Chang Gung Forum: An Exemplary Strategy for Implementing a Multidisciplinary Network of Experts in Craniofacial Anomalies.

INTRODUCTION: The Chang Gung Forum has been dedicated to the care of craniofacial anomalies since 2000. This annual continuing medical education program focuses on orofacial cleft and surgery-first orthognathic surgery by providing up-to-date information and management guidelines. This study explored how the Chang Gung Forum has influenced medical perspectives, decisions, and practices in a multidisciplinary craniofacial team. METHODS: Between 2000 and 2022, 20 Chang Gung Forums have been held. A questionnaire was distributed among 170 attendees who had participated in the forum more than once. The questionnaire collected information on the participants' experiences and levels of satisfaction with the educational program and whether or how it had influenced their clinical practice. RESULTS: Valid responses from 86 attendees (response rate, 50.6%) who had participated more than once were collected and analyzed. The overall satisfaction rate of the Chang Gung Forum based on the respondents' most recent visits was 4.28 ± 0.63 out of 5. Of the respondents, 90.9% acknowledged changes in their clinical practice, with modifications in surgery plans and decisions being the most notable (48.5%). In addition, comprehension increased throughout years of attending the annual forum (P < 0.001). CONCLUSION: The Chang Gung Forum has contributed markedly to the community of congenital craniofacial anomalies. The program will continue providing updated information and influencing the clinical decision-making of health care professionals.

Reworking Classification of First Branchial Cleft Anomalies.

OBJECTIVES: To evaluate the clinical features of first branchial cleft anomalies (BCAs) and their relationship to pre-operative imaging, pathologic data, and post-operative surveillance outcomes. Additional aims were to assess the validity of the Work classification and describe features of recurrent cysts. METHODS: Records for 56 children (34 females, 22 males; age at surgery of 5.6 ± 4.4 years) collected over a 12-year period (2009-2021) were reviewed. Imaging and pathologic slides were re-reviewed in a blinded fashion by experts in those respective areas. Parents were contacted via telephone to obtain extended follow-up. An alternate classification method based on the presence (type II) or absence (type I) of parotid involvement is provided. RESULTS: Only 55% of first BCAs could be successfully classified using Work's method. First BCAs within the parotid were more likely to present with recurrent infections, involve scarred tissue planes and lymphadenopathy, and demonstrate enlarged lymphoid follicles on pathology. The overall recurrence rate was 16%, and recurrence was 5.3 times more likely when external auditory canal cartilage was not resected. Preoperative imaging was useful for predicting the extent of surgery required and the presence of scarred tissue planes. CONCLUSION: First BCAs within the parotid gland involve more difficult and extensive surgical resection and the potential for morbidity related to facial nerve dissection. Appropriately aggressive surgical resection, which may include the resection of involved ear cartilage, is necessary to prevent morbidity related to recurrence. LEVEL OF EVIDENCE: 4 Laryngoscope, 134:459-465, 2024.

Proboscis Lateralis With Choanal Atresia.

The case of a female patient who was born with proboscis lateralis, choanal atresia, and telecanthus is submitted. A report is made on the initial management of this patient, the clinical follow-up that has been carried out so far, and a review of the literature is conducted, taking into account the limited information found in this specific pathology, in order to contribute to its diagnostic orientation and treatment from a plastic and craniofacial surgery point of view.

European Guideline Robin Sequence An Initiative From the European Reference Network for Rare Craniofacial Anomalies and Ear, Nose and Throat Disorders (ERN-CRANIO).

A European guideline on Robin Sequence was developed within the European Reference Network for rare and/or complex craniofacial anomalies and ear, nose, and throat disorders. The guideline provides an overview of optimal care provisions for patients with Robin Sequence and recommendations for the improvement of care.

[One-stage operation surgical efficacy observation of congenital preauricular fistula infection and static period of inflammation in children].

Objective:To compare the clinical effect of surgical treatment of congenital preauricular fistulas in children during the local infection period and static inflammatory period. Methods:Forty children with congenital preauricular fistula infection treated in our hospital from January 2020 to December 2022 were selected as the experimental group, and 39 children with congenital preauricular fistula inflammation at static period were selected as the control group. The fistula of the two groups of children aged between 1-14 years old was located in front of the foot of the ear wheel or the foot of the ear wheel, and all were unilateral fistulas. The postoperative follow-up was 6 months to 2 years, and the efficacy of the two groups was compared. Results:There was no significant difference in the healing rate of stage Ⅰ and stage Ⅱ between the two groups（P>0.05）. There was no significant difference in fistula recurrence rate and satisfaction with the preauricular scar between the two groups after treatment（P>0.05）. There was no significant difference in postoperative hospital stay between the experimental group and the control group（P>0.05）. Conclusion:The effect of surgical treatment of congenital preauricular fistula in the infected period is similar to that of surgical treatment in the static period of inflammation, and it can reduce the pain of dressing change under local anesthesia in children, avoid the second operation in children, and reduce the economic cost. This treatment method is worthy of clinical promotion. Appropriate incision and resection method were designed according to the fistula and infection sites.

Three-Dimensional Digital Technology: A Powerful Assistant for the Reconstruction of Complex Craniofacial Deformities.

Three-dimensional digital technology has been widely applied in craniofacial surgeries, particularly in conventional procedures such as facial contouring and orthognathic surgery. To some extent, the process has already been standardized. As to reconstruction surgeries of complex craniofacial deformity, however, surgical plans need to be patient-specific. Traditionally, individualized surgical treatment largely relies on the surgeon's clinical experience. The application of digital technology mainly focuses on preoperative planning and postoperative evaluation of surgical outcomes rather than intraoperative patient-specific implants or osteotomy guides. For patients with complex craniofacial deformities, requirements for accurate 3-dimensional digital technology can be much higher. This study presents a favorable surgical reconstruction of the severe craniofacial deformity using 3-dimensional digital technology, providing references for craniofacial surgeons, which could be beneficial to clinical practice and treatment efficacy.

Congenital Midline Cervical Cleft and Thyroglossal Duct Fibrous Cord-Like, is There a Mixed Presentation?

INTRODUCTION: Congenital midline cervical cleft is a rare condition and is frequently misdiagnosed as thyroglossal duct cyst. Otherwise, the combination of congenital midline cervical cleft and thyroglossal duct fibrosis in the same patient is as rare as important to be registered with the intention to inform and offer specific managements details for the literature. CASE PRESENTATION: Eight-year-old boy with simultaneous congenital midline cervical cleft and a thyroglossal duct fibrosis. The anatomic, clinical, radiologic, and pathologic characteristics of the congenital midline cervical cleft are described as well as surgical technique for removal and repair with Z-plasty. CONCLUSION: Congenital midline cervical cleft is a rare condition and when diagnosed must be surgically treated as early as possible. Its differential diagnosis is a clinical challenge.

Collaural fistula: Our experience.

Branchial cleft anomalies are congenital, arising from the first to the fourth pharyngeal clefts. The most common is a second arch anomaly. As it is congenital, it presents at birth though may become symptomatic later. The spectrum of anomalies includes sinus, cyst, or fistula formation or a combination of these. Here we present a case series based on first cleft anomalies. The principles of management include early diagnosis, excision of any fistulous tract, and prevention of injury to the facial nerve.

Management of the infected preauricular sinus.

INTRODUCTION: The preauricular sinus is a common finding in children and may often be complicated by infection. Complete excision of the sinus is the only definitive cure. Failure to recognise the presence of a sinus, particularly when infection erupts away from the sinus, may lead to inadequate management and unnecessary surgery. AIM: We report our experience in managing infected preauricular sinuses and highlight important points in our surgical technique. METHODS: A retrospective review was performed of our electronic patient database for all paediatric patients who underwent surgical excision of preauricular sinuses by the senior author at Great Ormond Street Hospital for Children between January 2013 and October 2022. RESULTS: A total of 10 patients underwent surgical management of 11 preauricular sinuses with a median follow-up time of 40 months (range 1-136). Eight patients underwent excision of the preauricular sinus due to infection. All infected cases affected the preauricular cheek skin and had previously undergone at least one unsuccessful surgical drainage prior to a referral to our unit. All cases were successfully operated on at our unit with no reported complications or recurrence. CONCLUSION: A lack of awareness of the presence of a sinus and identification of a preauricular pit by the inexperienced clinician will result in inadequate treatment of this condition and potentially unnecessary surgical procedures. Our paper highlights the importance of correctly identifying the extent of the sinus and describes a safe and reliable technique to ensure complete removal of the preauricular sinus with satisfactorily low recurrence rates.

Tessier's Cleft Number 6 Revisited: A Series of 26 new Cases and Literature Review of 44.

OBJECTIVE: To fix a gray zone left in Tessier's classification of rare clefts with cleft 6 and to give a more comprehensive description of cleft 6 anatomy. DESIGN: The material used for the research was a series of 26 clinical cases of patients with assessed cleft 6 and 44 cases found out of a literature review with enough data to be useful. The 70 cases were cross-examined by the authors. STUDY SETTING: The authors are senior craniofacial surgeons working in high-case load department from university centers where the patients are documented and receive primary as well as secondary treatment and follow-up. PATIENTS: The patients were selected out of the series of craniofacial deformities taken care of by the authors' department as rare clefts. MAIN OUTCOME: We describe the full spectrum of cleft 6 as an autonomous entity that could present itself in three subtypes: 6a is the most proximal and could be associated with cleft 8. The subtype 6b is medial toward the zygomatic arch and frequently associated with a bone and teeth appendage (frequently described as a "maxillary duplication"). The subtype 6C goes toward the external ear between the helix crus and the auditory meatus. CONCLUSIONS: The Tessier's opinion is that Treacher Collins syndrome was the association of clefts 6, 7, and 8 and is no longer sustainable in the light of modern genetics. Most of the cleft 6 are misdiagnosed in the literature.

Congenital first branchial cleft anomalies in children: a study of 100 surgical cases and a review of the literature.

OBJECTIVE: To investigate the clinical features and surgical outcomes of pediatric congenital first branchial cleft anomalies (CFBCAs). METHODS: We conducted a retrospective analysis of 100 children who were referred to Shanghai Children's Hospital from March 2014 to March 2022 for the treatment of CFBCAs. RESULTS: This study included 100 patients (33 males, 67 females) with an average age of 4.0 ± 2.7 years. 64 cases were type I FBCAs and 36 were type II. The main clinical manifestations included having a skin pit or discharge from it (62%), painless masses (5%), mucopurulent otorrhea (8%) and recurrent swelling with pain (90%) in the Pochet's triangle area. 92% had infection histories, 84% had incision and drainage histories, and 18% had surgical histories. 6 cases of tympanic membranous attachment were found by auricular endoscopy. Ultrasonography (US) was 55.6% (30/54) accurate and enhanced CT was 75% (75/100) accurate in diagnosing CFBCAs. We dissected the facial nerve (FN) in 46% cases. Lesions ended in the external auditory canal (EAC) wall in 86 cases. 69 exhibited close relationship with the parotid. The patients were followed up 0.25-8.2 years. 11 had postoperative temporary facial paralysis and all improved within 6 months. 3 had recurrence and they were secondarily successfully retreated. No EAC stenosis were found. CONCLUSIONS: CFBCAs often presented with repeated swelling and purulence in Pochet's triangle. CT, US and auricular endoscopy can assist in diagnosis and planning the surgical strategy. Complete excision in non-infection stage as soon as possible is the first choice for the treatment of CFBCAs.

Birk-Barel Intellectual Disability Dimorphism and KCNK9 Imprinting Syndrome: Craniofacial Surgery Considerations for an Exceedingly Rare Syndrome.

Birk-Barel intellectual disability dimorphism syndrome, also referred to as KCNK9 imprinting syndrome, is an exceedingly rare condition described in under 20 cases that presents with intellectual disability, hypotonia, scoliosis, dysphonia, dysphagia, and craniofacial dysmorphic features. The condition follows an autosomal dominant pattern of inheritance in the maternally expressed KCNK9 gene on chromosome 8. Due to the complexity of presentation, patients with Birk-Barel syndrome are optimally managed by a multidisciplinary team including a craniofacial surgeon. Previously described craniofacial dysmorphic features include micrognathia, cleft palate, dolichocephaly, broad nasal tip, and broad philtrum, among others. Here the authors describe a genetically confirmed case that has been managed in our institution's multidisciplinary cleft and craniofacial clinic. The authors aim to discuss Birk-Barel syndrome for a surgical and craniofacial audience with considerations for operative management in the context of a multidisciplinary team.

Historical Perspectives on the Management of Craniosynostosis.

The history of craniofacial surgery is one of many fundamental advances by monumental figures. Although craniosynostosis has been known to exist for multiple centuries, modern management has evolved over roughly the last century. An overview of early history, early scientific exploration, the advancement of surgical treatment of craniofacial deformities and the current state of craniosynostosis management is discussed. To fully appreciate the evolution of craniosynostosis surgery, one must understand the gradual advancements that have brought the specialty to this modern era.

Utilidad de la impresión 3D en la cirugía de craneosinostosis / 3D printing utility in craniosynostosis surgery

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Long-term follow-up of the inside-out technique for treatment of preauricular sinuses: observational study.

PURPOSE: This long-term follow-up study aims to externally validate the inside-out technique for surgical treatment of preauricular sinuses. METHODS: We analyzed the medical records of all patients who underwent the IO procedure at University Hospitals Leuven from 1 November 2005 to 30 November 2019 (N = 110). We were able to contact 77 of these 110 patients for a telephone interview, where we used a standardized questionnaire to evaluate recurrence, patient satisfaction and aesthetic result. RESULTS: We studied 110 patients (68 males, 77 females), resulting in 145 PAS (35 left, 40 right, 35 bilateral), with a median follow-up of 53 months. Fifteen PAS (10.3%) developed an early (< 4 weeks) postoperative complication (wound dehiscence, infection, abcedation, swelling). Two PAS (2 different patients) needed revision surgery because of recurrence, one after 15 months, the other after 4 years. The 5-year Kaplan-Meier recurrence-free percentage was 97.7%, equating to an estimated recurrence rate of 2.3%. Ninety-four percent was satisfied with the aesthetic result, giving 4 or 5 points on a 5-point Likert scale. CONCLUSIONS: This study was able to externally validate the IO technique for the treatment of PAS. The IO technique should be first choice, since it guarantees low recurrence rates, excellent aesthetic results, and good patient satisfaction.