Cleft palate-lateral synechiae syndrome: a case report.

Cleft palate-lateral synechiae syndrome (CPLSS) is an extremely rare congenital malformation syndrome with undetermined etiology, characterized by a cleft palate and lateral intraoral synechiae linking the free borders of the palate to the mouth floor. We report a case of a female neonate, admitted for suckling difficulties with a cleft lip and palate associated to multiple lateral intraoral synechiae. Resection of the synechiae allowed oral feeding. Cleft palate-lateral synechiae syndrome is an exceptional syndrome as only seventeen cases have been reported in the literature. Synechiae can be isolated or more frequently in association with other congenital anomalies such as cleft lip and/or palate. These synechiae can cause functional deficits, especially in the respiratory and feeding tracts, language disorders or recurrent otitis. Although it is exceptional, this malformative entity must be known by medical practitioners in order to set up a well-adapted therapeutic protocol.

Anesthetic management in an infant with tetra-amelia syndrome with congenital maxillomandibular fusion: A case report.

A 10-month-old girl who had tetra-amelia syndrome and congenital maxillomandibular fusion (syngnathia) was scheduled for the surgical fusion separation. Anesthetic management for this case was considerably challenging. Standard monitoring was still applied to the patient's extremities. IV access was suspected to be difficult but firmly needed before intubation to provide resuscitation during an emergency. Connecting anesthetic circuit with nasopharyngeal airway was the preferred technique due to its benefits such as maintaining spontaneous ventilation, providing inhaled anesthetic, as well as monitoring oxygenation and ventilation. Importantly, the cornerstones for handling such complicated cases are multidisciplinary approach and teamwork.

Craniofacial phenotypes associated with Robinow syndrome.

Robinow syndrome is characterized by mesomelic limb shortening, hemivertebrae, and genital hypoplasia. Due to low prevalence and considerable phenotypic variability, it has been challenging to definitively characterize features of Robinow syndrome. While craniofacial abnormalities associated with Robinow syndrome have been broadly described, there is a lack of detailed descriptions of genotype-specific phenotypic craniofacial features. Patients with Robinow syndrome were invited for a multidisciplinary evaluation conducted by specialist physicians at our institution. A focused assessment of the craniofacial manifestations was performed by a single expert examiner using clinical examination and standard photographic images. A total of 13 patients with clinical and molecular diagnoses consistent with either dominant Robinow syndrome (DRS) or recessive Robinow syndrome (RRS) were evaluated. On craniofacial examination, gingival hyperplasia was nearly ubiquitous in all patients. Orbital hypertelorism, a short nose with anteverted and flared nares, a triangular mouth with a long philtrum, cleft palate, macrocephaly, and frontal bossing were not observed in all individuals but affected individuals with both DRS and RRS. Other anomalies were more selective in their distribution in this patient cohort. We present a comprehensive analysis of the craniofacial findings in patients with Robinow Syndrome, describing associated morphological features and correlating phenotypic manifestations to underlying genotype in a manner relevant for early recognition and focused evaluation of these patients.

Anquiloglosia y problemas de succión, tratamiento multidisciplinar: terapia miofuncional orofacial, sesiones de lactancia materna y frenotomía / Ankyloglossia and breastfeeding problems, multidisciplinary management: Orofacial myofunctional therapy, breastfeeding sessions and frenotomy

Introducción. La limitación de la movilidad lingual en neonatos puede ocasionar problemas en la lactancia, entre ellos: dolor-grietas-mastitis en la madre, mal progreso de peso del neonato y duración excesivamente larga de las tomas. El objetivo de este trabajo es evaluar la efectividad de los tratamientos realizados en los pacientes con anquiloglosia y trastornos de succión. Material y métodos. Estudio descriptivo preliminar de la efectividad del circuito establecido entre los servicios de Cirugía Oral y Maxilofacial, Logopedia y Rehabilitación Orofacial y Lactancia Materna para el tratamiento de los pacientes que acuden con problemas de lactancia materna y se les diagnostica anquiloglosia. Resultados. Se trató a 61 pacientes de edades entre 0 y 6meses con anquiloglosia asociada a problemas clínicos relacionados con la lactancia: 20 niñas (32.8%) y 41 niños (67.2%). Se establecieron 3 grupos según el tratamiento realizado: grupo1 (n=6) únicamente las sesiones de lactancia materna (SLM), grupo2 (n=19) terapia miofuncional (TMF) y asesoramiento en SLM, y grupo3 (n=36) frenotomía y siguieron TMF y asesoramiento de SLM. La anquiloglosia tipoiii es el frenillo lingual más frecuente (57.4%). En el total de la muestra se observaron mejorías en los parámetros que valoran la efectividad y el confort de la lactancia materna. Conclusiones. Mejorar la succión es posible; se recomienda estimular la succión con terapia miofuncional antes y después de la frenotomía, y también en aquellos casos en los que no será necesaria la cirugía (AU)

Introduction. The limitation of lingual mobility in newborns can cause problems in lactation. Among these problems are, soreness, cracked nipples or mastitis in the mother, poor weight gain of the newborn, and an excessively prolonged period of time in each breastfeed. The aim of this study is evaluate the effectiveness of the treatment received by the newborns with breastfeeding problems and ankyloglossia. Material and methods. A preliminary study of the effectiveness of the circuit established between the Oral and Maxillofacial Surgery, Speech Therapy and Orofacial Rehabilitation and the Breastfeeding Department to treat patients that attended the hospital with breastfeeding problems and were diagnosed with ankyloglossia. Results. A total of 61 patients with ages between 0 and 6 months had ankyloglossia were seen due to clinical problems related to breastfeeding. Of these, 20 (32.8%) were girls and 41 (67.2%) were boys. Three groups were established in accordance with the treatment carried out: group1 (n=6) solely from the Breastfeeding Sessions (BFS), group2 (n=19) Myofunctional Therapy (MFT) and BFS, and group3 (n=36) Frenotomy, followed by MFT and BFS. Ankyloglossia type3 was the most frequent lingual frenulum (57.4%). From the total sample, improvements were observed in the parameters that assessed the effectiveness and comfort of breastfeeding. Conclusions. Improving breastfeeding is possible, and in some cases, surgery should not be necessary. If surgery is required, it is recommended to stimulate suction before and after the frenotomy with myofunctional therapy (AU)

Aplicaciones del colgajo sural medial en cirugía reconstructiva de cavidad oral y orofaringe / Applications of medial sural artery perforator flap in oral cavity and oropharynx reconstruction

Objetivos. Presentar nuestra serie clínica de colgajos de perforantes basados en la arteria sural medial revisando las indicaciones, las características del colgajo y las complicaciones asociadas. Material y métodos. Análisis retrospectivo de 25 pacientes con defectos de cavidad oral y orofaringe reconstruidos con colgajo sural medial desde 2010 hasta 2013 en el Servicio de Cirugía Oral y Maxilofacial del Hospital Universitario 12 de Octubre de Madrid. Resultados. El rango de edad fue de los 18 a los 82 años, siendo la edad media de 56,6 años. En cuanto a la distribución por sexos 14 eran varones y 11 mujeres. De los 25 colgajos realizados 15 fueron del miembro inferior izquierdo y 10 del derecho. La longitud media del pedículo fue de 11,21cm (7-16cm). En 8 casos presentaban 2 perforantes. No se evidenció ninguna perforante a menos de 8cm del pliegue poplíteo. El tamaño de la isla cutánea varió según las necesidades del defecto, entre 4 y 12cm de largo y de 2,5 a 6cm de ancho, con una media de 9,63×4,94cm. El cierre de la zona donante fue directo, sin precisar en ningún caso injerto dermoepidérmico. Dos colgajos presentaron necrosis total del mismo. Dos pacientes presentaron celulitis y dehiscencia parcial de la herida quirúrgica en miembro inferior, que evolucionó favorablemente con tratamiento antibiótico y curas locales. Conclusiones. El colgajo de perforantes de la arteria sural medial es una excelente opción en la reconstrucción de defectos de la cavidad oral y la orofaringe, proporcionándonos una adecuada adaptabilidad a la zona receptora y una mínima morbilidad de la zona donante (AU)

Objective. To present a clinical series of medial sural artery perforator flaps, including a review of its applications, characteristics, and related complications. Material and methods. A retrospective study was conducted on 25 patients who underwent oral cavity and oropharynx reconstruction with medial sural artery perforator flap from 2010 to 2013 in the Oral and Maxillofacial Surgery Department of the 12 de Octubre University Hospital (Madrid, Spain). Results. The ages ranged from 18 to 82 years (mean 56.6), with 14 males and 11 females. Of the 25 harvested flaps, 15 were from the left lower limb and 10 from the right. The mean pedicle length was 11.21cm (7-16cm). There were 2 perforations in 8 cases, but with no evidence of perforations less than 8cm away from the popliteal crease. The size of the skin paddle varied according to the defect, from 4 to 12cm long and 2.5 to 6cm wide, with a mean of 9.63×4.94. Direct closure of the donor site was performed in all cases with no skin graft needed. Two flap failures were reported. Two patients had inflammation and partial dehiscence of the surgical wound in the lower limb, with good results after local and antibiotic treatment. Conclusions. The medial sural artery perforator flap is an excellent alternative in oral cavity and oropharynx reconstruction, providing great adaptability to the defect and minimal donor site morbidity (AU)

Congenital oral synechia and ankyloblepharon filiforme adnatum: Case report and literature review.

INTRODUCTION: Congenital intraoral synechias are adhesions of intraoral structures involving soft tissue. These conditions and another anomaly called ankyloblepharon filiforme adnatum, which is partial fusion of the eyelid margins by bands of tissue, are rarely reported in the literature. The association of both anomalies of the craniomaxillofacial region is an even more unusual finding. PURPOSE: This article adds more information to the literature by reporting a case of a neonate with a rare association of bilateral synechia between the jaws, fusion of buccal mucosa to the ridges and between the tongue and palate, and ankyloblepharon filiforme adnatum in both eyes, without any other facial or systemic malformation. METHODS: A literature review of similar cases published in PUBMED/MEDLINE was conducted followed by a discussion and comparison to the new case. CONCLUSION: The rare association of these congenital defects, without the presence of syndromic symptoms, could be diagnosed and treated easily with good prognostic evolution.

Dos casos de angina bullosa hemorrágica espontánea / Two cases of spontaneous angina bullosa hemorrhagica

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Dry mouth: Xerostomia and salivary gland hypofunction.

BACKGROUND: Mouth dryness may present as salivary gland hypofunction (SGH), xerostomia or both. It is considered one of the most underappreciated, underdiagnosed and undermanaged oral health conditions. Despite its common presentation and adverse impact on life quality, it is also generally poorly understood. Increased awareness of the condition is important in addressing these problems. OBJECTIVE: This article discusses SGH and xerostomia, and the associated intra-oral and extra-oral implications. It also summarises currently available management approaches and the evidence behind them. DISCUSSION: SGH and xerostomia are complex problems. None of the currently available management approaches are entirely satisfactory. Addressing the causative or contributing factors is therefore paramount. While oral health complaints are generally left up to the dental professional to manage, the nature of mouth dryness necessitates increased dialogue between the dental and medical professions to ensure optimal patient care.

Lingual frenulum and malocclusion: An overlooked tissue or a minor issue.

CONTEXT: Tongue-tie (more formally known as ankyloglossia) is a congenital anomaly characterized by an abnormally short lingual frenulum, which may restrict mobility of the tongue tip impairing its ability to fulfill its functions. The clinical significance of ankyloglossia is varied; rarely symptomatic to a host of problems including infant feeding difficulties, speech disorders, malocclusions, and others. AIMS: The need of this study was to evaluate the occurrence and severity of tongue-tie and its association with the type of malocclusions in two populations and also to correlate the various malocclusion traits with the grades of tongue-tie. SUBJECTS AND METHODS: A total of 700 school children in the age group of 9-17 years were examined for the presence of tongue-tie, 350 from regular schools and 350 from special schools. The presence was evaluated, measured, and graded into Grades I-V according to Kotlow's method. The malocclusion was assessed according to Angle's classification. Severity of crowding was assessed by Little's irregularity index in each case. Various other malocclusion traits were visually assessed, in relation to the positions of the teeth. STATISTICAL ANALYSIS USED: Spearman's rank correlation method was used to test the relationship between type of malocclusions, grades of tongue-tie and severity of crowding. Significance was considered at the (P < 0.05) level. RESULTS: Statistically significant differences were seen between grades of tongue-tie and Angle's types of malocclusion and Spearman's correlation between them showed negative correlation. The Spearman's correlation showed a negative correlation between tongue-tie grades and severity of crowding. CONCLUSIONS: As the grade of tongue-tie increased, its association with Classes I and II malocclusion decreased. The lower grades of tongue-tie are associated with increased lower incisor crowding. Shorter, tight frenulums are more associated with maxillary constriction, anterior open bite, and spacing of the lower anteriors.

Breastfeeding difficulties and oral cavity anomalies: The influence of posterior ankyloglossia and upper-lip ties.

OBJECTIVE: Oral cavity anomalies may contribute to breastfeeding problems. The objective of this study was to describe our experience in a high-volume breastfeeding difficulty clinic with a focus on posterior ankyloglossia and upper-lip ties. METHODS: A retrospective review of patients from a dedicated breastfeeding difficulty clinic from January 2014 to December 2014 was performed. Those identified to have ankyloglossia and/or upper-lip ties underwent release procedures. Subjective breastfeeding changes were documented afterwards. RESULTS: Of the 618 total patients, 290 (47%) had anterior ankyloglossia, 120 (19%) had posterior ankyloglossia, and 14 (2%) had upper-lip tie. Some patients had both anterior ankyloglossia and upper lip-tie (6%), or posterior ankyloglossia and upper-lip tie (5%). For those with anterior ankyloglossia, 78% reported some degree of improvement in breastfeeding after frenotomy. For those with posterior ankyloglossia, 91% reported some degree of improvement in breastfeeding after frenotomy. Upper lip-tie release also led to improved breastfeeding (100%). CONCLUSIONS: Anterior and posterior ankyloglossia and upper-lip tie, or combinations thereof, were commonly recognized in our study population. Many newborns, however, also had no oral cavity anomalies. Although causation cannot be implied, these oral cavity anomalies may contribute to breastfeeding difficulties in some cases.

[Diagnosis and management of ankyloglossia in young children]. / Diagnostic et prise en charge de l'ankyloglossie chez le jeune enfant.

Ankyloglossia is a common condition. Its prevalence is between 3.2% and 4.8% depending on the series and is largely underestimated given the fact of non-diagnosis when the symptoms are limited. It is defined as a short lingual frenulum resulting in a limitation of the lingual mobility. It is due to a defect in cellular apoptosis embryogenesis between the floor of the mouth and tongue. The result is a fibrous and short lingual frenulum. Several classifications were used to make the diagnosis. However, these are the clinical implications, particularly on food and primarily breastfeeding in the baby and phonation in older children that will motivate the management. This is surgical and different techniques are available: infants before the age of 6 months and when the lingual frenulum is still a fine cellular membrane, frenotomy is recommended. Frenectomy with or without frenoplasty is indicated for the older child. The surgery is simple, the results are good and rapidly improving grievances. Complications are rare. Finally, speech therapy is important when there are implications for phonation.

Accessory tongue: Classification and report of a case.

BACKGROUND: Accessory tongue (AT) is a rare congenital anomaly. Due to rarity of AT, various terminologies are applied, including accessory tongue, bifid tongue, double tongue, cleft tongue, and supernumerary tongue. It seems that the anomaly has geographic distribution and most reported cases are from India and Middle East. No comprehensive classification has been introduced yet. So, we present a classification for AT according to review of all papers and documents that we found, and report a 2-month-old male infant with this anomaly.

Variability of systemic and oro-dental phenotype in two families with non-lethal Raine syndrome with FAM20C mutations.

BACKGROUND: Raine syndrome (RS) is a rare autosomal recessive bone dysplasia typified by osteosclerosis and dysmorphic facies due to FAM20C mutations. Initially reported as lethal in infancy, survival is possible into adulthood. We describe the molecular analysis and clinical phenotypes of five individuals from two consanguineous Brazilian families with attenuated Raine Syndrome with previously unreported features. METHODS: The medical and dental clinical records were reviewed. Extracted deciduous and permanent teeth as well as oral soft tissues were analysed. Whole exome sequencing was undertaken and FAM20C cDNA sequenced in family 1. RESULTS: Family 1 included 3 siblings with hypoplastic Amelogenesis Imperfecta (AI) (inherited abnormal dental enamel formation). Mild facial dysmorphism was noted in the absence of other obvious skeletal or growth abnormalities. A mild hypophosphataemia and soft tissue ectopic mineralization were present. A homozygous FAM20C donor splice site mutation (c.784 + 5 g > c) was identified which led to abnormal cDNA sequence. Family 2 included 2 siblings with hypoplastic AI and tooth dentine abnormalities as part of a more obvious syndrome with facial dysmorphism. There was hypophosphataemia, soft tissue ectopic mineralization, but no osteosclerosis. A homozygous missense mutation in FAM20C (c.1487C > T; p.P496L) was identified. CONCLUSIONS: The clinical phenotype of non-lethal Raine Syndrome is more variable, including between affected siblings, than previously described and an adverse impact on bone growth and health may not be a prominent feature. By contrast, a profound failure of dental enamel formation leading to a distinctive hypoplastic AI in all teeth should alert clinicians to the possibility of FAM20C mutations.

Systematic Evaluation of the Upper Airway in a Sample Population: Factors Associated with Obstructive Sleep Apnea Syndrome.

OBJECTIVES: To investigate the anatomy of the upper airway (UA) of a representative sample of the adult population of São Paulo city, Brazil, and to identify factors associated with the presence of obstructive sleep apnea syndrome (OSAS), as confirmed using full-night polysomnography (PSG). STUDY DESIGN: Cross-sectional study. SETTING: Population-based sample. METHODS: A 3-stage sampling procedure was used to proportionally recruit adult residents of São Paulo city according to gender, age, and socioeconomic status. A complete evaluation was performed, including a systematic evaluation of the UA prior to conducting PSG. RESULTS: Nine-hundred ninety-three (90.2%) of the participants were seen by an ear, nose, and throat (ENT) specialist. Individuals who were diagnosed with OSAS (32.9%) presented a higher frequency of nasal symptoms and structural abnormalities (both nasal and oropharyngeal) compared with those without OSAS. No anatomical differences were observed in the facial skeleton. An abnormal nasal structure visible via anterior rhinoscopy was the only UA factor predicting OSAS after adjustments for the other common OSAS risk factors (male sex, aging, obesity, and increased neck circumference). CONCLUSION: This is the first study in which a systematic evaluation of the UA was followed by a sleep study in a population-based sample. In a sample of the general population that had not previously been screened for OSAS, having an abnormal nasal structure was found to be a risk factor for OSAS, in conjunction with other well-established clinical and demographic factors, such as male gender, increased age, increased neck circumference, and body mass index.

Reconstrução de lábio utilizando o retalho randomizado cervical do músculo platisma / Lip reconstruction with cervical platysma muscle randomized flaps

INTRODUÇÃO: A reconstrução cirúrgica da deformidade labial exige bom conhecimento anatômico e técnica cirúrgica. Fatores como localização da lesão, extensão, profundidade e tratamento inicial adequado constituem variáveis que são analisadas em conjunto para a estratégia cirúrgica mais apropriada. O retalho de Tsur é definido como randomizado do músculo platisma que pode ser elevado do pescoço como tubo unipediculado ou bipediculado, dependendo do tamanho e da localização da perda de substância. Pode incluir área pilosa cervical e ser utilizado em defeitos de plano parcial ou total do lábio. MÉTODO: Foram operados três pacientes com perda de substância labial extensa. Foi utilizado retalho bipediculado em dois casos e unipediculado em um caso. RESULTADOS: No primeiro paciente, foi liberado um dos pedículos do retalho cervical no 15º dia do pós-operatório. Após 30 dias, o outro foi liberado após integração completa ao lábio superior. A partir deste, retalhos ao acaso foram confeccionados para reconstrução do lábio inferior, columela e ponta nasal. O segundo paciente evoluiu com necrose da porção central do retalho, sendo realizada confecção de leito de ancoragem próximo à comissura. Apresentou melhora da continência salivar. O terceiro paciente evoluiu bem, sendo liberado o retalho lingual utilizado para reconstruir o lábio superior após 3 semanas. CONCLUSÃO: A reconstrução com o retalho de Tsur mostrou-se útil nos casos de reconstrução total do lábio superior e inferior, além de ser ótima alternativa na situação de impossibilidade de utilização do retalho microcirúrgico e de permitir um resultado estético aceitável com recuperação da função mastigatória e da fala adequadas, apesar da complexidade inicial dos casos.

INTRODUCTION: Surgical reconstruction of lip deformities requires proper anatomical knowledge and surgical techniques. Factors such as location, extent, depth, and appropriate initial treatment of the lesion are parameters that are simultaneously analyzed to identify the most suitable surgical strategy. The flap described by Tsur is defined as a platysma muscle randomized flap, and can be raised from the neck as a unipedicled or bipedicled tube, depending on the size and location of the loss of substance. This may include the hairy cervical area, and it can be used in defects of the partial or total plane of the lip. METHOD: Three patients with extensive loss of lip substance underwent surgery. A bipedicled flap was used in two cases, and a unipedicled flap in one case. RESULTS: In the first patient, one pedicle of the cervical flap was released on the 15th postoperative day. After 30 days, we released the other, after full integration into the upper lip. From this, flaps were randomly made for the reconstruction of the lower lip, columella, and nasal tip. The second patient developed necrosis of the central portion of the flap, and the recipient bed was anchored next to the commissure. The patient showed improved salivary continence. The third patient progressed well, and the tongue flap used to reconstruct the upper lip was released three weeks later. CONCLUSION: Reconstruction with a Tsur flap was useful in cases of total reconstruction of the upper and lower lip, in addition to being a great alternative in situations in which it was impossible to use microsurgical flaps. This technique also allowed the achievement of satisfactory aesthetic outcomes, and resulted in the recovery of masticatory function and appropriate speech, despite the initial complexity of the cases.

Surgical speech disorders.

Most speech disorders of childhood are treated with speech therapy. However, two conditions, ankyloglossia and velopharyngeal dysfunction, may be amenable to surgical intervention. It is important for surgeons to work with experienced speech language pathologists to diagnose the speech disorder. Children with articulation disorders related to ankyloglossia may benefit from frenuloplasty. Children with velopharyngeal dysfunction should have standardized clinical evaluation and instrumental asseessment of velopharyngeal function. Surgeons should develop a treatment protocol to optimize speech outcomes while minimizing morbidity.

Syngnathia and obstructive apnea in a case of popliteal pterygium syndrome.

UNLABELLED: We describe an infant with popliteal pterygia, syngnathia, cleft lip and palate, and retrognathia diagnosed with popliteal pterygium syndrome (PPS). The neonatal course was complicated by severe obstructive apnea necessitating tracheostomy. CONCLUSION: This report illustrates the potential for airway compromise in PPS patients and the need for thorough neonatal airway assessment.

Comparisons of the latching on between newborns with tongue-tie and normal newborns.

BACKGROUND: Breastfeeding is recommended as the exclusive feeding for the first six months of the newborns life. Difficulty in latching and breastfeeding resulting from tongue-tie are believed to be a problem. OBJECTIVE: To compare the latching on between newborns with tongue-tie (ankyloglossia) and normal newborns. MATERIAL AND METHOD: The subjects were 833 normal, postpartum women who delivered without complications at HRH Princess Maha Chakri Sririndhorn Medical Center in Nakhon Nayok Province between January and June 2013. Their newborns oral cavities' were checked for tongue-tie screening and diagnoses using Kotlow's criteria. Latch scores were used for latch-on assessment at the second day postpartum. Demographic data and latch scores were collected and analyzed by the t-test, Chi-square test, Odds ratio with 95% confidence interval and One-way of ANOVA test. RESULTS: The data shows that the incidence of tongue-tie was 13.4% (6.2% with mild tongue-tie, 5.5% with moderate tongue-tie, and 1.7% with severe tongue-tie). The mean of latch scores in the tongue-tied groups were significantly lower than that in the normal group, especially in the moderate and severe tongue-tie subgroups. The odds ratios for latch scores were < or = 8 compared between the moderate and severe tongue-tied subgroups and the score in the normal and mild tongue-tied subgroups was 1.4. CONCLUSION: The latch scores in tongue-tied newborns were significantly lower than those in normal newborns.

The Tessier number 3 cleft: a report of 10 cases and review of literature.

The Tessier number 3 cleft is one of the most intricate and destructive of all facial clefts, presenting surgeons with a difficult task for reconstruction. We present a series of 10 patients with this rare cleft all treated by a single surgeon over 30 years. All patients with Tessier number 3 clefts treated between 1978 and 2008 by the senior surgeon were reviewed. Demographic data and all associated clinical findings including cranial and extracranial anomalies were recorded. Methods used to reconstruct each patient were also noted. Seven males and three females were identified and age at initial treatment ranged from 12 months to 12 years. Mean follow-up was 6.3 years. Multiple craniofacial anomalies were appreciated including other rare facial clefts, hypertelorbitism, lacrimal obstruction, anophthalmia, choanal atresia, and hemifacial microsomia. Amniotic banding was the most prominent extracranial finding noted in these patients. Tessier number 3 clefts can be associated with multiple other craniofacial anomalies making reconstruction challenging. Soft tissue and bony reconstruction must be considered separately, and a variety of tools may be employed to accomplish each goal. As the presentation can be highly variable, an individualized treatment plan must be made to meet each patient's specific needs.