Congenital Cervical Malformation Research Articles

A new magnetic resonance imaging-based PUMCH classification system for congenital cervical malformations: devising a standardised diagnosis pathway

ObjectivesTo develop an innovative magnetic resonance imaging (MRI)-based PUMCH (Peking Union Medical College Hospital) classification system aimed at standardising the diagnosis of congenital cervical malformations (CCMs) by identifying their distinctive MRI features.MethodsSeventy-nine consecutive patients with CCM underwent pre-treatment pelvic MRI; three experienced gynaecological radiologists retrospectively analysed these images. Qualitative assessments included Rock et al’s classification; PUMCH classification; haematometra; cervical signal features; ovarian endometriosis; haematosalpinx; and uterine, vaginal, urinary, and musculoskeletal malformations. Quantitative assessments involved the uterine volume, sagittal cervical length, and maximum ovarian cross-sectional area. The surgical treatment types were also recorded. Statistical methods were used to incorporate differences in clinical features and surgical methods into our classification.ResultsMorphologically, CCMs were categorised into three types: type I (53%) was characterised by the presence of a cervix with visible cervical canals; type II (23%) featured an existing cervix with concealed cervical canals; and type III (24%) indicated cervical aplasia, which involves a blind end in the lower part of the uterine corpus. Haematometra was significantly more prevalent in patients with type I CCM than in those with type II (p < 0.001). There were three cervical signal patterns: no signal (27%), no evident layer differentiation (21%), and multi-layer differentiation with haematocele (52%). Most patients (94%) had complete vaginal atresia. Type I CCM patients had a higher likelihood of regaining normal uterovaginal anatomy compared to types II and III.ConclusionsOur proposed PUMCH classification system has a high potential for enhancing the efficiency of clinical diagnosis among patients with CCM.Critical relevance statementThe proposed new PUMCH classification promised to elevate the conventional diagnostic trajectory for congenital cervical malformations, offering a valuable framework to refine the selection and planning of surgical interventions, thereby enhancing overall clinical efficacy.Key PointsEffective classification of congenital cervical malformations is desirable to optimise the diagnostic process.We presented a PUMCH classification of congenital cervical malformations using pelvic MRI.The new classification significantly aids clinical triage for congenital cervical malformations.Graphical

Abstract #1318033: Malignant Degeneration of the Thyroglossal Duct Cyst: About 4 Cases

Thyroglossal duct cysts (TDCs) are congenital cervical malformations, due to an abnormal persistence of the thyroglossal duct from the base of the tongue to its final anatomical location. Two theories may help explain the origin of TDC carcinoma: de novo within the TDC or via a natural route of dissemination of the carcinoma from the thyroid gland.

Perspectives on diagnosis and surgical treatment of congenital cervical malformations

Perspectives on diagnosis and surgical treatment of congenital cervical malformations

Congenital dynamic cervical instability in a dog: the role of computed tomography and 3D printing to plan and optimize the surgical approach

This work aims to report the use of computed tomography (CT) and 3D printing technology in the diagnosis of dynamic instability with spinal compression, and the planning of cervical stabilization surgical of a 3-month-old canine with congenital cervical malformations. The patient had non-ambulatory tetraparesis, and a radiograph showed the fusion of the vertebral bodies from C3 to T3. A CT scan pinpointed the site of the spinal compression and revealed dynamic instability and severe spinal compression between the C2-C3 vertebrae. Tomographic images were processed to print a 3D prosthesis of the patient’s craniocervical-thoracic region to optimize and plan the necessary surgery. After studying the 3D prosthesis and selecting the surgical implants required, cervical stabilization surgery through ventral access was performed. The patient’s clinical improvement was rapid and progressive. Fifteen days post-surgery he was already in a quadrupedal position, after approximately 50 days he started walking again. In a year and a half of follow-up, the patient did not present any recurrence of the clinical signs. The conclusion was that CT and 3D printing contributed substancially for the clinical and surgical management, inferring quality, precision, and agility in the therapy undertaken.

Congenital Cervical Vertebral Malformation in Lambs

Background: The occurrence of congenital defects in the sheep is estimated to be between 0.2% and 2%. For congenital anatomical alterations, diagnostic imaging allows objective understanding and evaluation, and is a great aid in the formulation of clinical decisions. Most of these anomalies are diagnosed by radiography; but computed tomography (CT) can provide important additional information. In the current literature there are no descriptions of the use of radiography andCT for a more detailed evaluation of the anatomical structures in cases of congenital cervical malformations in lambs. The objective of this study is to report the clinical, radiographic and tomographic findings in two cases of congenital cervical vertebral malformation in lambs.Cases: Two lambs with cervical morphological alterations since birth were attended. A 4-month-old mixed-breed lamb, weighing 11 kg, with lateroventrocaudal deviation of the neck was observed to the right side, not yielding to the attempt of repositioning. The owner reported that these changes were identified since birth, leading to difficulties in suckling colostrum, necessitating artificial feeding. With the growth of the animal, worsening of the cervical deviation resulted in the impossibility of grazing. The radiographs of the cervical spine identified marked scoliosis, and the axis presented small dimensions and morphological changes with a slight loss of atlantoaxial articular relationship. Spondylopathies were detectedalong the cervical spine. Other lamb of the Lacaune breed, weighing 4.2 kg, was presented shortly after birth with changes in the shape and posture of the neck, difficult locomotion and in sternal decubitus. The owner reported that the lamb came from a twin eutocic birth, with the other lamb being apparently normal. The animal was unable to ingest the colostrum, in which the sheep was milked and colostrum was offered through a bottle. Lateroventrocaudal deviation of the neck to the left side did not yield to the repositioning attempt, the mandible also presented left lateral deviation. The radiographs of the cervical spine showed morphological changes in atlas, and it was not possible to delimit its wings; itwas also observed that the dorsal blade was parallel to the spinal process of the axis. The presence of a hypoattenuating linear left lateral image of the dorsal arch and a right ventro-lateral aspect of the atlas body was identified with slightly irregular and sclerotic margins, suggesting fracture lines. The body of the axis presented a conformational alteration withirregular contours and a large free fragment in the cranial aspect, suggestive of being the odontoid process, with rotation and deviation to the left in relation to the atlas. The right lateral cranial articular process of the third cervical vertebra (C3) presented a conformational change and important lateral rotation of the axis. Mild stenosis of the medullary canal was observed in the segment adjacent to C3. The euthanasia was recommended.Discussion: Congenital cervical malformations in sheep are rare in the literature and may lead to serious decrease in the quality of life of the animals. Computed tomography was superior to radiography in morphological evaluation in cases of congenital cervical malformations in sheep. However, both diagnostic methods were important to establish the best clinical behavior. The etiology of most congenital malformations is unknown, simply because of the complexity of the mechanismsthat lead to the formation of an abnormality. The isolated episodes of this anomaly, in the herd without previous alterations, suggest a non-infectious cause, probably similar among the cases, but not established. The information presented can be used to validate clinical reasoning in future cases similar to those described, where imaging features are not available.Keywords: anomalies, fetus, radiography, computed tomography.

Congenital anomalies of the uterine cervix: lessons from 30 cases managed clinically by a common protocol

To outline the anatomic variations of malformations of the uterine cervix and to discuss the clinical management of cervical agenesis and dysgenesis. Thirty patients who were treated for cervical malformations in six institutions during a 69-year interval are reviewed. Hospital-based tertiary-care reproductive endocrine infertility units. Thirty women with congenital uterine-cervical anomalies. Exploratory laparotomy with cervicovaginal reconstruction or hysterectomy. All surgical findings were carefully reviewed to determine the anatomic characteristics of the malformed cervix. When cervical reconstruction was performed, the patient was followed to determine the need for reoperation or if the patient achieved a pregnancy. Patients lacked a cervix (cervical agenesis) or had one of three variants of cervical dysgenesis. Patients with cervical dysgenesis were characterized as having: 1) an intact cervical body with obstruction of the cervical os; 2) a cervical body consisting of a fibrous band or cord; or 3) cervical fragmentation. One patient conceived after reconstruction. Reoperation was less likely to occur among women who had an obstructed endocervical canal but an otherwise normal cervical body. There are two forms of anatomic congenital cervical malformations, the second of which may be considered as three distinct variations.