Naso-oropharyngeal choristoma (hairy polyps): an overview and current update on presentation, management, origin and related controversies.

This review presents a comprehensive and updated overview of bigerminal choristomas (hairy polyps) of naso-oropharynx/oral cavity, and discusses the controversies related to nosology and origin from a clinico-embryologic perspective. English-language texts of the last 25 years (January 1989-January 2014) were collected from the PubMed/MEDLINE database using the given keywords. Of the 330 records, 64 full-text articles (mostly case reports/series) were selected, incorporating clinical data from 78 patients, after screening through duplicates and the given exclusion criteria. With the available evidence, hairy polyps appear more common than generally believed, and are increasingly being recognized as an important, often-missed cause of respiratory distress and feeding difficulty in neonates and infants. Such a child without any apparent cause should be examined with flexible nasopharyngoscope to specifically look for hairy polyps which might be life-threatening, especially when small. The female preponderance as believed today has been found to be an overestimation in this review. These lesions are characteristically composed of mature ectodermal and mesodermal tissue derivatives presenting as heterotopic masses, hence termed choristoma. However, little is known about their origin, and whether they are developmental malformations or primitive teratomas is debatable. Involvement of Eustachian tube and tonsils as predominant subsites and the speculated molecular embryogenesis link hairy polyps to the development of the first and second pharyngeal arches. They are exceptionally rare in adults, but form a distinct entity in this age-group and could be explained as delayed pluripotent cell morphogenesis or focal neoplastic malformations, keeping with the present-day understandings of the expanded "teratoma family".

A rudimentary tragus in the nasopharynx: case report, literature review, and discussion of embryologic development.

We describe the rare case of a nine year-old girl with a several month history of mouth breathing and nasal obstruction due to a rudimentary tragus in the nasopharynx. We focus on the accessory tragus and its origins by describing the embryologic development of the external ear. Based on our review of the medical literature, this is the first report of a nasopharyngeal mass with a pathologic diagnosis of a rudimentary tragus.

Lateral congenital anomalies of the pharyngeal apparatus: part I. Normal developmental anatomy (embryogenesis) for the surgeon.

Knowledge of the embryogenesis of the pharyngeal apparatus is the only means of understanding the "architecture" of the neck. The embryonic pharynx (which includes future oral and nasal cavities) is a much more extensive area than the adult pharynx. The main feature of the developing pharynx is a series of arches, internal pouches, and external clefts, which together comprise the pharyngeal apparatus. This structure is associated with other developing splanchna of the neck, e.g., the thyroid and parathyroid glands, tonsils, and thymus. Within each of the pharyngeal arches are the developing aortic arches and, specific for each arch, cranial nerves. The complex relations of the mesenchymal derivatives of arches (muscles, cartilage, bones) with the neurovascular bundles within each arch are presented and explained. The pharyngeal apparatus undergoes dramatic transformations: pouches and clefts disappear without interruption (interruption would produce gills and support the misnomer "branchial apparatus"). In addition, in the lateroventral neck, somites migrate to produce other muscles such as sternocleidomastoid and trapezius innervated by spinal nerves. Lateral congenital anomalies largely rely on persistence of a cleft/and or pouch or communication between the two. Their tracts have a "crooked" course among other entities generated by alterations that take place during embryogenesis.

[Congenital and benign changes with regard to the pharynx]. / Kongenitale und benigne Veränderungen mit Bezug zum Pharynx.

DEVELOPMENT: The first part of this article is supposed to present a brief overview on the development of tissues and structures related to the pharynx, as far as they provide a better understanding of the most congenital lesions in this area of the body. BENIGN LESIONS: The second part of this article focuses on benign lesions originating in the pharynx or in neighbouring structures. Functional pharynx abnormalities are beyond the scope of this paper.

The prenatal pituitary gland--hidden and forgotten.

The sella turcica and pituitary gland in a human fetus (18 weeks gestation) with unilateral oro-ocular cleft combined with unilateral cleft lip and palate are described histologically. In this fetus the sella turcica was not a normal sella but a caudally open funnel. The adenopituitary gland tissue was positioned ectopically within the funnel canal and in the pharyngeal submucosa.

Pharyngeal pituitary: development, malformation, and tumorigenesis.

The development of the pharyngeal pituitary (PhP) in the fetal period was morphologically and, for the first time, immunohistochemically examined. PhP, found in every individual, begins its hormone production at the 17-18th week of gestation, that is, 4-8 weeks later than that of sellar pituitary (SP). Only 1 of 25 examined fetuses without any stigmata of developmental anomalies showed a residual pituitary fragment in the craniopharyngeal canal (craniopharyngeal pituitary, CPhP). An adult case of a rare clivus pituitary adenoma that we examined is demonstrated in discussing its relationship to PhP. Extracranial ectopic pituitary adenomas in the literature describe an exclusively sphenoid sinus/nasopharyngeal/clivus location of the tumor. Their location corresponded exactly with that of PhP, so that the origin of the tumors can be reasonably speculated as PhP, although another origin, e.g., CPhP, can not be excluded. A variety of malformations of PhP, although very rare, have been described for the fist time during the systemic examination of 16 fetuses with different cranioneural malformations, such as agenesis, unseparated PhP from SP (pharyngosellar pituitary), fragmentation, and residual pituitary tissue in the open craniopharyngeal canal. However, developmental anomaly of PhP was not specifically associated with cranioneural malformations except in cases of chromosomal aberrations. The hormone production in PhP in malformation cases tended to be retarded. Absence of SP was recorded in 50% of anencephalics in the literature; however, PhP was identified in all anencephalics in our series, independent of the existence of SP. This supports the opinion that agenesis of SP in anencephalics seems to be false information.

Congenital fistula of the apex of the pyriform sinus: an overlooked phenomenon of debatable origin.

Fistula of the pyriform sinus apex is an often overlooked entity which generally manifests itself as acute suppurative thyroiditis or recurrent deep neck abscesses in children or young adults. Two cases are reported. Arguments in favor of a fourth pharyngeal pouch origin are stressed and the surgical management is described.

Piriform sinus fistula and the ultimobranchial body.

Piriform sinus fistulae are an underlying abnormality common in patients with acute suppurative thyroiditis. The fistulae arise from the hypopharynx, and end in or adjacent to the thyroid lobe. These congenital fistulae seem to be remnants of one of the pharyngeal pouches in embryonic development, but their exact origin is still controversial. Resected specimens of the thyroid glands and fistulae from 15 patients were examined immunohistochemically with rabbit antisera to human calcitonin and thyroglobulin. The fistulae were lined by squamous, columnar or ciliated epithelium, and sometimes formed branches in the thyroid lobe. Near the branches solid cell nests existed. Mucous glands, follicular structures and thymic tissue were found in the fistula. The follicular structures stained for thyroglobulin. Immunostaining for calcitonin revealed aggregates of many C cells in the thyroid near the fistula. A few calcitonin-positive cells were also found in the fistula. These findings, along with the anatomical relation of the fistulae to major structures of the neck, strongly suggest that the fistulae are remnants related to the ultimobranchial body, and that the fistulae trace the migration route of the ultimobranchial body to the thyroid gland.

[Recurrent pseudothyroiditis and cervical abscess. The fourth branchial pouch's role. Apropos of 16 cases]. / Pseudothyroïdites et abcès cervicaux récidivants. Rôle de la 4e poche branchiale. A propos de 16 observations.

Among the causes of inflammatory swelling of the neck in children, the "cysts" and ducts joining in the hypopharynx deserve being individualized. This unrecognized pathology is indeed responsible for suppurative pseudothyroiditis or cervical abscesses relapsing in spite of adequate antibiotic treatment and incision-drainage. X-ray films may help as they often show the fistula. Diagnosis relies on hypopharyngoscopy. This investigation only may assess the origin of this "internal fistula" by showing the mucous opening of the bottom of the pyriform sinus, from which sometimes springs some pus when pressing the neck. The clear left predominance of this canal and its junction between the superior and inferior laryngeal nerves suggest that its origin could be the 4th branchial pouch. Its treatment consists of complete surgical excision, which avoids relapses.