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Tessier number 10 cleft is one of the rarest facial clefts. Surgical treatment of this type of cleft is challenging due to the complexity of periorbital and temporal soft tissue deformities. A 23-year-old male patient presented with typical facial deformities of Tessier number 10 cleft. The surgical procedure involved using a free anterolateral thigh flap to reconstruct the eye socket, while the superficial temporal artery pedicle scalp flap was used to reconstruct the eyebrow deformity. The patient had no complications and 16 months after surgery, the patient had good aesthetic results. A hair-bearing scalp flap with a pedicle of the frontal branch of the superficial temporal artery combined with an anterolateral thigh-free flap can effectively resolve most soft tissue deformities of Tessier number 10 cleft and reconstruct the orbital socket in a single surgery. At the same time, it augments the soft tissue of the frontotemporal area and provides good aesthetic results. [ABSTRACT FROM AUTHOR]

Les fentes frontales orbitaires supérieures font partie des fentes rares craniofaciales décrites par Tessier en 1976, dont la survenue est le plus souvent sporadique. Elles sont numérotées 9, 10 et 11 dans cette classification et siègent respectivement latéralement, médianement et médialement vis-à-vis de la partie supérieure de l'orbite. Leurs expressions cliniques sont variables sur les tissus mous et osseux, avec une dissociation d'atteinte possible, et vont d'un simple défaut esthétique à la mise en jeu du pronostic fonctionnel oculaire. Le scanner est systématiquement justifié dans ce cadre. Leur prise en charge doit être adaptée au vu du polymorphisme des atteintes et repose sur une prise en charge multidisciplinaire, avec une reconstruction chirurgicale des parties molles urgente en cas de mise en jeu du pronostic oculaire. Dans les autres cas, la prise en charge est différée mais doit rester précoce afin de permettre un bon développement de l'enfant. Superior orbital frontal clefts are one of the rare craniofacial clefts described by Tessier in 1976, and occur most often sporadically. They are numbered 9, 10 and 11 in this classification, and are located respectively laterally, in the middle and medially to the upper part of the orbit. Their clinical expression is variable on soft tissue and bone, with possible dissociation of involvement. They range from a simple aesthetic defect to an eyes functional prognosis. CT scans are systematically required in this context. Their management must be adapted to the polymorphism of the damage, and is based on multidisciplinary approach. In case of ocular risk, the eyelid reconstruction is an emergency. In all other cases, treatment is deferred, but must be carried out at an early stage to ensure the child's healthy development. [ABSTRACT FROM AUTHOR]

Background: Amniotic band syndrome (ABS) is a rare congenital disease characterized by a broad spectrum of congenital anomalies resulting from the strangulated developing organ(s) by the detached fibrous amniotic band. The prevalence of CNS involvement in ABS is rare, but the mortality rate in these cases is high, while morbidity among the surviving patients is inevitable. Case report: Three-month-old male, 9-month-old female, and newborn female babies were presented with head lump(s), severe facial cleft, syndactyly, and finger amputation. The patient's head imaging confirmed meningoencephalocele as the cause of the head lump in 2 patients; meanwhile, a porencephalic cyst was identified as the origin of head lumps in the other patient. VP shunt placement surgery was performed as the initial management in 2 patients, while one patient directly underwent meningoencephalocele resection surgery. Craniofacial and limb reconstructions were planned as the follow-up management in all cases. Unfortunately, one patient died of complications from suspected aspiration, while another never returned for follow-up treatment. Conclusion: Here, we report 3 ABS cases with CNS involvement. Despite the severe disfigurement and disability, the inexistence of fatal malformation might lead to long-term survival. The treatment of malformation(s) that might predispose to another fatal condition and surgery(-ies) to improve functional outcomes and patient's social acceptability should be prioritized in managing the surviving ABS patients. [ABSTRACT FROM AUTHOR]

Background: Patients with craniofacial clefts who have had reconstructive surgery often develop problems, such as contour deformity that will cause psychological issues. Micro and nano fat grafting are various methods utilized to overcome these issues. Case and Operation Technique: This case series describes two patients with facial contour deformities due to Tessier 3 and 4 craniofacial clefts, respectively. Each patient underwent secondary reconstruction correction surgery followed by a combination of micro and nano fat grafting. Discussion: Results on day 7 after surgery were promising. Growth factors and stem cells in nano fat complemented the micro fat properties, thus increasing the survivability rate. Conclusion: This case series demonstrates that the combination of micro and nano fat shows promising results for overcoming facial contour deformity. [ABSTRACT FROM AUTHOR]

Facial clefts are rare facial anomalies. Among them, oro-ocular cleft remains a challenging malformation due to ectropion of the inferior palpebra which can constitute an emergency when the patient's globe is exposed leading to exposure keratitis and blindness. Here we report surgical procedures to correct lower eyelid ectropion using tarsoconjunctival-skin flap performed on 2 cases. In conclusion, tarsoconjunctival-skin flap can be provided to effectively correct lower eyelid ectropion and is a better option when nose correction is not involved simultaneously. [ABSTRACT FROM AUTHOR]

Background: 3D printing is being utilized in almost every aspect of medicine. 3D printing has especially been used in conjunction with 3D ultrasonography to assist in antenatal assessment and presurgical planning with fetal malformations. As printing capabilities improve and applications are explored there may be more advantages for all parents to visualize and touch 3D printed models of their fetus. Case presentation: We present three cases involving 3D printed models and four different but interrelated psychological constructs- antenatal depression, antenatal anxiety, maternal-fetal attachment, and paternal-fetal attachment. Each case shows for the first time possible beneficial effects within these prevalent and significant problems. Conclusions: The degree to which the anxiety, depression, and attachment scores improved after the presentation of the 3D printed models is encouraging. Randomized controlled trials utilizing 3D printed models to improve psychological constructs should be supported considering the findings within these four cases. [ABSTRACT FROM AUTHOR]

Embryonic craniofacial development depends on the coordinated outgrowth and fusion of multiple facial primordia, which are populated with cranial neural crest cells and covered by the facial ectoderm. Any disturbance in these developmental events, their progenitor tissues, or signaling pathways can result in craniofacial deformities such as orofacial clefts, which are among the most common birth defects in humans. In the present study, we show that Rdh10 loss of function leads to a substantial reduction in retinoic acid (RA) signaling in the developing frontonasal process during early embryogenesis, which results in a variety of craniofacial anomalies, including midfacial cleft and ectopic chondrogenic nodules. Elevated apoptosis and perturbed cell proliferation in postmigratory cranial neural crest cells and a substantial reduction in Alx1 and Alx3 transcription in the developing frontonasal process were associated with midfacial cleft in Rdh10 -deficient mice. More important, expanded Shh signaling in the ventral forebrain, as well as partial abrogation of midfacial defects in Rdh10 mutants via inhibition of Hh signaling, indicates that misregulation of Shh signaling underlies the pathogenesis of reduced RA signaling-associated midfacial defects. Taken together, these data illustrate the precise spatiotemporal function of Rdh10 and RA signaling during early embryogenesis and their importance in orchestrating molecular and cellular events essential for normal midfacial development. [ABSTRACT FROM AUTHOR]

Tessier Craniofacial Clefts Numbers 0 and 1 represent unique facial deformities, with Number 0 involving midline structure hypoplasia and Number 1 exhibiting features like a notched soft triangle and affected alar dome. These anomalies can extend near the midline, leading to complications like telecanthus, necessitating innovative surgical strategies for reconstruction. A five-month-old girl presented with Tessier 0 and 1 clefts and a dorsal nasal lipoma, challenging traditional repair methods due to structural limitations. This case required a comprehensive approach, including aesthetic excision of the lipoma and reconstruction of both the internal and external aspects of the nose. The patient underwent successful nasal reconstruction using a transpositional alar flap with a pedicle from the angular artery and a hinge flap for the inner lining. The procedure involved cranial dissection through the flap incision for lipoma excision. This case highlights the complexity of nasal reconstruction in the presence of facial clefts and demonstrates the effectiveness of the alar transpositional flap as a viable technique for achieving aesthetically pleasing outcomes. The case underscores the necessity for precise surgical planning and execution to address both cosmetic and functional aspects of nasal defects in facial cleft patients. • Unique challenge: Tessier 0 and 1 clefts with dorsal nasal lipoma complicate reconstruction • Alar and hinge techniques: employed for effective aesthetic nasal repair • Tailored surgical strategy: addresses both internal and external nasal defects [ABSTRACT FROM AUTHOR]

One of the rarest types of craniofacial malformations is Tessier cleft type 5. These are oblique facial clefts with bony defect representing an alveolar cleft in the premolar region. This extends to the lateral surface of the maxilla up to the infraorbital rim and orbital floor. The cleft begins in the upper lip, just medial to the commissure, and extends up to the eyelid. They may also show deficient maxillary growth on the side of the cleft, clinically represented as constriction in the maxilla and occlusal cant. It becomes very challenging to treat such cases and requires a team approach. The goals of such cases include surgical repair of the lower eyelid and outer canthus, expansion of the arch and alignment, Bone grafting for the alveolar cleft, zygomatic region, and orbital rim to restore the continuity. The following is a case report of Tessier type 5 facial cleft describing the clinical features, diagnosis, and treatment approach for the case.

Objective: Facial cleft involves complex malformations. No study assessed the facial deformity of Tessier No. 0 cleft with a bifid nose. Thus, we used anthropometric measurements to access the nose in patients.Methods: A total of 24 bifid nose deformities underwent surgery at our institution between 2010 and 2019. Standardized photographs were taken preoperatively and postoperatively. Landmarks were identified on these images; measurements for nasal analysis were performed and compared with the established Chinese norms. Surgical method differences were also analyzed.Results: The median follow-up time was 2.51 years. Postoperatively, there is a significant difference in comparison with preoperative in the nasal index, medial canthus and nose width index, nasolabial angle, nasofacial angle, ala length and nasal bridge length index, nasal tip protrusion and nasal width index, and nasal width and ala length index. Furthermore, the medial canthus and nose width index, and nasal width and ala length index were significantly larger in ordinary people, while ala length and nasal bridge length index and nasal tip protrusion and nasal width index were smaller. After surgery, most angles and index were standard except the nasolabial angle in the females, and ala length and nasal bridge length index in the males. Moreover, as for the group of costal cartilage transplantation, most index and angles have improved after surgery including nasolabial angle, nasofacial angle, ala length and nasal bridge length index, nasal tip protrusion and nasal width index, and nasal width and ala length index. However, only nasal tip protrusion and nasal width index, columella length and nasal tip protrusion index, and nasal width and ala length index in the silicone prosthesis group implantation has significance. Costal cartilage transplantation can also better improve ala length and nasal bridge length index than the silicone prosthesis implantation.Conclusion: Most defects can be repaired with surgery, but the outcome has a lack of evaluation. Thus, anthropometric assessment can serve as a material for nasal and reconstructive surgery.

Macrostomia is a congenital deformity found in Tessier no. 7 facial clefts defined as an enlargement of the mouth at the oral commissure. Several techniques are described in literature to achieve optimal functional and aesthetic results, with varying results and surgeon preferences. In this case series we report surgical repair of macrostomia with a vermillion square flap method for the oral commissure combined with either Z-plasty or W-plasty closure for the skin. A retrospective case analysis of 12 patients with macrostomia operated over the past 7 years at our plastic surgery division was performed (by two different operators; 11 cases by A.S. and 1 case by R.S.). Clinical features of the patients were analyzed through photography documentation, and patient description such as age of operation, operation technique, and complications were obtained through patient records. Macrostomia was corrected with a vermillion square flap method for commissure, overlapping muscle closure, along with either Z-plasty or W-plasty closure for the skin. Quality of lip commissure position, symmetry, thickness of vermillion, and scar result were recorded. In all twelve patients repaired with the overlapping muscle closure and square flap, the lip commissures were formed with satisfactory shape, position, and thickness with no commissure contracture during the follow up period. The Z-plasty was a simpler method compared to the W-plasty, and resulted in comparable scars. One patient (adult with hemifacial macrostomia and W-plasty skin closure) underwent revision surgery for more accurate symmetry and position of the oral commissure. There are many varieties of surgical repair for macrostomia, and each method should be adjusted and combined according to each patient. Overall, macrostomia repair with this technique combination produced satisfactory aesthetic and functional results in all twelve patients. Z-plasty for skin closure after muscle and vermillion closure was a simpler technique and resulted in comparable scars than W-pasty closure in this case series. • Macrostomia is defined as an enlargement of mouth at the oral commisure • Operative goals for macrostomia include functional repair dan enhanced aesthetic • Square flap and Z/W-plasty skin closure will reduced contractile scar and minimize the visible scar [ABSTRACT FROM AUTHOR]

The Rationale: Craniofacial clefts are rare malformations. According to their localization, 15 types are classified. Patient Concerns: They referred to impaired eyelid function, lateral protection of the eye-bulb, and aesthetic appearance. Diagnosis: A three-dimensional-computerized tomography scan and a stereolithographic model led to the diagnosis of a rare intraorbital Tessier 6 cleft. Treatment: A newly designed lateral orbital rim osteotomy, presurgically simulated on a stereolithographic model, was performed to forward position the lateral orbital rims bilaterally by osteodistraction. Outcomes: Both lateral orbital rims were distracted anteriorly for around 12 mm over a period of 9 weeks, without any side effects to the patient. Take-away Lessons: In this intraorbital Tessier 6 cleft, an improved eyelid function with better lateral protection of the eye bulb and more favorable aesthetic appearance was achieved with this novel lateral orbital rim osteotomy. [ABSTRACT FROM AUTHOR]

The anatomical study describes the normal aspects of the conformation of a certain species, that is, the characteristics that occur with high frequency in this. In this way, when an event occurs in most individuals of a species, it is considered as normal. Although anatomical variations are also observed, characterized by the alteration in the normal pattern in anatomy, it is defined as a small deviation from the normal morphological aspect of an organ or the deviation from the general plane of organization of an individual, events that in animals and humans is well studied. In the present case, craniofacial fissure is considered a rare congenital anomaly of unknown etiology. The location is usually in certain lines of eyelids, eyebrows, nostrils, lips and jaws, reaching soft and bony parts. In veterinary medicine it is little studied and its classification is based on the changes observed in humans based on the anatomical relations. The cracks may present with the characteristics of excess tissue or agenesis of the same. In humans, depending on the case, the treatment is based on suture of the soft tissues, in three planes: cutaneous, muscular and mucous; zetaplasty; orthodontic treatment; bone grafts, when necessary already in animals in the consulted bibliography there were no cases with treatment. The present report describes the changes related to a craniofacial fissure associated to palatoschisis, cheiloschisis, macroglossia, absence of the ocular orbit and consequently the eyeball in a newborn of dog. [ABSTRACT FROM AUTHOR]

Résumé Introduction L'alimentation des nouveau-nés avec une fente faciale reste problématique et la stratégie d'alimentation à la naissance est essentielle. L'objectif de cette étude était d'identifier l'impact de la fente sur la prise de poids physiologique et la technique d'alimentation la plus efficace pour la récupération du poids de naissance. Matériels et méthodes Il s'agit d'une étude rétrospective chez des nouveau-nés porteurs d'une fente isolée, nés à terme entre 1996 et 2016 et opérés en chirurgie plastique pédiatrique au centre hospitalo-universitaire de Montpellier. Les variables étudiées étaient le type de fente, la courbe de poids, les techniques d'alimentation, le type de lait utilisé et la durée de séjour. Résultats Quatre-vingt-dix-sept nouveau-nés ont été inclus avec 27 % de fentes labiales, 67 % de fentes labiopalatines et 6 % de fentes palatines. Au total, 33 avaient une courbe de poids pathologique (34 %). L'analyse multivariée retrouve une influence significative du type de fente (p = 0,03) sur la reprise de poids alors que le caractère unilatéral ou bilatéral de la fente (p = 0,28), la technique d'alimentation (p = 0,63), le type de lait (p = 0,84) et le type de tétine (p = 0,33) n'avaient aucune influence. Conclusions Les difficultés d'alimentation entraînant une reprise de poids pathologique sont présentes chez 1/3 des nouveau-nés porteurs de fente. Cela est essentiellement dû à la présence de fentes de type labiopalatines. Il n'a pas été mis en évidence de technique particulièrement plus efficace qu'une autre pour la reprise du poids mais les différentes techniques doivent être connues afin de s'adapter au nouveau-né. Summary Introduction Feeding newborns with a facial cleft remains problematic and feeding strategy at birth is essential. The objective of this study was to identify the impact of the cleft on physiological weight gain and the most effective feeding technique for the recovery of birth weight. Materials and methods This is a retrospective study in neonates with an isolated cleft, born at term between 1996 and 2016 and operated in pediatric plastic surgery at Montpellier University Hospital. The variables studied were the type of cleft, the weight curve, the feeding techniques, the type of milk used and the length of stay. Results Ninety-seven newborns were included with 27 % cleft lip; 67 % cleft lip and palate and 6 % cleft palate. In total, 33 had a pathological weight curve (34 %). The multivariate analysis found a significant influence of the type of cleft (P = 0.03) on the recovery of weight whereas the unilateral or bilateral character of the cleft (P = 0.28), the feeding technique (P = 0.63), the type of milk (P = 0.84) and the type of nipple (P = 0.33) had no influence. Conclusions Difficulties of feeding leading to a pathological recovery of weight are present in 1/3 of the newborns clefts carriers. This is mainly due to the presence of labio-palatal clefts. There is no evidence of a particularly more effective technique than another for weight recovery, but the different techniques must be known in order to adapt to the newborn. [ABSTRACT FROM AUTHOR]

Tomoko Miyake,1 Shota Kojima,1 Tetsuya Sugiyama,2 Mari Ueki,1 Jun Sugasawa,1 Hidehiro Oku,1 Kensuke Tajiri,1 Yuka Shigemura,3 Koichi Ueda,3 Atsuko Harada,4 Mami Yamasaki,4 Takumi Yamanaka,4 Hidetsuna Utsunomiya,5 Tsunehiko Ikeda1 1Department of Ophthalmology, Osaka Medical College, Takatsuki City, 2Nakano Eye Clinic of Kyoto Medical Co-operative, Kyoto, 3Department of Plastic and Reconstructive Surgery, Osaka Medical College, Takatsuki City, 4Department of Pediatric Neurosurgery, Takatsuki General Hospital, Takatsuki City, 5Department of Radiological Science, International University of Health and Welfare, Graduate School, Fukuoka, Japan Introduction: The incidence of facial cleft is rare and ranges between 1.43 and 4.85 per 100,000 births. To date, there have been few reports of detailed ophthalmologic examinations performed in cases of facial cleft. Here, we report a case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft. Case report: A 9-day-old female infant was delivered by cesarian section at 34 weeks of gestational age (the second baby of twins) and weighed 2,276 g upon presentation. She had a facial cleft and ectrodactyly at birth. Right eye-dominant blepharophimosis was obvious. Examination of the right eye revealed inferior corneal opacity with vascularization, downward corectopia, and optic-nerve hypoplasia. The corneal diameter was 8 mm in both eyes, and tonometry by use of a Tono-Pen® XL (Reichert Technologies, Depew, NY, USA) handheld applanation tonometer revealed that her intraocular pressure was 11–22 mmHg (Oculus Dexter) and 8 mmHg (Oculus Sinister). B-mode echo revealed no differences in axial length between her right and left eyes. When she was 15–16 months old, we attempted to examine her eyes before she underwent plastic surgery under general anesthesia. She had a small optic disc in both eyes and the right-eye disc was tilted. After undergoing canthotomy, gonioscopy and ultrasound biomicroscopy revealed that almost all directions were open except for the peripheral anterior synechia. Since magnetic resonance imaging revealed ventriculomegaly associated with an interhemispheric cyst at birth, a ventriculoperitoneal shunt was inserted at 12 days of age. At 25 months of age, her condition suddenly deteriorated due to occlusion of the ventricular shunt catheter, and she died 5 days later. In this patient, amniotic band syndrome was presumed to be the primary cause due to the clinical findings. Conclusion: We experienced a case of optic-nerve hypoplasia and anterior segment abnormality that occurred with facial cleft. The cause of these abnormalities is unclear, yet amniotic band syndrome is a possible candidate. Keywords: facial cleft, optic-nerve hypoplasia, amniotic band syndrome

Goldenhar syndrome is associated with various other congenital anomalies of gastro-intestinal tract, heart, kidney and genitourinary system. We report a rare association of Goldenhar syndrome with vestibular fistula and esophageal atresia.

Description and successful management of a patient with Tessier no. 0 and 3 facial cleft is being presented. Appropriate evaluation was done to rule out the presence of median cleft face syndrome. Lip cleft was repaired by straight line technique with staggering at the vermilion border. Nasal cleft was reconstructed by a transposition flap and the flap taken from the upper lip after correction of the midline cleft. Satisfactory outcome was achieved for this singular deformity by conforming to the basic tenets of plastic surgery.

A simple subcutaneous lesion such as an epidermoid cyst can present as an aesthetic challenge when located in the nasal tip. Usually the nasal dermoid presents as swelling over the dorsum of nose extending from the nasal tip to the nasal bridge with or without intracranial extensions. In some cases, the overlying skin gets rupture and underlying abnormal structures developed from pluripotent cells of dermoid become visible with variable discharges such as cerebrospinal fluid or sequestrum. Origin of nasal dermoid sinus cyst (NDSC) during embryological development requires two necessary and sufficient conditions. One is competence of the ectodermal and meso-ectodermal cells to form dermoid tissue with epithelial-mesenchymal interactions and second, presence of a topographical site of ectodermal inclusion, which fits well with the various clinical presentations of NDSCs. The case presented here has naso-ethmoidal dermoid cyst with ectopic teeth at the tip of the nose. The patient had a cranial defect with bulging of tissue in naso-ethmoidal region that was in line of 1-13 facial cleft of Tessier. Computerized tomography revealed a bony gap in the region of right fronto-ethmoidal region and brain cyst with craniosynostosis in left frontoparietal suture. Transcranial approach was used to manage the case and all the teeth at the tip of the nose were removed.

This Australian study was conducted to understand some of the issues involved where a child is unexpectedly born with a cleft lip and/or palate (CLP). Parents involved in this study had followed their child's development from birth to adulthood having completed all surgery, and each now involved in the community as adults. Because these children were now more than 26 years old, many resources available to parents now were not accessible when these children were young, for example, early ultrasound screening. This study found that there were expected parental concerns for each child, particularly when surgery was imminent. These concerns were minimized by the support provided by the relevant health professionals, as well as CleftPals, an Australian cleft lip and palate support group, together with their immediate and extended families support during the difficult times. [ABSTRACT FROM AUTHOR]

Facial cleft is a morphological disruption or defect of facial structure, a rare and challenging craniofacial malformation. The treatment of rare facial cleft is complex, and evaluating its long-term outcome is challenging because of its low incidence. In case one, a five-month-old boy presented with unilateral facial cleft Tessier 3. In case two, a four-month-old girl presented with bilateral facial cleft Tessier 4. Both were treated with soft tissue reconstruction. Several combinations of sutures were performed to give maximum results, and several surgical steps were conducted to treat facial clefts. A one-step closure procedure of facial clefts could be done and significantly improve the patient and family's quality of life. One-step closure can also close defects as soon as possible to provide psychological support to the family even though the function is not perfect. • Facial cleft is a morphological disruption or defect of facial structure • This craniofacial malformation is challenging because its complex treatment and challenging long-term outcome evaluation caused by its low incidence • Case one showed a boy with unilateral facial cleft Tessier 3, case two showed a girl with bilateral facial cleft Tessier 4 • Soft tissue reconstruction including several combinations of sutures, several surgical steps were conducted • Technique selection is adjusted to patient's condition and no technique is better than the other for facial cleft case [ABSTRACT FROM AUTHOR]

Congenital transverse and oblique facial clefts are rare congenital anomalies, and cooccurence of these is still more a rare anomaly. The condition most commonly is often associated with anomalies of the first and second branchial arches. The manuscript intends to present one such rare case of Tessier's cleft type 3-7 and present its early management as well as long-term treatment plan. [ABSTRACT FROM AUTHOR]

Otocephaly is an extremely rare lethal congenital anomaly characterized by the absence or underdevelopment of the mandible. The clinical presentation is variable. Some cases may present with severe micrognathia as the only anomaly seen prenatally. The key to early diagnosis is careful assessment of the location of the fetal ears on 2D ultrasound examination. [ABSTRACT FROM AUTHOR]

The aim of therapy in patients affected with a cleft in orofacial region is a functional and aesthetic rehabilitation of the soft tissues of the face, defect of the jaw and dentition. The complex therapy is related to the type and extent of the defect, postnatal development and general health of the affected child and requires participation of a team of specialists, while the interdisciplinary care is concentrated to specialized cleft centers. In the Czech Republic there are long-term traditional centers in Prague and Brno. Orthodontic therapy is an integral part of the therapeutic protocol. The following article summarizes the most important phases of orthodontic therapy subsequent to surgical reconstruction of the middle face.

Objective: To examine the accuracy and timing of diagnosis of fetal cleft lip with or without cleft palate (CL ± P) in the years following the introduction of a national screening program, and to assess the completeness and accuracy of information in The Netherlands Perinatal Registry.Methods: A list was obtained of cases with a prenatal or postnatal diagnosis of CL ± P from two fetal medicine units between 2008 and 2012. All cases of CL ± P were included irrespective of the presence or absence of additional anomalies. Cases were included if the estimated date of delivery was between 1 January 2008 and 31 December 2012.Results: During the study period, 330 cases of CL ± P were identified, with a prevalence of 15 per 10 000 pregnancies. The number of cases that were detected before 24 weeks' gestation increased during the study period, while the rate of termination of pregnancy did not change significantly (P = 0.511). CL ± P was isolated in 217 (66%) cases and karyotype was abnormal in 69 (21%) cases. In 5% of the cases in which CL ± P seemed to be isolated during the 18-23-week anomaly scan, postnatal array comparative genomic hybridization (array-CGH) revealed an abnormal karyotype and 50% of these cases had major additional anomalies. Examination of data from The Netherlands Perinatal Registry demonstrated that in 37% of cases CL±P was not recorded in the pregnancy records.Conclusion: CL ± P is increasingly being diagnosed prenatally, without a significant effect on the rate of pregnancy termination. Further improvement in the diagnostic accuracy may be achieved by advocating prenatal array-CGH to reduce the frequency of unexpected anomalies being diagnosed after birth. It is important that healthcare providers register accurately the presence or absence of anomalies in the birth records to ensure that, in the future, data from The Netherlands Perinatal Registry can be relied upon to monitor prevalence. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Objective: To examine the frontal space (FS) distance in first-trimester fetuses with bilateral, unilateral or median cleft lip and palate and in those with retrognathia.Methods: This was a retrospective study using stored two-dimensional ultrasound images of fetal profiles that were recorded at the time of the nuchal translucency (NT) scan at three prenatal medical centers. Images of 300 normal fetuses and 53 fetuses with facial defects were obtained. To measure the FS distance, a line was drawn between the anterior edge of the mental protuberance of the mandible and anterior edge of the maxilla (MM line) and extended upwards in front of the forehead. The perpendicular distance (FS distance) between the MM line and the skin at the point of largest excursion of the fetal forehead was measured. In cases in which the MM line was located anteriorly to the forehead, the distance was measured in the same fashion but was multiplied by -1. Two operators measured the FS distance twice, independently of each other. FS distances were transformed into Z-scores based on the linear relationship with crown-rump length (CRL) in normal fetuses. The distribution of FS distances in fetuses with bilateral, unilateral or median cleft lip and palate and those with retrognathia were compared with that in the normal group using Student's t-test.Results: A search of the centers' databases identified 53 abnormal cases including 20, nine and eight with a bilateral, unilateral and median cleft lip and palate, respectively, and 16 cases of retrognathia. In fetuses with bilateral, unilateral and median clefts and those with retrognathia, median delta NT was 1.00 mm, 0.37 mm, 4.00 mm and 0.26 mm, respectively. Among these affected groups, 12 (60.0%), six (66.7%), two (25.0%) and eight (50.0%) fetuses had an abnormal karyotype. In the normal population, FS distance was dependent on CRL measurement (FS = 6.62 - (0.08 × CRL); r = -0.539; P < 0.0001). In fetuses with a bilateral and median cleft and in those with retrognathia, FS distance was significantly different from that in the normal population (all P < 0.0001), however, the difference was not significant in fetuses with unilateral clefts (P = 0.103). The respective Z-scores of FS distance for fetuses with bilateral, unilateral and median clefts and retrognathia were -9.7 ± 2.0, -3.1 ± 5.1, 8.2 ± 3.4 and -7.3 ± 2.3. Measurements were ≥ 99(th) and ≤ 1(st) centiles in all but one (98.1%) case.Conclusion: The FS distance appears to be a helpful tool in the detection of facial clefts at 11-13 weeks' gestation. Copyright © 2015 ISUOG. Published by John Wiley & Sons Ltd. [ABSTRACT FROM AUTHOR]

Introduction: The incidence of facial cleft is rare and ranges between 1.43 and 4.85 per 100,000 births. To date, there have been few reports of detailed ophthalmologic examinations performed in cases of facial cleft. Here, we report a case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft. Case report: A 9-day-old female infant was delivered by cesarian section at 34 weeks of gestational age (the second baby of twins) and weighed 2,276 g upon presentation. She had a facial cleft and ectrodactyly at birth. Right eye-dominant blepharophimosis was obvious. Examination of the right eye revealed inferior corneal opacity with vascularization, downward corectopia, and optic-nerve hypoplasia. The corneal diameter was 8 mm in both eyes, and tonometry by use of a Tono-Pen® XL (Reichert Technologies, Depew, NY, USA) handheld applanation tonometer revealed that her intraocular pressure was 11-22 mmHg (Oculus Dexter) and 8 mmHg (Oculus Sinister). B-mode echo revealed no differences in axial length between her right and left eyes. When she was 15-16 months old, we attempted to examine her eyes before she underwent plastic surgery under general anesthesia. She had a small optic disc in both eyes and the right-eye disc was tilted. After undergoing canthotomy, gonioscopy and ultrasound biomicroscopy revealed that almost all directions were open except for the peripheral anterior synechia. Since magnetic resonance imaging revealed ventriculomegaly associated with an interhemispheric cyst at birth, a ventriculoperitoneal shunt was inserted at 12 days of age. At 25 months of age, her condition suddenly deteriorated due to occlusion of the ventricular shunt catheter, and she died 5 days later. In this patient, amniotic band syndrome was presumed to be the primary cause due to the clinical findings. Conclusion: We experienced a case of optic-nerve hypoplasia and anterior segment abnormality that occurred with facial cleft. The cause of these abnormalities is unclear, yet amniotic band syndrome is a possible candidate. [ABSTRACT FROM AUTHOR]

We describe a paramedian cleft of the lower lip that cannot be explained by embryological development in a child with only one predisposing factor, which was fetal reduction for a multiple pregnancy. To the best of our knowledge, there has been no report of a cleft that has been induced by the reduction of a multifetal pregnancy. [ABSTRACT FROM AUTHOR]

Background Repair of facial clefts implies wide tissue mobilization with multi-stage surgical treatment. Authors propose pre-surgical orthopedic correction for naso-oro-ocular clefts and a novel surgical option for Tessier No. 3 cleft. Methods Two male infants, a Tessier No. 3 cleft (age 7 months) and another Tessier No. 4 (age 3months), were treated with a modified orthopedic Latham device with additional septo-premaxillary molding and observed to age four years. Tessier No. 3 orthopedic measurements were obtained by image corrected cephalometric analysis. Subsequent repair included tissue expansion on Tessier No. 4 and naso-frontal Rieger flap combined with myocutaneous upper lid flap on Tessier No. 3. Results Orthopedic movements ranged from 18.5 mm in bi-planar to 33 mm in oblique analyses. Tissue margins became aligned with platform normalization. Tissue expansion on Tessier No. 4 improved distances from ala base-lower lid and subalar base-lip. The naso-frontal flap combined with myocutaneous upper lid flap on Tessier No. 3 had similar achievement, but also sufficiently lengthened ala base-canthal distance. Conclusions Repairs were facilitated by pre-surgical orthopedic correction. The naso-frontal flap combined with an upper lid myocutaneous flap seems viable as a single-stage option to lengthen ala base-canthal distance to advance repair achievement in unilateral Tessier No. 3. [ABSTRACT FROM AUTHOR]

In 1976 Dr. Paul Tessier described numeric classification for rare craniofacial clefts. He first emphasized that a fissure of the soft tissue corresponds, as a general rule, to a cleft of the bony structure. The classification, easy to understand, became widely accepted because the recording of the malformations was simple and facilitated communication between observers. The aim of this study was to present our own experience with treatment of patients with rare facial clefts. Material and methods. Our Department has 11 patients with rare craniofacial clefts under its care. This group includes 8 boys and 3 girls. The patients aged from 2 months to 18 years at the time of the first consultation. Results. In two patients the cleft was median, in seven patients it was one-sided and in two - bilateral. The most common type of cleft was number 6, and the rarest were 2, 3, and 7. All patients underwent surgical treatment. Conclusions. Atypical facial clefts are rare congenital anomalies, however because of functional and aesthetic disturbances they constitute a serious medical and therapeutic problem. Facial clefts are characterized by variable clinical presentation and require individualized treatment plan. [ABSTRACT FROM AUTHOR]

Tessier cleft types 3 and 4 are both rare craniofacial anomalies. Here we present the first case of a girl born with a combined anomaly of Tessier clefts 3 and 4 with severe bilateral cleft lip, a displaced premaxilla, and three-dimensional underdevelopment of the hard and soft tissues of the maxilla and zygoma. This type of rare facial cleft poses a major operative challenge. Over a period of years, presurgical alveolar molding with an active appliance was followed by seven operations. A satisfactory esthetic outcome was obtained. A multidisciplinary approach to treatment with a plastic surgeon in charge of the operations and an orthodontist in charge of the cleft deformity is essential. [ABSTRACT FROM AUTHOR]

Here we report a case of a Tessier number 14 cleft, the rarest form of craniofacial cleft, and our step-wise approach to its surgical correction. The patient's appearance was analyzed over a 20-year follow-up period. At the fourth and final operation, the interorbital distance was reduced, the maxilla was advanced, rib and costal cartilage were grafted for augmentation of the nasal bone, and a double eyelid fold operation was performed. Although the surgical results were not entirely satisfactory from an esthetic point of view, this step-wise surgical approach has allowed gradual improvement in the patient's facial appearance. [ABSTRACT FROM AUTHOR]

Parry–Romberg syndrome, also known as progressive hemifacial atrophy, is a rare developmental disorder characterized by progressing unilateral facial atrophy slowly, which may affect the skin, fat, muscle and bone. It can also be associated with different systemic manifestations and deformities. In this article, we present the two cases with Parry–Romberg syndrome. Of them, one has additional and rare facial deformity with rare facial cleft and the other has a special tongue feature. [ABSTRACT FROM AUTHOR]

Résumé: Introduction: Les chronologies opératoires des malformations labio-maxillo-palatines ne font pas l’objet d’un consensus international. Depuis 20ans, nous proposons à nos patients une prise en charge précoce que nous argumentons avec une correction chirurgicale plus immédiate de la malformation de la lèvre qui permet une meilleure intégration du bébé au niveau familial. De récentes études tendent à remettre en cause cette pratique néonatale. Patients et méthode: Nous avons tenu à vérifier lors d’une étude rétrospective, l’innocuité des interventions précoces au niveau anesthésique et chirurgical, ainsi que les résultats obtenus par cette pratique pendant les 20 dernières années pour une série de 42 enfants opérés de la lèvre avant le 28e jour après leur naissance. Tous ont été opérés par le même chirurgien senior. Résultats: Les bébés sont opérés généralement au cinquième jour de vie. Le temps d’intervention chirurgical est de 45minutes pour les fentes unilatérales et de 70minutes pour les atteintes bilatérales. L’alimentation par voie orale est reprise le soir du jour opératoire. La durée d’hospitalisation médiane est de quatre jours. Les résultats nous montrent l’absence de complication anesthésique. Quatre enfants ont bénéficié d’une correction secondaire de la lèvre. Conclusion: Le risque anesthésique et chirurgical des interventions labiales précoces est très limité à condition de pouvoir bénéficier d’un environnement médico-soignant multidisciplinaire, spécialisé dans la prise en charge des enfants opérés en période néonatale et pouvant assurer une surveillance postopératoire adéquate. Le taux de complications est très faible. [ABSTRACT FROM AUTHOR]

Abstract This resource is an interactive educational module depicting human face and palate development. The module was created to augment embryology instruction in medical and dental curricula. Three dimensional visualization of morphogenesis during embryonic development is a difficult task, and this module was designed to alleviate that difficulty. Students access the module after a traditional didactic instruction on facial development. They should spend approximately half an hour going through the material and consolidating the instructor's content with the 3D imaging. The efficacy of the materials was assessed using first-year medical and dental students. Although the student groups were small (15–20 students), the materials tended to enhance student learning, as indicated by pre- and post-testing. The resource was found to be advantageous when used in conjunction with lecture or small-group instruction. No assessment of the module's impact as a stand-alone educational tool has been undertaken. The module should be considered a limited tool for understanding 3D tissue movements and interactions during facial development. Further instruction is required to gain a thorough knowledge of facial development. Expansion and improvement of the resource are planned to incorporate earlier developmental time points and more detailed features of development, such as tooth and cranial nerve development.

Abstract: We present prenatal diagnosis and molecular cytogenetic characterization of de novo interstitial deletion of 7q (7q22.1→q31.1) by aCGH, FISH and QF-PCR in a fetus with an abnormal maternal serum screening result and ultrasound findings of facial cleft and hypogenitalism. We discuss the genotype–phenotype correlation and the consequence of haploinsufficiency of ZKSCAN5, ARPC1A, CYP3A43, RELN, LAMB1, IMMP2L and DOCK4 in this case. [Copyright &y& Elsevier]

Background: Congenital anterior staphyloma is a rare, complex malformation syndrome of the anterior segment. Only a few reports on associated systemic malformations have been published. We herein present a rare manifestation of congenital anterior staphyloma (CAS) combined with amniotic band disruption syndrome (ABS). Patient and methods: Shortly after birth, a massive enlargement of the left eye was observed in a female child. Furthermore, an extensive bilateral congenital cleft lip and cleft alveolar ridge with oblique facial cleft extending into the left medial canthal region, coloboma(s) of the left eyelids, extensive adhesions between lids and eye bulb, as well as circumferential grooves, clubfeet, and terminal transverse defects in both hands and feet were present. Due to severe progression of eye bulb protrusion with thinning of the sclera, enucleation of the left eye was performed at the age of 3 years in order to prevent complications including perforation of the globe and with the aim of improving cosmetic aspects. Results: Histopathological examination of the enucleated eye disclosed findings typical of congenital anterior staphyloma, including massive corneal staphylomatic deformation with superficial vascularization and elapsed corneoscleral margin, destruction of Bowman's layer, absence of Descemet's layer, corneal endothelium, and angle structures. The lens was only partially formed, and had mainly dissolved. The neural retina appeared normal. The optic nerve disc revealed a pronounced excavation. Facial clefts, lid colobomas, congenital constriction bands, and amputation of distal limbs match ABS. This malformation complex develops in early pregnancy, probably prior to 35 days post conception. Conclusion: This is the first report on an association of these two rare complex congenital malformations, congenital anterior staphyloma and amniotic band syndrome. The anterior staphyloma was unilateral, and related to facial clefts and lid coloboma in the area adjacent to the anterior staphyloma. Furthermore, the systemic deformities are clearly due to the amniotic bands, and the timing of the development of both complex malformations seems to be similar. All findings suggest that the presence of amniotic bands is a causative factor for all observed abnormalities including anterior staphyloma. [ABSTRACT FROM AUTHOR]

Abstract: Tessier clefts type 3 and 4 are rare. In this paper the authors report on the management of a wide Tessier 3 cleft. There is no standardized protocol or timing of the surgical procedures in this rare disfiguring condition. Generally speaking, the aim is to preserve the function of important anatomical structures (e.g., a seeing eye.) and reconstruct, as best as possible, harmonic facial features. The authors present a “step by step” solution of the malformation pointing out the limitations of the surgical procedures they used and the goals they wanted to obtain. Despite of the uniqueness and the complexity of the pathology, the authors think they obtained reasonable results both in term of function and aesthetics, permitting the patient to be accepted in the social environment. [Copyright &y& Elsevier]

This article presents two cases of amniotic band syndrome associated with orofacial deformities. There was an uncommon presentation in one of them as Tessier nos. 5, 7 and 30 with irregularly distributed amniotic facial band. The surgical repair of the facial clefts and difficulties in overall management in our environment are further discussed. [ABSTRACT FROM AUTHOR]

INTRODUCTION: Midface hypoplasia is a skeletal defect involving all three space planes, hence needing a three-dimensional repositioning. This research study shows two cases of cranio-facial dysostosis, on which two Le Fort III variants were performed. CASE REPORT: In the first case we report the performing the two types of osteotomy simultaneously. Le Fort I, however, has been performed without any pterygomaxillary disjunctions, thus accomplishing a complete midface mibilization without any variation of the occlusal ratios. In the second case a modified Le Fort III osteotomy has been performed with median disjunction; reduction of the transverse interdacryal diameters and of the pyriform opening. DISCUSSION: Among all osteotomic variants we would like to mention the one introduced by Obwegeser in 1969 where, in patients with acceptable dental occlusal ratios, Le Fort III and Le Fort I have been performed in conjunction. This technique allows a different midface and dental occlusion repositioning. CONCLUSIONS: In adult patients with permanent dentition and normal occlusal ratios -- this technique may be chosen for a midface advancement without compromising the dento-skeletal relations, in order to achieve the best functional and aestetical results. [ABSTRACT FROM AUTHOR]

Abstract: A 14 month old, 19 kg female infant with aminotic band syndrome and severe craniofacial malformations presented for placement of an open gastrostomy tube. Spontaneous ventilation was maintained while an “awake look” was performed with the video laryngoscope using intravenous propofol (30 mg total) for sedation. A Grade I Cormack-Lehane score was obtained on the monitor. Succinylcholine was then administered intravenously and orotracheal intubation was performed on the first attempt with a size 4.5 endotracheal tube. [Copyright &y& Elsevier]

Copyright of La Radiologia Medica is the property of Springer Nature and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Facial clefts are rare congenital malformations. In the literature these are sometimes reported in combination with limb malformations, especially ring constrictions. This article describes three children with facial clefts and limb ring constrictions with various expressions. The first case has a lateral cleft with associated limb malformations. This combination has, to our knowledge, not yet been reported. The literature about facial clefting and the amniotic band syndrome and the possible etiology of clefting and constrictions in these cases are discussed. [ABSTRACT FROM AUTHOR]

Abstract: Smad7 is a negative regulator of TGFβ superfamily signaling. Using a three-component triple transgenic system, expression of the inhibitory Smad7 was induced via doxycycline within the NCC lineages at pre- and post-migratory stages. Consistent with its role in negatively regulating both TGFβ and BMP signaling in vitro, induction of Smad7 within the NCC significantly suppressed phosphorylation levels of both Smad1/5/8 and Smad2/3 in vivo, resulting in subsequent loss of NCC-derived craniofacial, pharyngeal and cardiac OFT cushion cells. At the cellular level, increased cell death was observed in pharyngeal arches. However, cell proliferation and NCC-derived smooth muscle differentiation were unaltered. NCC lineage mapping demonstrated that cardiac NCC emigration and initial migration were not affected, but subsequent colonization of the OFT was significantly reduced. Induction of Smad7 in post-migratory NCC resulted in interventricular septal chamber septation defects, suggesting that TGFβ superfamily signaling is also essential for cardiac NCC at post-migratory stages to govern normal cardiac development. Taken together, the data illustrate that tightly regulated TGFβ superfamily signaling plays an essential role during craniofacial and cardiac NCC colonization and cell survival in vivo. [ABSTRACT FROM AUTHOR]

Summary: Absence of half-nose is an extremely rare congenital malformation, which has a devastating impact on the patient and the family. A review of indexed English-language literature found 91 cases of half-nose, including 50 patients with proboscis lateralis. Pathogenesis is not clear, and the reported cases have sporadically occurred. Many aspects must be considered when reconstructing a congenital half-nose, such as timing of surgery, type of tissue to be used and the need to reconstruct nasal airway. The aim of this article is to present personal experience in seven cases of half-nose reconstruction, in order to review the literature regarding to this rare entity, highlighting aspects of incidence, pathogenesis and surgical treatment. Nasal reconstruction was performed at ages of 5–7 years to minimise psychological trauma. Forehead skin demonstrated to be an excellent donor site to re-surface the nose. For the inner lining, contralateral cutaneous nasal flap was our preference. Concerning the nasal framework reconstruction, alar contour was restored using a cartilage graft from the lower portion of ear tragus and concha. [Copyright &y& Elsevier]

Abstract: The birth of a child with a facial cleft is often seen as a very stressing event for both parents. Risks of comorbidity, difficulty in nutrition or just aesthetical reasons are frequently invoked while describing this moment. The announcement of the deformation is felt like a brutal intrusion in representations associated to pregnancy. Then, we can observe a kind of disinvestment of the coming baby. Through our study, we find that high stress has not always a bad issue on relationship between parents and infants. In contrary, this kind of chronic stress can preserve the well-being of the relationship, especially for fatherhood. The time of facial deformation announcement also plays its part in representations about the baby. Before birth, it opens room for reordering and modifying representations, then the baby will be accepted as it should be on his birthday. [Copyright &y& Elsevier]

The Tessier no.5 facial cleft is an extremely rare congenital malformation. Onl y 26 cases have been described in the English-language literature. The cleft begins in the upper lip just medial to the oral commissure, extending across the cheek as a groove ending at the junction of the middle and lateral thirds of the lower eyelid. The bone involvement usually includes an alveolar cleft in the premolar region, extends across the maxilla lateral to the infraorbital nerve, up to the infraorbital rim and orbital floor. The goals of the surgical procedure include reconstructing the lower eyelid, repositioning the lateral canthus, closure of the labiomaxillary cleft, and restoration of the skeletal continuity (including the orbital floor defect) with bone grafts. We present six patients with the Tessier no. 5 facial cleft who have been treated in our combined centers and discuss the surgical options and difficulties faced in the reconstruction of this rare and challenging craniofacial malformation.To date, we have treated six patients (two with bilateral and four with unilateral clefts).Three of the patients with unilateral clefting had an associated no.4 cleft and one patient with a bilateral cleft had an associated no.3 cleft. This paper represents the largest series to date documenting surgery for patients with the Tessier no.5 facial cleft. [ABSTRACT FROM AUTHOR]

Facial clefts and Robin sequence (RS) share the timing of the diagnosis during the course of pregnancy, their association with genetic diseases and the subsequent management following the initial diagnosis. If a suspicion of a facial cleft or RS is made, a detailed anatomical examination of the fetus should be carried out to identify further anomalies. This may also involve genetic testing including a microarray or an exome analysis. Interdisciplinary counseling, including pre- and postnatal experts with sufficient experience in the management of such neonates, should be involved in this counseling. Parents should be informed about disease-specific therapeutic options and postnatal outcome. Delivery should take place in a center with experience in craniofacial malformations where clinicians are prepared for potentially life-threatening airway obstruction immediately after birth. [ABSTRACT FROM AUTHOR]

Choanal atresia is one of the most commonly observed congenital abnormalities of the nose. This defect is frequently associated with other congenital anomalies. One of the anomalies, which is rarely encountered is a Tessier’s facial cleft 2. In this report, we present a 6-year-old girl who was brought to our department with complaints of nasal obstruction and chronic nasal discharge. On examination, a scar was seen on the left ala of her nose due to the former repair of a congenital defect. We found hypoplastic nasal cavities and bilateral choanal atresia demonstrated by rigid and flexible endoscopy. A CT scan supported these findings. This coexistence of bilateral choanal atresia with a number 2 facial cleft is an addition to a formerly proposed classification of laterofacial clefts. We think that this classification is better for describing the extent and origin of these complex lesions. [Copyright &y& Elsevier]

A 3-year-old boy with bilateral congenital macrostomia as an isolated clinical entity is presented. Although this is a rare deformity, it causes functional and cosmetic problems which are difficult to correct. The skin, muscle, and mucosal components of the deformity were separately repaired using a modification of Kaplan's technique. [ABSTRACT FROM AUTHOR]

Copyright of Medicina Oral (16656024) is the property of Colegio Nacional de Cirujanos Dentistas Militares A.C. and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)