Variants of the hyoid-larynx complex, with implications for forensic science and consequence for the diagnosis of Eagle's syndrome.

Thorough anatomic knowledge of the hyoid-larynx complex is necessary for forensic radiologists and ear-nose-throat surgeons, given the many anatomic variations that originate in embryology. In forensics the anomalies must be distinguished from fractures because the latter are indicative of violence on the neck. In this manuscript we describe the anatomical variations that can be found in the hyoid-larynx complex and explain their etiology. 284 radiological scans of excised hyoid-larynx complexes were examined with X-ray and CT. Some rare cases from literature and historical collections were added. Two third of the examined hyoid-larynx complexes deviated from the anatomical standard and showed uni- or bilateral ankylosis in the hyoid bone and/or so-called triticeal cartilages. In one fifth of the cases we found striking anatomical variants, mostly derived from the cartilage of the second pharyngeal arch. Anatomical variations of the hyoid-larynx complex can be explained by embryological development. The aberrant hyoid apparatus and the elongated styloid processes (Eagle syndrome) should be considered as one clinical entity with two different expressions as both anomalies are derived from the cartilage of the second pharyngeal arch. Several variants can mimic fractures in this region, so our study is important for radiologists and forensic experts assessing cases of possible violence on the neck.

Symmetrical Apophyses on the Posterior Margins of the Thyroid Cartilage: A Previously Undescribed Anatomical Variation With Potential Forensic Implications.

Differential diagnosis between normal/variant anatomy and pathological/traumatic findings represents one of the main matters of investigation of the so-called forensic clinical anatomy. In the literature, many anatomical variations of the laryngeal thyroid cartilage have been reported, with potential implications in forensic pathology. They include triticeal cartilages, foramen thyroideum, asymmetry, segmentation or agenesis of the superior thyroid horns, bone connections with the hyoid bone, absence of cricothyroid facets, hypertrophy of the inferior thyroid tubercle, and asymmetries in the thyroid notch, isthmus, or length of the inferior horn. In this report, we describe a laryngeal anatomical variation never described before, consisting of 2 apophyses symmetrically arising from the posterior margins of the thyroid laminae. Postmortem computed tomography performed on the laryngeal visceral block excluded previous traumatic injuries or natural pathologies of the laryngeal cartilages, confirming the congenital origin of the finding. An "omega epiglottis" and 3 laryngeal cysts in the piriform sinuses also coexisted, suggesting the possibility of underlying common developmental mechanisms.

Laryngeal anomalies: Pitfalls in adult forensic autopsies.

OBJECTIVE: This is the first paper to group together most of adult laryngeal anomalies or malformations which may be misinterpreted by the forensic pathologist and taken for a proof of violence. MATERIAL AND METHODS: A review of the literature, to list the main pitfalls, to explain their nature and their origins. RESULTS: We found two main categories, the congenital defects and the acquired anomalies. CONCLUSIONS: The laryngeal region is complex. The pathologist must keep in mind anatomical variations or malformations, but also sequelae of old injuries and iatrogenic lesions. The survey, the patient's clinical history, the findings of the whole autopsy and, if necessary, histology may help to interpret a laryngeal anomaly.

Laryngotracheal anomalies in children with syndromic craniosynostosis undergoing tracheostomy.

Children with syndromic craniosynostosis may present with airway anomalies. We reviewed a cohort of such individuals who underwent tracheostomy at the Great Ormond Street Hospital for Children (London, UK) between 1999 and 2012 from a prospectively collated database. A case note review was undertaken in 11 patients. We evaluated the indication for tracheostomy in these children and the presence of laryngotracheal anomalies. The most common indication for tracheostomy was upper airway obstruction refractory to medical and first-line surgical management. Laryngotracheal anomalies were detected both at diagnostic microlaryngoscopy and bronchoscopy and at the time of tracheostomy. The commonest anomaly was a tracheal cartilaginous sleeve, but we also describe the figure-of-eight trachea that, to the best of our knowledge, has not been described before in this group of patients. A mutation of a fibroblast growth factor gene was found in 71% of our patients with a laryngotracheal anomaly. We present the largest cohort of children with syndromic craniosynostosis and confirmed anatomical anomalies of the airway and uniquely describe the figure-of-eight appearance of the trachea. These findings highlight the importance of thorough airway evaluation and the special care needed in airway management for children with syndromic craniosynostosis.

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La epiglotis o el cartílago epiglótico es una lámina delgada, flexible, ovalada de cartílago elástico localizada en la parte antero superior de la laringe y posteriormente al cartílago tiroides. Su función principal es la de protegerlas vías aéreas durante la deglución, evitando la entrada de sólidos y líquidos en las mismas (figura1). Está formada por una cara posterior recubierta de mucosa de la laringe; una cara anterior que se relaciona con otras estructuras como el cartílago tiroideo, la membrana tirohioidea, hueso hiodes y base de la lengua y unos bordes laterales. Deriva de la porción caudal de la eminencia hipobranquial, apareciendo hacia la 3ª semana y se diferencia de la base de la lengua hacia la 6ª semana. En cuanto a su situación y como parte de la laringe, al comienzo de su desarrollo se coloca cranealmente y luego se desplaza progresivamente en sentido caudal. Este desplazamiento caudal va a perdurar durante el nacimiento y los primeros años de vida. Durante la lactancia, la epiglotis alcanza la nasofaringe y se coloca por detrás de la cara superior del velo palatino. Esto es importante ya que el neonato debe respirar y beber al mismo tiempo. En la infancia, la extensión del cuello eleva el paladar blando permitiendo la ventilación oral en vez de la respiración nasal propia del neonato, permitiendo el descenso de la epiglotis así como de otras estructuras como el cuerpo del hioides, la glotis, el margen inferior del cricoides y la bifurcación traqueal. Siendo esta evolución más marcada en hombres que en mujeres debido a los cambios estructurales propios del desarrollo y los cambios fonatorios. Por su localización la epiglotis es una estructura que rara vez puede observarse a la exploración oral sin la utilización de espejo laríngeo. Si bien durante la infancia puede ser observada en algunos casos, los posteriores cambios anatómicos y el desarrollo de la voz dificultan su visualización directa en la edad adulta (AU)

The incidence of the foramen thyroideum in the South African population / Incidencia del foramen tiroideo en la población de sudáfrica

The foramen thyroideum is described as an occasional opening existing in one or both laminae of the thyroid cartilage which may or may not contain a neurovascular component. Foramen thryoideum was first described in the literature by Segond in 1847. Some authors consider its existence a structural variation rather than an anomaly, with classical texts of anatomy providing little detail when describing this foramen. This study was undertaken to investigate the incidence and characteristics of the foramen thyroideum in the South African population. A total of 80 formalin fixed cadaveric laryngeal specimens (obtained from the Department of Clinical Anatomy, University of KwaZulu-Natal, South Africa) were dissected. Larynges were dissected with the aid of a Stemi DV 4 light microscope. The incidence, location, dimensions and contents of the foramen thyroideum were recorded. The horizontal and vertical extent of each foramen was measured with a digital caliper. Six of the 80 (7.5 percent) specimens examined had distinctly identifiable foramina. Five of the six cases (4 male, 1 female) displayed bilateral foramina (6.3 percent), with one case (1 female) of a unilateral foramen (1.3 percent). Of the larynges that had bilateral foramina, a single case presented with two foramina on the same (right) thyroid lamina. A total of twelve foramina were observed. Gender distribution of foramen thyroideum was: male: right 5, left 4; female: right 2, left 1. Preceding investigators of the foramen thyroideum have rightly indicated that awareness of its presence is of paramount importance in order to preserve the structures that traverse it and also to comprehensively treat or contain laryngeal cancer.

El foramen tiroideo se describe como una apertura ocasional existente en una o ambas láminas del cartílago tiroides, la cual puede o no contener un componente neurovascular. El foramen tiroideo fue descrito por primera vez en la literatura por Segond en 1847. Algunos autores consideran su existencia como una variación estructural y no una anomalía, los textos clásicos de anatomía proporcionan pocos detalles al describir este foramen. Este estudio se realizó para investigar la incidencia y características del foramen tiroideo en la población Sudafricana. Fueron disecados 80 especímenes cadavéricos de larínge fijados con formalina (obtenidos del Departamento de Anatomía Clínica de la Universidad de KwaZulu-Natal, Sudáfrica). Las laringes fueron disecadas con la ayuda de un microscopio de luz Stemi DV 4. La incidencia, localización, dimensiones y contenido del foramen tiroideo fueron registradas. La extensión horizontal y vertical de cada foramen se midieron con un caliper digital. Seis de los 80 (7,5 por ciento) especímenes examinados tenían foramen tiroideos claramente identificables. Cinco de los seis casos (cuatro hombres y una mujer) mostraron forámenes bilaterales (6,3 por ciento), y un caso (una mujer) foramen unilateral (1,3 por ciento). De las laringes que presentaron forámenes bilaterales, un solo caso presentó dos forámenes en la misma lámina del cartílago tiroides (derecha). La distribución por sexo del foramen tiroídeo en hombres fue 5 derechos y 4 izquierdos, mientras que en mujeres 2 derechos y 1 izquierdo. Los resultados indican que el conocimiento de la presencia del foramen tiroideo es de vital importancia para preservar las estructuras que lo atraviesan y también para el tratamiento de integral o contención del cáncer de laringe.

[Surgical treatment of laryngomalacia in children]. / Leczenie operacyjne wiotkosci krtani u dzieci.

INTRODUCTION: Laryngomalacia is the most common congenital malformation of the larynx which causes stridor in newborns and infants. The stridor is inspiratory and it is first noted at birth but sometimes may not develop until 2 weeks of age. It increases in severity during crying, nursing, agitation, excitement and is made worse with head flexion or supination. Other symptoms such as supraclavicular, intraclavicular, intercostal and abdominal retractions may be associated. Boys are affected over twice as often as girls. Diagnosis of laryngomalacia is made by rigid or flexible laryngoscopy. Although this disorder is usually self-limited, in some cases the symptoms are so severe that operative intervention cannot be avoided. MATERIAL AND METHODS: Between 2002 and 2005, 13 infants were operated for severe form of laryngomalacia in the Pediatric ENT Dept in Poznan. In all patients the rigid or flexible laryngoscopy was performed to confirm the diagnosis. Then aryepiglottoplasty or epiglottoplasty were performed in all cases. It involved excision of the redundant mucosa over the arytenoid cartilages, the offending parts of he aryepiglottic folds, and trimming the lateral edges of the epiglottis, using microlaryngeal scissors and forceps. RESULTS: In all children symptoms such as: laryngeal stridor, feeding difficulties and dyspnea improved or completely resolved. In one case post-operative ventilation for several hours was required. All patients except one demonstrated significant airway improvement in the immediate postoperative period. CONCLUSIONS: Direct laryngoscopy must be performed prior to the aryepiglottoplasty to decide what amount of tissue to resect. Endoscopic aryepiglottoplasty with use of microlaryngeal instruments is an effective and safe method of the treatment of severe form of laryngomalacia. It is better to perform this procedure in general anesthesia without intubation.

Surgical repair of laryngotracheoesophageal clefts by tracheoesophagoplasty with two overlapping flaps.

We report a technique for surgical repair of laryngotracheoesophageal clefts from type II to IV through an anterior approach that involves performing a tracheoesophagoplasty with two overlapping flaps (TEP-TOF). We offer a technical description, a retrospective study, and a review of the literature. Ten children were operated on for laryngotracheoesophageal clefts of types II, III, or IV of the Benjamin and Inglis classification by the TEP-TOF procedure. After a median follow-up of 58 months (2 months to 12 years), neither complications nor recurrences were observed. All surgical details of the TEP-TOF procedure, including the approach, the kind of sutures, the method of creating the flaps, and the preoperative and postoperative management, are discussed and compared with the data found in the literature.

Anomalies and alterations of the hyoid-larynx complex in forensic radiographic studies.

The authors illustrate the anatomic interest of the hyoid-larynx complex in the differential diagnosis of traumatic lesions, purely anatomic anomalies, and lesions resulting from natural causes. Forty larynges from corpses undergoing autopsy at the Institute of Legal Medicine of the University of Trieste were studied during the period May 1996 to May 2001. The larynges were excised and examined through radiographic magnification with dedicated mammographic equipment. Triticeous cartilages were evidenced in 12 (30%), they were bilateral in 7 (17.5%) and monolateral in 5 (12.5%). These cartilages were more frequent in men (8 out of 26; 31%) than in women, 4 out of 14 (28.5%). Total fusion of the body of the hyoid bone with the greater horns was revealed in 10 (25%), partial fusion in 4 (10%), and evident articular rima between the body and the greater horns of the hyoid bone in 14 (35%). The lesser horns of the hyoid bone were symmetrical in 29 (72.5%), asymmetric in 11 (27.5), absent in 1 (2.5%). While confirming the validity of the radiographic method used, we conclude that great care should be taken when diagnosing traumatic lesions of the hyoid-larynx complex. This diagnosis is often difficult to establish owing to the considerable frequency of anatomic anomalies of the complex.

Anterior approach bilateral musculomucosal flaps repair for laryngotracheoesophageal clefts.

Laryngotracheoesophageal clefts (LTEC) are uncommon congenital anomalies that occur when the primitive foregut fails to separate into the esophagus and trachea. The surgical repair strategy involves separation or partition of the common tract. Reported here is the authors' experience with 2 cases of LTEC (type 3) repaired with bilateral musculomucosal flaps through the anterior wall of the trachea.

[Aortopexy as a surgical treatment method in infantile congenital tracheo-chondrohypoplasia and tracheomalacia]. / Aortopexia, mint a csecsemökori congenitalis tracheo-chondrohypoplasia és a tracheomalatia mütéti kezelése.

Congenital tracheal chondrohypoplasia and tracheomalacia have come to present a significant challenge in recent paediatric surgery. Authors shortly describe their pathology, pathophysiology, symptoms, diagnostic evaluation and outline the most frequency surgical procedures for them. The first three aortopexies in the authors' departments are described in this paper, with particular attention paid to the clinical problems of congenital tracheal chondrohypoplasia and tracheomalacia and the role of this method in surgical treatment, respectively. Aortopexy is quick, easy to carry out and atraumatic for the growing trachea. Authors want to draw attention to the fact that not every form of congenital tracheal chondrohypoplasia and tracheomalacia may be treated by conservative therapy, and that surgical treatment is recommended not only for life threatening forms, but for other severe forms as well.

Patterned expression in familial Klippel-Feil syndrome.

Klippel-Feil syndrome (KFS) is characterised by congenital fusion of vertebrae within the rostral spine. The first KFS gene (SGM1) locus identified on chromosome 8 segregates with vertebral fusions and associated vocal impairment within the KF2-01 family (Clarke et al., '94, '95). Here, we describe the unique pattern of variable phenotypic expression within the KF2-01 family. The pattern of anomalies revealed a cumulative, rostrocaudal graded sequence of skipped vertebral fusions. This fusion pattern presents striking similarities with the mutant phenotype and gene expression profile of the Drosophila segment polarity gene engrailed.

Klippel-Feil syndrome associated with malformed larynx. Case report.

We report a case of a 45-year-old man with Klippel-Feil syndrome with fusion of the C2-3 and C4-5 cervical vertebrae and severe voice impairment associated with malformation of the laryngeal cartilages. The condition was also complicated by bilateral inflexibility of the arms and legs and external malformation of the ears. This case broadens the spectrum of anomalies, of branchial arch derivation, now identified in association with Klippel-Feil syndrome. We discuss the possibility that perturbation of segmentation, distinct from somitogenesis, may be linked to Klippel-Feil syndrome-associated craniofacial abnormalities.

Laringomalacia em criança: revisäo de 92 casos / Laryngomalacia in children: a review of 92 case-reports

Em revisäo de 356 endoscopias respiratórias rígidas realizadas em crianças do Hospital da Criança Santo Antonio, no período de março de 1989 a março de 1992, foram observadas 92 crianças com laringomalacia. Esta representou 60 por cento dos diagnósticos observados nas regiöes glótica e subglótica. As manifestaçöes clínicas que estiveram associadas significativamente com o diagnóstico de laringomalacia foram estridor , engasgo alimentar e cianose a esforços . Em somente 11 por cento dos pacientes foi necessária intervençäo cirúrgica (laringoplastia endoscópica). Em 30 por cento destes pacientes operados näo houve melhora, tendo sido necessária realizaçäo de traqueostomia

Laryngeal findings in short rib polydactyly syndrome: case report and embryological correlations.

Short Rib Polydactyly Syndrome (SRPS) is a group of rare congenital disorders characterized by polydactyly, short limbs, and short ribs. Infants with type I SRPS (Majewski syndrome) may exhibit dysgenesis of the larynx, which is manifested by epiglottic hypoplasia. Photographic documentation of laryngeal findings obtained at autopsy in an affected infant is provided. To our knowledge, such photographic documentation has not been previously published. Normal laryngeal development is reviewed, based on the Carnegie system of staging. The developmental errors leading to SRPS are thought to occur at 33 to 48 days of fetal life (Carnegie stages 15 through 19).

Congenital subglottic stenosis: the elliptical cricoid cartilage.

Various malformations of the cricoid cartilage produce congenital subglottic stenosis. The elliptical cricoid cartilage first was demonstrated histopathologically in horizontal sections of six postmortem specimens and now has been identified clinically in ten patients. The clinical diagnosis of the elliptical cricoid cartilage, suggested by anteroposterior soft tissue neck films, is confirmed at direct laryngoscopy. Delineation of the precise location, extent, and histopathology of subglottic stenosis provides the basis for rational management.

Minor congenital laryngeal clefts: diagnosis and classification.

Cleft larynx is a rare congenital anomaly. Detection of an unsuspected minor cleft may be difficult, but the pediatric laryngologist should suspect the possibility of cleft larynx from the clinical features. Four minor clefts are reported, three cases of supraglottic interarytenoid cleft and one of partial cricoid cleft. The technique for endoscopic diagnosis and the distinctive features are described and a classification into four types is proposed.

[Ventral enclosure of the thyroid cartilage by the hyoid bone. A complex inhibition abnormality of the laryngeal structure]. / Die ventrale Umfassung des Schildknorpels durch das Zungenbein. Eine komplexe Hemmungsmissbildung des Kehlkopfskelettes.

Performing laryngeal CTs we identified a "ventral enclosure of the thyroid cartilage by the hyoid bone" in 3 patients with vocal disturbances and demonstrated for the first time the morphological and clinical characteristics of this complex inhibition malformation: --persistence of the embryonal close relation between the thyroid cartilage and the hyoid bone with normal position of the total larynx --feminine configuration of the thyroid cartilage --tendency to disturbances of growth of the thyroid cartilage with effect on the endolarynx --reduced vocal ability and predisposition to vocal disturbances.

Ventral cleft of the larynx in an adult. Case report.

Congenital clefts of the larynx are rare and usually found dorsally. This case report describes a patient with a partial anterior nonfunsion, or ventral cleft, of the thyroid cartilage. This was first noted on computed tomography of the larynx used for delineation of carcinoma and was confirmed by horizontal whole mount histologic sections of the resected larynx. The thyroid cartilage suggested arrested fusion of the laminae in the middle to late embryonic period. This patient had an adult form of this rarely reported anomaly and the first, to our knowledge, detected with CT scan.

Development of congenital laryngeal atresias and clefts.

Congenital supraglottic laryngeal obstruction still is being described as subglottic stenosis. There are three clearly defined types of congenital laryngeal atresia, which result from arrest at consecutive developmental stages. Type 1 consists of a supraglottic obstruction, absent vestibule, and stenotic infraglottis, and type 2 is a supraglottic obstruction that separates a shallow primitive vestibule above from a nonstenotic infraglottis. These two types usually are accompanied by other defects, many of them severe. In type 3, a perforated membrane partly obstructs the glottis. Arrest of the dorsal advance of the chondrifying cricoid before the ends meet to form the dorsal lamina results in a complete laryngeal cleft, and incomplete fusion of the ends may leave a notch and/or foramen within the lamina. Failure of the interarytenoid tissue to develop will produce a local cleft, and maldevelopment of the cricoid cartilage or infraglottic submucosa can result in true subglottic stenosis without supraglottic obstruction.