Cardiovascular anomalies in patients with Tessier syndrome: a systematic review.

Tessier clefts are skeletal and soft tissue abnormalities of a neonate's facial structures. They could be classified as syndromic and non-syndromic clefts, which can be attributed to disruptions in fetal development and genetic mutations, respectively. Reported cases of these clefts typically document the presence of additional abnormalities associated with these clefts. In this systematic review, we analyzed reports of Tessier clefts accompanied by cardiovascular anomalies, as one of the commonly encountered anomalies. We systematically searched PubMed (MEDLINE), Scopus, Web of Science, Science Direct, and Google Scholar. We selected and included case reports, case series, and case reviews on patients with Tessier cleft and cardiovascular anomalies. The critical appraisal of the included studies was performed by two independent investigators using the Consensus-based Clinical Case Reporting Guideline Development (CARE) checklist. Overall, 20 reports (18 case reports and 2 case series) were eligible for inclusion in this review. Tessier clefts 3 and 30 were the most commonly observed. In addition, the most prevalent cardiovascular anomalies consisted of the ventricular septal defect (VSD), double-outlet right ventricle, and atrial septal defect (ASD). Most of the patients received cosmetic and cardiovascular surgeries. However, some were not proper candidates for cardiovascular surgery because of their unstable condition and therefore did not survive.   Conclusion: Regardless of the focus placed on the cleft and subsequent plastic surgery procedures in these cases, it is important to prioritize other abnormalities that may be associated with mortality. A complete cardiovascular system and associated disorders assessment should be performed before facial cosmetic surgeries. What is Known: • Tessier clefts are congenital defects in the soft tissues and bones of the face and like many other congenital defects, they are accompanied by defects in other parts of the body. • In the current literature, the emphasis is on clefts and the cosmetic issues rather than the coinciding defects, particularly cardiovascular anomalies. What is New: • Review the cardiovascular anomalies that are commonly encountered in patients with Tessier clefts.

Tessier Number 9 Craniofacial Cleft Associated with Goldenhar Syndrome and Its Surgical Management: A Report of a Rare Case.

Purpose: To report surgical repair of a rare case of Tessier number 9 craniofacial cleft. Methods: Case report. Results: Tessier number 9 craniofacial cleft is the rarest cleft anomaly. This article reports a congenital eyelid coloboma in a 21-year-old woman that involved the lateral third of the left upper eyelid and extended to the lateral canthus, consistent with number 9 craniofacial cleft Tessier classification. The additional findings included a fibrotic band between the globe and the remnant of the upper lid, which caused a small-angle exotropia. There were also skin appendages in the preauricular area and the inner surface of the nasal columella consistent with Goldenhar syndrome. The eyelid coloboma was repaired by releasing the adhesions and using a composite graft of the hard palate to repair the posterior lamella. The anterior lamella was repaired by creating a skin advancement flap. The esthetic and functional outcomes were acceptable in the 2-year postoperative follow-up period. Conclusion: The composite hard palate graft can be used to repair posterior lamella defect in the case of Tessier number 9 craniofacial cleft.

To the lip and beyond: A case report of a midline tessier 30 Cleft

Objective@#To present the case of a midline Tessier 30 cleft in a baby boy who initially underwent a glossoplasty, cheiloplasty and mentoplasty. @*Methods@#Design: Case Report. Setting: Tertiary Government Training Hospital. Patient: One. @*Result@#A 4-month-old boy with a complete midline cleft of the lower lip, alveolus and mandible, and bifid distal tongue that was fused with the floor of the mouth, underwent glossoplasty, cheiloplasty and mentoplasty with subsequent excellent aesthetic outcome and normal oral competency. @*Conclusion@#Tessier 30 is a rare congenital midline mandibular cleft. Prompt glossoplasty, cheiloplasty and mentoplasty can correct the gross deformity, restore oral competency, and address functional needs such as feeding, swallowing and early speech development. Future bony repair will hopefully complete the reconstruction.

Management of median and paramedian craniofacial clefts.

BACKGROUND: Median and paramedian craniofacial clefts are associated with hypertelorism, anterior encephalocele, positional abnormalities of the maxilla, and nasal deformity. Cleft lip and palate, eyelid coloboma, and widow's peak are frequently present. METHODS: The authors collected data from 30 patients (mean age, 5.8 years; range, 4 months to 18 years) operated between 1986 and 2017 with median or paramedian craniofacial clefts of differing degrees of severity. Malformations of the different anatomic units and their surgical treatment were assessed, as well as complication rates. RESULTS: All patients presented nasal malformations and either telecanthus (n = 16) or hypertelorism (n = 14). Most patients (n = 23) had anterior encephalocele. All patients underwent nasal corrections, and most of them had medial canthopexy (n = 24). Excision of encephalocele was associated with fronto-orbital remodeling. Medialization of the orbits was performed in 11 patients, mainly by box shift (n = 9). Patients from outside Switzerland (n = 23) were operated at an older age than those in the native patient group. Because of staged reconstruction, 13 patients had more than one operation. Surgical complications included three infections and one expander exposition. One patient had bone resorption of a frontal bone flap. Nasal correction needed more than one procedure in 5 patients, and medial canthopexy had to be repeated in 7 patients. Esthetic results were satisfactory, permitting social integration. CONCLUSION: Median and paramedian craniofacial clefts need adapted and carefully planned corrections respecting the growth of anatomic units. The quality of the medial canthal and nasal reconstruction is to a large extent responsible for the overall result.

The Median Mandibular Symphysis Tessier 30 Cleft:: 3D Imaging Analysis and Review of the Literature.

Atypical craniofacial clefts of the upper facial region have been well documented; however, the mandibular clefts remain rare and reported as isolated case reports. We report a case of a median mandibular cleft within the context of a Tessier 0-14 axis that we have followed over a 5-year period without surgical/orthodontic intervention. The mandibular symphysis cleft remained open without evidence of the fusion, in contrast to ossification of the metopic dysraphism. Within this context, we present a review of the median mandibular cleft cases from 1819 to 2015.

Report of an unsual case of anophthalmia and craniofacial cleft in a newborn with Toxoplasma gondii congenital infection.

BACKGROUND: We present one unusual case of anophthalmia and craniofacial cleft, probably due to congenital toxoplasmosis only. CASE PRESENTATION: A two-month-old male had a twin in utero who disappeared between the 7th and the 14th week of gestation. At birth, the baby presented anophthalmia and craniofacial cleft, and no sign compatible with genetic or exposition/deficiency problems, like the Wolf-Hirschhorn syndrome or maternal vitamin A deficiency. Congenital toxoplasmosis was confirmed by the presence of IgM abs and IgG neo-antibodies in western blot, as well as by real time PCR in blood. CMV infection was also discarded by PCR and IgM negative results. Structures suggestive of T. gondii pseudocysts were observed in a biopsy taken during the first functional/esthetic surgery. CONCLUSIONS: We conclude that this is a rare case of anophthalmia combined with craniofacial cleft due to congenital toxoplasmosis, that must be considered by physicians. This has not been reported before.

An unusual craniofacial cleft: amniotic band syndrome as a possible cause.

We report the case of a no. 4 Tessier cleft in association with an unknown cleft of the mandible extending to the external auditory meatus. This has not been previously published in the literature and its underlying pathology remains undetermined. The nature of the cleft, possible classifications, and potential embryologic origins will be discussed. Amniotic band syndrome is the most likely cause of the cleft.

A case series of Tessier 3, 4, 7 and combined 4, 7 craniofacial clefts

@#<p style="text-align: justify;"><strong>OBJECTIVE:</strong> To report a case series of Tessier 3, 4, 7 and combined 4,7 craniofacial clefts, their clinical presentations, surgical approaches and outcomes in light of the current literature.<br /><strong>METHODS:</strong><br /><strong>Design:</strong> Case series<br /><strong>Setting:</strong> Tertiary Government Hospital<br /><strong>Subjects:</strong> Five patients<br /><strong>RESULTS:</strong> Five  patients aged 3 to 14-years-old with Tessier 3, 4 (2  cases), 7  and combined 4,7 were included in this study: Tessier 3 - medial orbitomaxillary cleft extending through the bony skeleton traversing obliquely across the lacrimal groove, Tessier 4 - median orbitomaxillary cleft traversing  vertically  through  the  inferior  eyelid,  infraorbital rim and  orbital  floor extending  to the lip between the philtral crest and the oral commissure  (2 cases), Tessier 7 - macrostomia and cleft oral commissure and combined Tessier 4 and 7, combining features described above. Four underwent 2- or 3-stage surgeries while one declined.<br /><strong>CONCLUSION:</strong> Five  craniofacial  clefts  were  presented.  Because  of  the  varying  patterns of craniofacial  deformities,  a  series of surgical  procedures,  tailor-made  for  each  individual  were performed  on  four. Otolaryngologists who perform maxillofacial and cosmetic surgery  should have good background knowledge about craniofacial defects and be familiar with the  surgical approaches at their disposal to yield favorable results that are appropriate to their local contexts.</p>

Africa has unique and urgent barriers to cleft care: lessons from practitioners at the Pan-African Congress on Cleft Lip and Palate.

BACKGROUND: The goals of this study were to delineate the protocols employed for managing patients with cleft lip and palate deformities, delineate the challenges facing practitioners and patients, and to determine the patient and physician barriers to cleft care delivery in the region. METHODS: Survey questionnaires were administered to practitioners attending the second Pan-African Congress on Cleft Lip and Palate (PACCLIP), which took place in Ibadan, Nigeria, West Africa from February 4-7, 2007. The conference included 225 participants, representing 17 African countries RESULTS: Protocols for repair of cleft lip and palate deformities were varied, with Millard's and von Langenbeck's techniques being the preferred approach for the management of cleft lip and palate deformities, respectively. A large proportion of providers have limited access to core cleft care supporting teams, especially speech language pathologists, orthodontists, and audiologists. Several challenging barriers to cleft care were also identified at both the institutional and individual levels and are reported. CONCLUSION: Geographic separation in Africa presents a similar challenge due to isolationism as it does to surgeons in Europe. Specific to Africa are the increased barriers to care, and economic and financial hardship at various levels. A focus on funding, team building, infrastructural support, and patient education appear to be crucial in improving the care and lives of children with facial clefts in Africa.

Surgical Correction of a Median Cleft of the Upper Lip Associated with Enlarged Frenulum and Palatal Masses

PURPOSE: Median cleft of upper lip is defined as any congenital vertical cleft through the midline of the upper lip. It is uncommon, its embryological pathogenesis remains unexplained to date. The authors hereby report a rare case of median cleft of the upper lip associated with enlarged frenulum and palatal mass. This case offers some understanding of the possible embryologic development of this anomaly. METHODS: A 10-month-old boy born by normal vaginal delivery at full-term had a notch in the midline of the upper lip with widened philtrum along with enlarged median frenulum, alveolar cleft, and mass of the hard palate. We performed en bloc resection of the enlarged frenulum and palatal mass and cheiloplasty under general anesthesia. RESULTS: Histological examination revealed that the frenulum and palatal mass was consisted of fibrous tissue with normal mucous membrane. The postoperative course was satisfactory. CONCLUSION: A rare case of median cleft of the upper lip with associated enlarged frenulum and palatal mass was presented with proper surgical management. The surgical technique includes marginal excision of the clefted epithelium and reconstruction of orbicularis oris muscle, in addition to en bloc resection of the palatal mass and frenulotomy.

Tessier No. 2 Oblique Facial Cleft Not Associated with Cleft Lip or Palate: a Case Report

Surgical Repair of Canalicular Defects and Congenital Eyelid Colobomas Associated with Tessier No. 3 Cleft

PURPOSE: To report a single case of surgical repair of the canalicular defects and congenital eyelid colobomas associated with Tessier No. 3 craniofacial cleft. CASE SUMMARY: A one-month-old girl presented with eyelid colobomas and discharges from the eyes. The patient was diagnosed with a Tessier No. 3 craniofacial cleft with bilateral lower eyelid colobomas medial to the puncta. At the age of 55 months, examination under general anesthesia revealed mid-canalicular obstructions in both lower canaliculi. After pentagonal excision of eyelid colobomas in the left upper and both lower eyelids, both ends of the canaliculi were found at the cut edge of the lower eyelids. After the repair of canalicular defects and bilateral nasolacrimal duct silicone tube intubation, the primary closure of the eyelid defect was performed layer by layer. Although there was no subjective improvement of epiphora in the left eye, a subjective improvement of epiphora in the right eye was achieved, and tear meniscus height in the right eye was halved. Additionally, the eyelid colobomas were cosmetically well repaired at postoperative 6 weeks. The patient still had mild tearing symptoms, but did not complain any longer of discharge at postoperative 4 months. CONCLUSIONS: Tessier No. 3 craniofacial cleft with eyelid colobomas can be associated with canalicular defects and nasolacrimal duct obstructions. Surgical repair of the canalicular defects associated with eyelid colobomas should be considered to achieve a functional recovery of the lacrimal drainage system.

Congenital Symblepharon Associated with No. 3 Craniofacial Cleft

PURPOSE: To report a case of congenital symblepharon associated with No. 3 craniofacial cleft. METHODS: A 40-month old girl with left eyelid lesion underwent an ophthalmologic examination at the department of ophthalmology and a face examination at the department of plastic and reconstructive surgery. RESULTS: Congenital symblepharon associated with No. 3 craniofacial cleft including bifid uvula, partial cleft lip, and coloboma of the lower medial eyelid was found. She did not have amblyopia or strabismus. A MEDLINE search of the literature revealed only one reported case of symblepharon associated with a craniofacial cleft.

Median and Paramedian Craniofacial Cleft

Craniofacial clefts are rare, mysterious and variable congenital anomalies. The incidence of craniofacial clefts is at least 1 per 100,000 live births. The cause of craniofacial clefts remains obscure, and attempts to classip craniofacial clefts are replete with problems. Tessier presented his classification in 1973 and used the orbit to distinguish the facial clefts from the cranial clefts. He noted that those lesions that involve both the face and cranium tend to do so in a consistent, predictable fashion. The clefts are numbered from 0 to 14 and are oriented around the orbit like spokes of a wheel. Van der Meulen proposed a classification in 1983 on an embryologic basis. The term dysplasia was used instead of cleft, since some of the malformations do not represent true clefts. Between 1997 to 1998, authors experienced 3 cases of median and paramedian facial cleft. One was No.2 facial cleft and the others were No.0-14 facial cleft with orbital hypertelorism. Their ages ranged from 1 to 5 years.