must be corrected effi ciently on time to allow proper phonation. A number of 126 cleft palate patients treated and followed during 10 years in a private childrens hospital, in Buenos Aires, are included, and between them, 17 have Pierre Robin sequence. Their age, sex, treatment, surgical results and phonetic results are included.

Distracción ósea mandibular con aparatos reabsorbibles: reporte de tres casos / Mandibular distraction osteogenesis with resorbable devices: report of three cases

La micrognatia e hipoplasia unilateral mandibulares son un rasgo característico en algunos síndromes que incluyen defectos faciales, pero también se pueden presentar como secuelas de traumatismo sobre los tejidos duros del complejo maxilofacial. Dichas alteraciones adquiridas o congénitas, ocasionan grandes trastornos estéticosy funcionales. La distracción ósea descrita por Ilizarov como un método de elongación de los huesos largos, aplicado al área maxilofacial en años recientes, ha demostrado ser una técnica eficaz para la corrección de estos defectos. En este artículo se reportan tres casos de corrección de deformidades mandibulares en niños mediante la técnica de distracción ósea utilizando aparatos reabsorbibles, siendouna de sus ventajas el que no se requiere una segunda intervenciónquirúrgica para su retiro. Los pacientes fueron intervenidos en la Unidad Médica de Alta Especialidad (UMAE) No. 71 del Instituto Mexicano del Seguro Social, en donde desde hace 10 años se ha estadoutilizando el método de distracción ósea para manejo de deformidades mandibulares en niños con muy buenos resultados.

Mandibular micrognathia and unilateral mandibular hypoplasia are atypical feature of certain facial deformity syndromes, though can alsoappear as sequelae of maxillofacial hard tissue trauma. Acquired orcongenital alterations of this nature can have a signifi cant aestheticand functional impact. Ilizarov describes distraction osteogenesis as amethod for lengthening long bones, one that in recent years has beenapplied to the maxillofacial region and proved an eff ective technique forcorrecting these defects. This article reports three cases of mandibulardeformity correction in children, carried out using the distraction osteogenesistechnique with resorbable devices. One of the advantagesof using such devices is that the need for a second surgical interventionto remove them is eliminated. All patients were treated at AdvancedSpecialty Medical Unit (UMAE) 71 of the Mexican Institute of SocialSecurity, which, for the last ten years, has been using the distractionosteogenesis method to correct mandibular deformities in children,with optimal results.

A new way to anchor the external device in mandibular distraction: three case reports with a Pierre Robin sequence.

Pierre Robin sequence is a pathology derived from alteration in the first and second branchial arch. Patients have breathing problems due to micrognathia and glossoptosis, causing severe upper airway obstruction. One surgical treatment is distraction osteogenesis. Three patients with Pierre Robin sequence (case 1, 3 months old; cases 2 and 3, 1 month old) with severe upper airway obstruction requiring mechanical ventilator assistance, underwent mandibular distraction osteogenesis prematurely with a new anchoring system, thus avoiding tracheostomy and its consequences. An intraoral approach was used to avoid scarring. A new anchoring device with transfixing Kirschner wire in the proximal (mandibular ramus) and distal segment (chin zone) was used. This diminishes the risk of distractor device displacement, guaranteeing optimal stability. A more anterior installation reduces the risk of damaging tooth buds in the mandibular body and the inferior alveolar nerve. The more anterior the fixation, the more horizontal the distraction vector becomes. The position and stability of the device are crucial. In these three patients the placement of two transfixing Kirschner wires using an intraoral approach showed good results and stability during the period of distraction and consolidation, with optimal results on the upper airway, avoiding tracheostomy.

Tracheostomy-dependent child with temporomandibular ankylosis and severe micrognathia treated by piezosurgery and distraction osteogenesis: case report.

Ankylosis of the temporomandibular joint in children is one the most difficult and complex conditions managed by oral and maxillofacial surgeons, and often leads to some facial deformity. Distraction osteogenesis of the mandible provides an excellent treatment for mandibular airway obstruction in children who do not respond to conservative measures, and allows for early removal of the tracheostomy. We report the case of a 1-year-old boy with severe micrognathia and temporomandibular ankylosis who was dependent on a tracheostomy; he was treated with piezosurgery and mandibular advancement by distraction osteogenesis.

Mandibular distraction osteogenesis: a clinical experience of the last 17 years.

Mandibular distraction is the treatment of choice for the surgical correction of mandibular hypoplasia of different etiology. It is a surgical technique that is less invasive and time intensive; also, it has a significantly decreased morbidity rate.The experience of lengthening mandibles in the last 17 years had shown that in more severe mandibular hypoplasia, better results will be obtained in the long term with earlier treatment, especially in the more severe grades of hemifacial microsomia types IIB and III. Orthodontics plays a very important role to correct postdistruction occlusal changes and to promote a balanced growth.Besides the impressive aesthetic and functional results obtained in most cases, undoubtedly, the simultaneous expansion of the overlying soft tissues is the mayor advantage of this technique when it is compared with conventional osteotomies.With this clinical experience in the mandible using distraction osteogenesis, we confirm the farewell to major osteotomies to treat a wide range of deformities of this sophisticate anatomic structure.

Mandibular distraction osteogenesis in a patient with Melnick-Needles syndrome.

Melnick-Needles syndrome is an X-inked-dominant skeletal dysplasia in which there is deficient osteoblastic activity. Patients present with craniofacial anomalies consisting of a prominent forehead, exorbitism, mandibular hypoplasia, cheek fullness, and class II malocclusion. Severe mandibular hypoplasia leads to upper airway restriction, an increased incidence of sleep apnea and pneumonias, and occasionally respiratory failure. This is a report of a patient with Melnick-Needles syndrome who presented to our unit after multiple bouts of respiratory failure and with a tracheostomy in whom mandibular distraction osteogenesis was used to retire her tracheostomy and to cure her sleep apnea. The patient underwent bilateral, external, unidirectional mandibular distraction with a vector parallel to the occlusal plane. After a latency period of 5 days, distraction was initiated at a rate of 1 mm/day for 34 days. At this point, the patient was able to breathe with the tracheostomy plugged, and her occlusion had changed from a class II to a class III relationship. She no longer snored, and pulse oximetry on room air was normal while standing or supine. Interestingly, the patient's consolidation phase was prolonged--255 days--possibly attributable to altered bony metabolism. To our knowledge, this is the first reported case of mandibular distraction osteogenesis used to cure obstructive sleep apnea and eliminate the need for tracheostomy in a patient with Melnick-Needles syndrome. In the future, prophylactic mandibular distraction may prevent the need for tracheostomy in this group of patients.

Carotid artery injury during mandibular distraction.

BACKGROUND: Mandibular distraction is an effective treatment for mandibular hypoplasia. Special care must be done while performing the corticotomies to prevent injuries to blood vessels or nerves nearby. A rare iatrogenic severe injury occurred while performing this operation. REPORT OF PATIENT: A 3 year-old girl was operated upon to correct mandibular hypoplasia. A rotating saw was used to perform corticotomies in the mandibular angle. In the right side occurred acute bleeding and in the saw there was a piece of a blood vessel that was 15 mm long and 2 mm in diameter with an open lumen. Exploration of the neck revealed that the most distal segment of the internal carotid artery was severed, with a thrombus in the proximal end. The distal segment retracted into the temporal bone without bleeding. The proximal artery was ligated. After recovery she moved well. A computed tomogram was normal and further condition improved clinically. CONCLUSION: This complication from the use of a rotary saw has not been documented previously and it must be prevented by using some protection behind the bone.

Swallowing disorders in Pierre Robin sequence: its correction by distraction.

Pierre Robin sequence is characterized by micro-gnathia, glossoptosis, feeding difficulties, and upper respiratory obstruction, which are frequently complicated by bronchial aspiration and pulmonary infection. Gastroesophageal reflux is also common in these patients. To assess the results of mandibular distraction, a study was performed in 18 patients to detect swallowing disorders associated with apnea episodes and gastroesophageal reflux. Polysomnography, barium pharyngoscopy, determination of blood gases, and esophageal pH measurements were undertaken before and 4 months after distraction osteogenesis. Bilateral corticotomies, followed by distraction with external devices, were performed, achieving 7 to 19 mm of elongation (mean = 12 mm). Gastroesophageal reflux was found in 83% of cases associated with apnea episodes, but it disappeared after distraction osteogenesis. Mean preoperative oxygen saturation was 72%, and it was 93% afterward. The preoperative apnea index was 18.3, and the preoperative 8.5 hypopnea index was 8.5; both disappeared. Pharyngeal transit time became less than 1 second after treatment. Abnormal tongue movements and barium stasis in the pharyngeal recess and in the trachea were eliminated in all the patients.

Treatment of temporomandibular joint ankylosis in children: is it necessary to perform mandibular distraction simultaneously?

Temporomandibular joint (TMJ) ankylosis in children disturbs not only mandibular growth, but also facial skeletal development. Costochondral graft was used to ensure growth, but it had proven to be unpredictable. The authors evaluate retrospectively 41 patients who underwent temporomandibular joint reconstruction during the last 10 years. Twenty were treated by costochondral graft, 15 by arthroplasty, and 6 by other surgical procedures, and they were excluded. The etiology was septic in 54% of the cases. Follow-up was at least 12 months in all cases. Arthroplasty was a quicker and easier procedure than the costochondral graft, reducing operating time, risk of blood transfusion, and hospital stays and costs. It also was associated with less risk of reankylosis, 13%vs 25%. Furthermore, it was associated with a minor morbidity and secondary complications. Seventy-five percent of the patients treated with bone graft required additional secondary surgery. Radiographically, the authors observed a remodeled neocondyle at the level of proximal mandibular end in cases treated by arthroplasty. On clinical examination, patients showed variable degrees of facial deformity and an unknown potential of mandibular growth after TMJ arthroplasty. The authors also observed improved clinical and radiologic appearance after ankylosis correction. Is it reasonable to perform ankylosis release and mandibular distraction simultaneously without knowing which patients will be able to experience growth with time? In that case it would be necessary a predict growth to apply the exact amount of mandibular distraction for obtaining stable results. Timing of mandibular distraction, after TMJ arthroplasty is performed and mandibular function restored, must be specific to each patient's needs, assuring the best distraction conditions and planning. The authors present their treatment protocol, including TMJ joint arthroplasty with temporal muscle interposition, and mandibular distraction osteogenesis, as a second procedure, to correct residual asymmetry or retrognathism if necessary.

Tratamiento ortodóncico-quirúrgico de un caso de micrognatismo / Orthodontic-surgical treatment in a case of micrognathia

En algunos pacientes el déficit estético es de tal magnitud que los recursos ortodóncico-quirúrgicos que podemos utilizar pueden no ser suficientes para lograr un resultado satisfactorio. Esto se hace más crítico aún cuando el problema dentario impone limitaciones a la planificación del tratamiento. El caso que aquí presentamos abunda en limitaciones que lo transforman en un verdadero desafío para el logro de objetivos estéticos(AU)

Osteogenic distraction and orthognathic surgery to correct sequelae of ankylosis of the temporomandibular joint: a case report.

A 17-year-old female patient presented with sequelae to ankylosis of the temporomandibular joint, which included vertical maxillary protrusion, anterior open bite, labial incompetence, micrognathia, undefined neck angle, facial asymmetry, Class II molar relationship, and Class III canine relationship. She presented with the following cephalometric and soft tissue data: SNA angle = 78 degrees, SNB angle = 70 degrees, incisor-nasion-point A = 11 degrees, incisor-nasion-point B = 33 degrees, Frankfort-mandibular plane angle = 43 degrees, occlusal plane = 25 degrees, subnasale-stomion = 20 mm, stomion superius-stomion inferius = 9 mm, stomion inferius-soft tissue menton = 30 mm, neck angle = 144 degrees, and chin projection = 10 mm. Orthognathic surgery and mandibular osteogenic distraction were employed, specifically Le Fort I osteotomy to decrease a vertical excess of 12 mm, augmentation genioplasty of 17 mm, and bilateral extraoral distractors of bidirectional vector for a 14-mm augmentation of the mandible. The result was satisfactory with minimal adverse complications.