Congenital Midline Cervical Cleft Research Articles

Unveiling a Misdiagnosed Midline Cervical Cleft: A Case Report and Review of the Literature

Congenital midline cervical cleft (CMCC) is a rare congenital abnormality of the ventral neck that is often misdiagnosed. We present a case of a 12-year-old male with a history of incomplete CMCC excision and limited neck extension. We highlight the need for increased awareness of this anomaly and propose a clinical algorithm for clinicians when faced with this rare congenital condition. CMCC typically presents at birth and involves a defect along the midline of the neck, characterized by a nipple-like protrusion of the skin and a fibrous cord with an underlying sinus. Micrognathia and mandibular growth restriction are anticipated consequences of CMCC, requiring early intervention. Current treatment guidelines recommend surgical excision prior to age two using a Z-plasty technique. This report emphasizes the rarity and diagnostic challenges of CMCC. It underscores the significance of accurate identification and appropriate surgical planning in a timely manner. Based on the authors’ experience with this case and a review of the literature, we propose a clinical algorithm aimed to enhance CMCC diagnosis and management.

Modified Z-plasty repair for congenital midline cervical cleft in a child

Modified Z-plasty repair for congenital midline cervical cleft in a child

Вроджена серединна розщілина шиї (огляд літератури та опис власних клінічних спостережень)

Midline cervical cleft - (synonym: webbed neck, pterygium colli medianum, neck ugliness), belongs to rare congenital defects which, unfortunately, have not been widely covered in the specialized medical literature at the modern stage of the development of medicine. It is confirmed by the fact that until 2014 according to the literature search there were a little over 100 detailed descriptions of the respective pathology. Purpose - to summarize the experience accumulated by experts in order to understand deeper all the aspects of Congenital midline cervical cleft, to introduce the present position of this rare congenital malformation to colleagues, to learn the structural parts of this pathology and the tactic for its treatment. The article represents the last data from scientific literature about Congenital midline cervical cleft and the analytic results of the two medical cases with this congenital malformation in children. Studying these cases, we used general clinical tests and histological research for the removal tissues. Conclusions. The current lack of information on the midline cervical cleft as well as the results of histological examinations in different clinical cases confirm the necessity of further study of this rare congenital defect. In this case, a deeper analysis of embryological, genetic and environmental factors influencing the process of formation of this pathology can be of help. In our opinion, performing the surgery becomes appropriate before two-year age in order to prevent the relapse of this pathology and severe neck contractures in the future. The study was carried out in accordance with the principles of the Helsinki declaration. The informed consent of the patient was obtained for conducting the studies. No conflict of interests was declared by the authors.

Congenital Midline Cervical Cleft: A Rare Window to the Neck.

Facial Plastic Surgery & Aesthetic MedicineVol. 25, No. 2 ObservationsCongenital Midline Cervical Cleft: A Rare Window to the NeckDavid A. Shaye, Hirwa Nteyumwete, Aimee Kamaliza, Theophile Muhawenimana, Josiane Umutoni, Isaie Ncogoza, and Victor NyabyendaDavid A. Shaye*Address correspondence to: David A. Shaye, MD, MPH, FACS, Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology-Head & Neck Surgery, Massachusetts Eye & Ear, Harvard Medical School, 243 Charles Street, Boston, MA 02114, USA. E-mail Address: david_shaye@meei.harvard.eduhttps://orcid.org/0000-0002-7786-7812Division of Facial Plastic and Reconstructive Surgery, Department of Otolaryngology-Head & Neck Surgery, Massachusetts Eye and Ear, Harvard Medical School, Boston, Massachusetts, USA.College of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this author, Hirwa NteyumweteCollege of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this author, Aimee KamalizaCollege of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this author, Theophile MuhawenimanaCollege of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this author, Josiane UmutoniCollege of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this author, Isaie NcogozaCollege of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this author, and Victor NyabyendaCollege of Medicine & Health Sciences, University Teaching Hospital Kigali, University of Rwanda, Kigali, Rwanda.Search for more papers by this authorPublished Online:3 Mar 2023https://doi.org/10.1089/fpsam.2022.0101AboutSectionsView articleView Full TextPDF/EPUB Permissions & CitationsPermissionsDownload CitationsTrack CitationsAdd to favorites Back To Publication ShareShare onFacebookTwitterLinked InRedditEmail View article"Congenital Midline Cervical Cleft: A Rare Window to the Neck." Facial Plastic Surgery & Aesthetic Medicine, 25(2), pp. 184–185FiguresReferencesRelatedDetails Volume 25Issue 2Apr 2023 InformationCopyright 2023, American Academy of Facial Plastic and Reconstructive Surgery, Inc.To cite this article:David A. Shaye, Hirwa Nteyumwete, Aimee Kamaliza, Theophile Muhawenimana, Josiane Umutoni, Isaie Ncogoza, and Victor Nyabyenda.Congenital Midline Cervical Cleft: A Rare Window to the Neck.Facial Plastic Surgery & Aesthetic Medicine.Apr 2023.184-185.http://doi.org/10.1089/fpsam.2022.0101Published in Volume: 25 Issue 2: March 3, 2023Online Ahead of Print:June 29, 2022PDF download

A delayed diagnosis of congenital midline cervical cleft

Congenital midline cervical cleft (CMCC) is an extremely rare anomaly of the neck that typically presents in the neonatal period as a thin suprasternal vertical band of erythematous skin with a nipple‐like projection. We present the management of this uncommon and rarely described entity in a 9‐year‐old girl.

An unusual case of the congenital mesenchymal hamartoma of the neck associated with the midline cervical cleft in neonate

Abstract Objectives Hamartomas are non-neoplastic developmental anomalies, mostly congenital, characterized by uncontrolled, disorganized proliferation of local endogenous tissue, which can normally be found at the site of origin and are very often mesodermally derived. It is well known that hamartoma can be associated with congenital midline cervical cleft and therefore cause a variety of symptoms. In general, they are benign and indolent, but they can be the cause of complex morbidity if they are localized within specific regions, such as the head and neck, which represent highly sensitive and vulnerable areas. Case presentation The reported case is unusual because of the presence of a congenital mesenchymal hamartoma along with the median cervical cleft, in a 1-day-old neonate, without the presence of any respiratory symptoms or associated congenital features. Although extremely rare, hamartomas should be included in the differential diagnosis of congenital neck masses, with emphasis on diagnostic approach, to avoid overly aggressive treatment and possible complications, such as infection, further mass growth, malignant transformation and compression of the adjacent neck structures. Conclusions Appropriate and timely treatment of the hamartoma of the neck in neonates, with further follow-up is necessary to avoid an overly aggressive treatment and to distinguish benign from malignant lesions, which is necessary for successful curative outcome.

Congenital Midline Cervical Cleft: A very rare clinical entity

Congenital midline cervical cleft is an extremely rare anterior neck defect. We describe a case of a male newborn with midline cervical cleft outlining aspects of diagnosis and stressing the importance of early diagnosis and surgical treatment.

Congenital midline cervical cleft

Midline cervical cleft is an extremely rare congenital anomaly of the anterior neck, which accounts for 1.7–2% of congenital neck malformations. Here, we report a case of an 18-month-old boy with congenital midline cervical cleft who was successfully treated with surgical resection.

Congenital Midline Cervical Cleft: A Variant of Tessier Number 30 Cleft Causing Micrognathia.

Congenital midline cervical cleft is a rare and generally isolated malformation of the ventral neck region with no clear etiology established. Mandibular deformities, such as micrognathia, could be considered as a consequence of a cleft cervical contracture. Complete surgical excision of the subcutaneous fibrous cord at an early age is the primary treatment modality, minimizing growth development problems on surrounding affected tissue. The aim of this study is to describe the clinical, surgical, and histological findings in a female child with congenital midline cervical cleft along with a relevant literature review. Three years follow-up after surgery exhibited satisfactory functional and cosmetic results.

Midline Cervical Cleft: An Anatomical Finding and a Proposal for a New Approach.

Congenital midline cervical cleft is a rare malformation. Typical case shows an area of hypotrophic skin, a cranial nipple-like structure, and a caudal blind sinus. Cervical extension is limited. Relapse of the retraction is common following cutaneous z-plasty. The aim of this study is to describe the radiological, surgical, and histological findings of the 4 cases treated in our center in the last 8 years and communicate the finding of a contractile structure, anterior to the platysma, composed by striated muscle, figure not previously described. This distinct muscular band is responsible for neck retraction. Removal of this releases cervical tension and is essential to avoid the relapse.

Midline cervical cleft: a case report

A 14-year-old boy presented with complaints of a sinus in the middle of neck, with mucoid discharge present since birth. MRI sinogram along with MRI neck was taken which revealed a sinus tract opening in midline, extending posteriorly and traversing inferiorly to end blindly above sternum. Patient was planned for an elective excision of sinus tract, with z-plasty for decreased scarring. Congenital midline cervical cleft is a rare entity that was first described in 1924. Subsequently there have been fewer than 100 cases reported in medical literature. It typically manifests at birth as a cleft at level of suprasternal notch with a pseudonipple above it. Pathogenesis of congenital midline cervical clefts remains speculative. Differential diagnosis includes thyroglossal duct cysts or branchial cleft anomalies.

Hamartomas and midline anomalies in association with infantile hemangiomas, PHACE, and LUMBAR syndromes.

The pathogenesis of infantile hemangiomas (IH), PHACE, and LUMBAR syndromes remains unknown. We aim to describe histopathologic features of midline anomalies associated with IH, including patients with PHACE and LUMBAR syndromes. A multicenter retrospective chart review was performed to identify patients with IH, PHACE, and LUMBAR syndrome with histopathologic specimens from sternal or midline anomalies. A total of 18 midline lesions from 13 patients were included. Out of 18, 14 midline lesions underwent both histopathologic and clinical review. Three hamartoma-like chin plaques and one supraumbilical raphe underwent only clinical review. All 13 patients had midline lesions and IH. Histopathologic diagnoses were as follows: rhabdomyomatous mesenchymal hamartoma (3), folliculosebaceous cystic hamartoma (1), fibroepithelial polyp (1), verrucous epidermal hyperplasia with vascular proliferation and fibroplasia (1), congenital midline cervical cleft (1), pericardium with fibrosis (1), fibrous components with increased collagen (1), atrophic skin/membrane (3), angiolipomatous mass with neural components (1), and lipomatous mass (1). Due to the retrospective nature of this study, it was not possible to obtain pathology slides for all midline lesions that had previously been biopsied or resected. We show clinically and histopathologically a new association between PHACE syndrome and rhabdomyomatous mesenchymal hamartoma (RMH), in addition to demonstrating the association between PHACE syndrome and chin hamartomas. We also display histopathologic findings seen in midline lesions resected from LUMBAR patients. Rhabdomyomatous mesenchymal hamartoma is thought to be related to aberrations of mesenchymal cells during development; therefore, this may provide clues to the pathogenesis of IH and related syndromes.

Congenital midline cervical cleft: a case report

BackgroundMidline cervical cleft is a very rare congenital anomaly. According to a literature search, until 2014 only 205 cases were reported.Case presentationWe present a classic case of congenital midline cervical cleft. This was a case of a 3-year-old Middle Eastern boy. The lesion was excised and the defect was closed via multiple Z-plasties.ConclusionsMidline cervical cleft, although a rarity, when presented needs surgical treatment, which comprises surgical excision and closure that lessens the possibility of scar visibility and contracture.

Congenital midline cervical cleft in a neonate born to diabetic mother – An unusual association

Congenital midline cervical cleft is a rare developmental anomaly of the neck region with only 200 cases reported till date. This case report highlights an unusual association of congenital midline cervical cleft and maternal diabetes. We report a neonate born to a mother with uncontrolled type 2 diabetes who presented with a midline neck lesion characterized by a proximal skin tag, middle mucosal surface and a distal blind sinus causing micro retrognathia with no other visible malformation. Most of the cases reported till date have a sporadic occurrence. This is the first possible report of this anomaly in a neonate of diabetic mother. This raises a possibility of being a part of diabetic embryopathy, however occurrence by chance cannot be completely ruled out.

Congenital Midline Cervical Cleft and W-Plasty: Our Experience.

Objectives Congenital midline cervical cleft (CMCC) is a very uncommon congenital anomaly of the midline anterior neck, and although it has very pathognomonic features (including nipple-like protuberance), it could be mistaken for other congenital neck lesions, such as thyroglossal duct cyst and branchial apparatus anomalies. Thus, it represents a challenging diagnosis. In this 21-patient series, we discuss the clinical features of CMCC, its pathophysiology characteristics, and its modalities management. Material and Methods We conducted a retrospective chart review of children presenting with CMCC at our institution, between January 1998 and January 2016. Results Twenty-one patients were identified with CMCC. Ages ranged between 1 day and 14 years. The length of the lesion varied from 0.5 to 5 cm, and the size of the skin tag varied from 0.2 to 1.5cm. No other significant associated anomalies were found. Surgery was the mainstay treatment, and no recurrence was found. W-plasty was used in most patients to close the defect. Conclusion With a little more than 200 published cases, our series represents the largest series worldwide. The lesion is usually isolated, and no further investigation is required. Surgery is the mainstay of treatment, with complete excision being usually curative. It should be treated at an early age to prevent complications. In our experience, W-plasty was a good alternative to the most commonly used Z-plasty, in skin closure, with respect to both aesthetic and functional results.

Congenital midline cervical cleft

Abstract Congenital midline cervical cleft (CMCC) is a very rare congenital malformation of the neck. Although its embryologic origin is uncertain, the most widely accepted embryologic defect is impaired midline fusion of the distal branchial arches. We present a case of female child with this anomaly and review of relevant literature. In literature review, CMCC has been described almost exclusively in Caucasian Female, the present case documents the occurrence of this developmental anomaly in the Egypt populations.

Facial morphology and growth following surgery for congenital midline cervical cleft patients

Congenital midline cervical cleft (CMCC) is a rare condition that consists of a cutaneous midline neck lesion with a sinus extending inferiorly towards the sternum. A fibrous band that extends superiorly to the mandible is a consistent feature of the condition. Restriction of growth of the mandible, possibly due to incomplete removal of the band, is the most significant long-term problem. It remains unclear whether early removal of the fibrous band might allow catch-up growth of the mandible. This study utilized non-invasive three-dimensional photographs to objectively evaluate the facial growth of six CMCC patients. The growth of these CMCC patients was compared to the average growth of age- and sex-matched controls from a database of three-dimensional facial photographs of clinically normal subjects. After surgical removal of the fibrous cord, CMCC patients experience growth in the chin at the same rate as in the normal population; no evidence was found for catch-up growth. As a result, individuals with CMCC are likely to require further surgical intervention to correct the residual retrognathia on completion of facial growth. Early excision of the lesion including aggressive resection of the fibrous band is still recommended, as this should optimize the early growth of the mandible in infancy.

Congenital Midline Cervical Cleft –Clinical and Pathological Review of Three Cases

Congenital Midline Cervical Cleft –Clinical and Pathological Review of Three Cases

Congenital Midline Cervical Cleft: A Case Report with Review of Literature

ABSTRACTAimTo highlight a rare case of a congenital midline cervical cleft (CMCC) in context with embryological theories/hypothesis, presentation, and management along with review of literature.IntroductionCongenital midline cervical cleft is a rare but interesting anterior neck anomaly with controversial theories/ hypothesis regarding its embryogenesis.Case reportWe describe here a classical case of midline cervical cleft that presented at birth with a cephalocaudal orientation, extending from the level below the hyoid bone to the suprasternal notch with a length of 3 cm and width of 0.5 cm. At 6 months of age, the lesion was excised and closure was done by multiple Z-plasty, with satisfactory results.DiscussionAlthough the diagnosis is clinical, it is frequently misdiagnosed. The associated clinical features could include thyroglossal duct cysts, cleft lip/mandible/sternum, cervical contractures, mandibular spurs, microgenia, and/or bronchogenic cysts. If it is not treated at an early age, it can result in complications like webbing of the neck, dental malocclusion, and restricted neck movements.ConclusionEarliest recognition of CMCC and proper intervention can provide better esthetic and functional prognosis.Clinical significanceA correct earlier recognition of the lesion and appropriate surgical management are key to avoid longterm complications.How to cite this articleJaiswal AA, Behera BK, Membally R, Mohanty MK. Congenital Midline Cervical Cleft: A Case Report with Review of Literature. Int J Head Neck Surg 2017;8(1):25-30.

Congenital midline cervical cleft: A retrospective case series of 8 children

ObjectivesCongenital midline cervical cleft is a rare developmental abnormality of the ventral neck of unclear etiology. It consists of a midline skin defect. This study reports a case series of 8 patients with congenital midline cervical cleft. MethodsRetrospective review chart including all children referred with congenital midline cervical cleft over 5 years in tertiary center. The study was conducted to determine the presence of associated malformations, to specify the cleft pathology, to analyze the nature of associated cysts, and to discuss surgical procedure. ResultsEight patients ranged from 3 days to 5 years. Two had an associated cervical midline cyst, 3 had a significant micrognatia. Pathological observations were in favor of a branchial origin. There was no recurrence of cervical contraction after a mean follow-up of 20 months. ConclusionCongenital midline cervical cleft is a rare and generally isolated congenital malformation. It does not require either extensive assessment or specific genetic. Described associated cysts might be part of the cleft and not bronchogenic or thyroglossal cysts. Early surgical excision reduces cervical contracture, but linear or Z-plasty closure is still debated.