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The Tessier Number 4 cleft is one of the rarest, most complex craniofacial anomalies that presents difficulties in surgical treatment. In this article, we report a case of simultaneous facial depression, eye displacement, and medial canthus deformity. In this case, the maxillary bony defect was reconstructed using computer-assisted design computer-assisted manufacturing (CAD-CAM) polyether-ether-ketone (PEEK) material, and the orbital floor defect was repaired with AO prefabricated titanium mesh. Additionally, the medial canthus was modified with canthopexy and a single Z-plasty flap. Owing to its relative rarity and varied clinical presentations, no definitive operative methods have been accepted for Tessier No. 4 facial cleft. This study presents the combination of CAD-CAM manufactured PEEK material and titanium mesh as an alternative approach for reconstructing the bony defect of Tessier No. 4 facial clefts.

Aim To explore a new accurate way for the treatment of congenital midfacial dysplasia in facial cleft patients. Materials and methods Between November 2015 and November 2016, 8 patients with nasal deformity and midfacial dysplasia (Tessier Nos. 3-11 cleft) were collected (median age, years; range = 15-20 years). Expanded frontal flap for nasal reconstruction and image-guided navigation-assisted surgery for modified nasal-maxillary-hard palatine osteotomy to advance the peri-pyriform bone structure were performed in all the patients. After 6 to 12 months of follow-up, the authors analyzed the differences between preoperative planning and postoperative results through computed tomography data. Results Patients were satisfied with surgery, and computed tomography data showed that there was little difference between preoperative planning and postoperative results with the navigation-assisted surgery. Conclusion Using expanded frontal flap with navigation-assisted surgery for peri-pyriform advancement, the authors could treat congenital nasal deformity and midfacial dysplasia effectively, accurately, and safely in craniofacial cleft patients.

Of all the craniofacial abnormalities, facial clefts are the most disfiguring. Facial clefts are classified according to the affected anatomical area as described by Tessier. Through this classification, the location and extent of the cleft can be designated numerically.A 2-month-old male infant was referred to authors' craniofacial unit, from a hospital in a rural province of South Africa, with a problem of a supranasal encephalocoele. Bilateral raised eyebrows were noted as was a right-sided upper lid central third coloboma. Computed tomography and magnetic resonance imaging scans confirmed the presence of a supranasal encephalocoele with a large frontal bone defect and splayed nasal bones. Bilateral enlarged orbits were noted with tented orbital roofs that we classified as Tessier number 10 facial clefts. The child was booked for an encephalocoele excision and calvarial reconstruction at 4 months of age.As a result of the encephalocoele, the supraorbital bar with its adjacent nasal bones was cleaved in 2, resulting in a significant frontal bone defect. Osteotomies were performed to remove the supraorbital bar and nasal bones from the calvarium. The supraorbital bar segment was remodeled and plated with absorbable poly-L-lactic acid plates. Osteotomies of the nasal bones allowed them to be united centrally, also with absorbable plates. This entire construct was transferred and secured to the calvarium, but in a more caudal position thereby obliterating the frontal bone and Tessier number 10 facial cleft defects with a naturally contoured construct.

INTRODUCTION Oblique facial clefts are extremely rare and cause significant morbidity. Treatment of these clefts is complex and requires a fundamental understanding of cleft classification and techniques used for treatment of clefts. METHODS We describe a novel single-staged technique to repair the Tessier no. 4 soft tissue cleft and reconstruct the buccal sulcus and bilaminar lower eyelid by preserving normally excised tissue combined with standard procedures. We also present a case report demonstrating the technique in an adolescent female. The procedure incorporates turnover flaps from soft tissue preservation within the cleft, a Mustarde cheek advancement flap, an anatomical subunit lip repair, a dorsal nasal Rieger flap for ala repositioning, and a lateral nasal flag flap. RESULTS The single-staged soft tissue repair eliminated the Tessier no. 4 cleft while simultaneously reconstructing the bilaminar lower eyelid and buccal lining. Our patient had no complications within the perioperative period. CONCLUSIONS This novel single-staged technique for the treatment of the soft tissue Tessier no. 4 cleft not only repairs the cleft but also reconstructs the buccal sulcus and bilaminar lower eyelid with turnover flaps preserved from the normally discarded excess soft tissue within the cleft. The novel repair allows for the creation of a deeper fornix to aid with placement of an orbital prosthesis and is ideal for use in underserved or remote locations.

Frontonasal dysplasia is a rare entity. It has characteristic physical deformities: hypertelorism, broad nasal root, median facial cleft of the upper lip or palate, clefting of the nasal alae, poorly formed nasal tip, cranium bifidum occultum, and a widow’s peak hairline. Fibrous dysplasia is a benign bone tumor in which normal bone is replaced by fibrous, poorly formed osseus tissues. We present a patient with frontonasal dysplasia who desired correction of her hypertelorism. Incidentally, fibrous dysplasia was found in her left orbit complicating surgical correction. In addition, the patient has velopharyngeal insufficiency and a class III malocclusion. The interplay of all these craniofacial defects makes the sequencing and timing of surgery important in this unique patient.

PURPOSE Facial asymmetry can be produced by congenital or acquired factors, and surgical correction is one of the very important and difficult fields in plastic surgery. Zygoma plays a key role to keep facial symmetry, and diverse methods have been tried to correct asymmetric face. We used the malarplasty with tripod osteotomy to correct nontraumatic asymmetric face. METHOD From December 2008 to February 2010, 3 patients presenting with facial asymmetry and enophthalmia were selected, and their average age was 18 years. One of the patients has undergone surgeries 4 times without improvement for rare facial cleft no. 4 from other hospitals. The other 2 cases had hypoplastic zygoma complex caused by early enucleation and radiation therapies because of retinoblastoma in their childhood. Through a bicoronal approach, malarplasty and calvarial bone graft were done after tripod osteotomy including the zygomaticofrontal suture, zygomatic arch, and zygoma body. RESULTS The surgery results were evaluated by radiographic inspection and the photographs of the patients. Differences in preoperative heights of both normal and hypoplastic zygoma-orbit complex in Waters and zygomatic view were estimated. After corrective operation, the height of the zygoma complex was almost close to normal height, and orbital volume was increased, and ectropion was corrected at the same time in patients. CONCLUSIONS Using corrective malarplasty with tripod osteotomy, we achieved satisfactory results of zygoma expansion and improvement in orbital area. Therefore, malarplasty via tripod osteotomy cannot be applied only on the patients with traumatic zygoma deformation, but also on the patients with nontraumatic facial asymmetry.

A case involving concomitant presentation of a #7 lateral facial cleft with a complete cleft of the ipsilateral lip, alveolus, and palate is presented. The mandibular defect was Pruzansky III with a foreshortened body, absent ramus and absent masseter. Taking advantage of developmental field theory, reconstruction of the osseous defect was undertaken using the autogenous periosteum as a source of mesenchymal stem cells. Expansion of the periosteum was followed by implantation of Helistat (Integra Life Sciences, Plainsboro, NJ) collagen sponge saturated with recombinant human bone morphogenetic protein-2. Stimulation of this distraction-induced envelope by rhBMP-2 resulted in abundant production of bicortical membranous bone in situ within 12 weeks. The neoramus was subsequently suspended from the cranial base, and a temporalis muscle transfer was used to provide motor control of the jaw. Synthesis of bone in this manner is termed DISO (distraction-assisted in situ osteogenesis). The biologic rationale and clinical implications of DISO are discussed.

The effect of maxillary advancement on speech may have benefits on articulation improvement but compromises velopharyngeal (VP) closure by increasing the nasopharyngeal distance. The purpose of this study was to evaluate the static VP anatomic changes on lateral cephalograms in patients who underwent maxillary advancement through distraction osteogenesis (DO) with a rigid external distraction device and to correlate these changes with clinical speech data. Twenty-two patients (5 female and 17 male) underwent maxillary advancement through DO utilizing a rigid external distraction device (age, 5.2 to 25.7 years) with various diagnoses, including 13 unilateral cleft lip and palate (CLP) patients, 5 bilateral CLP patients, 1 isolated cleft palate patient, 2 facial cleft patients, and 1 patient with craniosynostosis. Lateral cephalograms of preoperative, immediate postdistraction, and 1-year postdistraction were obtained for analysis. Speech evaluation was performed preoperatively, immediate postdistraction, and then at 6-month intervals, and included assessment of air pressure flow, hypernasality, and articulation. With an average amount of 8.9 mm maxillary forward advancement, 14% of patients (3 of 21) presented deterioration in hypernasality. However, 57% of patients (12 of 21) demonstrated improvement in articulation. The cephalometric analysis demonstrated an increase in nasopharyngeal depth by 8.5 mm (1:1 ratio with bony movement) and velar angle by 14.1 deg. The length of the soft palate remained unchanged. The need ratio (intersection of palatal plane and posterior pharyngeal wall-posterior nasal spine/posterior nasal spine--tip of uvula) worsens after distraction. The deterioration of hypernasality was related to the amount of forward distraction, especially in patients without a preexisting pharyngeal flap (PF). Speech evaluation is an important aspect concerning treatment planning for maxillary distraction. The increase in nasopharyngeal depth may compromise VP closure. The increase in velar angle was considered to be part of the compensation in the VP mechanism. An adverse effect of a preexisting PF on maxillary distraction was not observed; however, it prevented postoperative hypernasality.

The etiopathogenesis behind the formation of atypical craniofacial facial clefts remains unknown. To test the hypothesis that physical restricting forces such as amniotic bands can lead to the formation of these unusual clefts in the postorganogenesis period, we have modified a previously reported fetal lamb model of amniotic band syndrome to examine the effects of these bands on craniofacial development. Five 70-day gestation fetal lambs (term, 140 days) were exposed via a maternal hysterotomy. In each animal, an attempt was made to create a lateral craniofacial cleft by applying a 2-0 nylon suture as a constriction band to the growing face. The sutures were attached to either the zygomatic arch or the infraorbital rim externally and then looped circumferentially into the oral commissure. Each suture was positioned so as to create either a Tessier type 5 or a Tessier type 7 cleft. Four of five fetal lambs survived to term. Both types of lateral facial clefts were effectively produced using this model. In each group, the presence of an intraoral constriction band led to the formation of macrostomia, with an average 7.4-mm lateral displacement of the oral commissure. In addition to these soft tissue changes, each animal also had partial bony clefting (i.e., a bony groove) induced by the pressure of the restriction band across the growing facial skeleton. In the two lambs with the Tessier type 7 cleft, incomplete bony clefts developed across the zygomatic arch. In three animals with bands placed across the medial infraorbital rim, significant infraorbital and malar bony clefts formed similar to a classic Tessier type 5 facial cleft. No evidence of tissue necrosis, maceration, or ulceration was noted in any animal. These data present, for the first time, evidence that the constriction of craniofacial growth by external forces such as a swallowed amnionic band can lead to the development of lateral facial clefting involving both soft tissue and bony elements. These malformations are likely due to a combination of directly tethering normal tissue migration and an increase in local pressure, which produces cellular ischemia and apoptosis. Furthermore, our data demonstrate that these clefts can occur later in fetal development during a period of facial growth rather than during the period of primary facial morphogenesis.

Midline clefts of the lower lip, mandible, tongue, and neck are rare congenital deformities and are classified as facial cleft no. 30 by Tessier. This is a report of a patient who presented with congenital fibrotic neck cord but no lower lip cleft, which has been the most frequently observed pathognomonic sign of this anomaly. The patient was treated initially for thyroglossal remnant. In the patients who present with a fibrotic cord located at the cervical midline without a lower lip cleft, the diagnosis of facial cleft no. 30 should be kept in mind.