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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]