Embryogenic cervico-thyro-piriform tract.

Branchial cleft fistulae are rare congenital anomalies that arise from the abnormal persistence of branchial remnants. Branchial arch anomalies are rare. They usually present as a lateral neck mass or abscess in the form of acute suppurative thyroiditis. A complete fistula of the third arch is extremely rare. We describe such a case in a 13-year-old girl who presented with a small opening in the left lower neck, from which a mucoid discharge had been present since birth. The fistula was accompanied by recurrent neck swelling. Computed tomography with contrast injection into the external skin opening revealed a continuous tract that extended to the base of the piriform sinus. Total excision of the tract up to the piriform sinus with a left hemithyroidectomy was performed. At follow-up 28 months postoperatively, the patient exhibited no evidence of recurrence.

Congenital isolated midline sinus of the upper lip: clinical and embryological approaches.

OBJECTIVES: Congenital sinuses or fistulas of the lip are uncommon malformations, yet true medial upper-lip fistulas (MULFs) are extremely rare. We present a new case of congenital upper-lip fistula located in the midline of the philtrum of an 8-month-old girl. INTERVENTION: Complete surgical removal was performed with a combined extra- and intraoral approach. Histological examination revealed that the fistula was lined by squamous epithelium with sebaceous and mucous glands and hair follicles. RESULTS: Several embryological hypotheses have been proposed concerning these anomalies. This article reexamines and discusses major embryological theories on pathogenesis of sinuses or fistulas of the upper lip. We propose that early ectodermal inclusion events may occur in the medial fusion area during formation of the intermaxillary process. This embryological approach is highly concordant with our recent hypothesis on nasal dermoid sinus cysts (NDSCs) pathogenesis, in which we proposed an embryological hypothesis with early ectodermal inclusion phenomenon in the midline suture area to explain NDSCs pathogenesis. CONCLUSIONS: Common early ectodermal inclusion phenomena could be involved in both NDSCs and MULFs pathogenesis.

[Clinical and embryological approaches to nasal dermoid sinus cysts]. / Kystes et fistules du dos du nez: de l'embryologie à la clinique.

Nasal dermoid sinus cyst (NDSC) is an uncommon congenital lesion presenting as a large panel of midline craniofacial anomalies. Thirty children with nasal midline masses and/or sinus ostia were surgically treated in the pediatric ENT and cervicofacial surgery department of Trousseau's children's hospital (Paris-France) between 1995 and 2002. All our patients underwent radiological evaluation including CT scan (including axial and coronal planes) and/or MRI (in all three planes) in search of intracranial extension. Thirteen of our patients presented with a midline cyst only, ten had nasal pit only, and seven had combined anomalies. Preoperative radiology and surgery showed an intracranial extension in 3 patients, which exhibited contact of the cyst with the dura. Recurrence being expected if any dermal tissues were left in place, "one-time-excision management" was the rule. CT and MR imaging features were reviewed. The external rhinoplasty procedure resulted in a wide surgical approach, low recurrence and good esthetic results. The embryological and anatomical origins of NDSCs are reviewed. Development of NDSC during embryological development implicates two necessary and sufficient conditions: competence of the ectodermal and mesectodermal cells to form dermoid tissue with epithelial-mesenchymal interactions, and a topographical site of ectodermal inclusion, which fits well with the various clinical presentations of NDSCs. We propose to distinguish NDSCs of anterior topography, located at the anterior skull base level from the basal NDSCs, located at the middle skull base level. We reviewed the various localizations of NDSCs, revisiting a forgotten embryological theory, which unify the various clinical localizations of anterior NDSCs.

Management of the preauricular sinus.

The preauricular sinus is a relatively common physical finding especially in the pediatric population. It is defined as a congenital lesion in which a small skin opening located in front of the external ear communicates with a subcutaneous network of cysts. The vast majority are benign in nature and require no intervention. Draining sinus tracts are prone to infection and should be excised. Complete excision of the pit and sinus tract provides the only definitive cure. To prevent problematic recurrence, we recommend wide exposure of the lesion by the technique described.

Congenital cheek fistula: a report of three cases.

Three cases of the rare condition of congenital cheek fistulae are presented. These differ from preauricular fistulae in terms of their location and the direction in which the fistula is lying. Each cheek fistula seemed to be situated along the line of the junction between the mandibular and maxillary processes of the first branchial arch.

Congenital prepubic sinus.

We report the fifth case of congenital prepubic sinus that goes from the anterior wall of the bladder through the public symphysis to the skin. The various embryological theories that could be responsible for this pathology are discussed, and according to us, it seems to be a variant of dorsal urethral duplication.

Imperforate anus associated with a recto-bulbar-cutaneous fistula.

This is the report of two newborn boys with imperforate anus associated with a long rectocutaneous fistula running deep into the scrotum and communicating, in its midportion, with the bulbar urethra. The findings are compared with those of a somewhat similar case in the literature, with some speculations as to the possible embryogenesis of the lesion.