Congenital cysts and fistulae of the face and the neck]

The authors review the embryological, clinical and therapeutic aspects of congenital facial and cervical cysts (C) and fistulae (F), based on a personal series of 85 cases (facial: 18, latero-cervical: 29, mediocervical: 38) observed during a 5-year period. The facial forms are the result of an incomplete coalescence of the facial buds and most often present as helical F (17/18). Laterocervical C and F are due to abnormal evolution of branchial clefts; the main clinical forms are related to anomalies of the 2nd branchial cleft (24/29), usually presenting as sinus localized at the anterior border of the lower third of the sternocleidomastoid muscle (8) and amygdaloid cysts. Thyroglossal duct cysts are the most frequent of the medio-cervical C and F (35/38); they usually present as a mediocervical cyst in the thyro-hyoid space which may be revealed by an infection or a fistulization. The only appropriate treatment of congenital facial and cervical C and F is surgery providing that the resection is meticulous with complete resection of the fistula in order to avoid relapse. Complete resection also suppresses the risk of secondary malignant degeneration of amygdaloid and thyroglossal duct cysts.     

Pharyngeal cleft sinuses and cysts, and other benign neck lesions

Few pediatricians can expect to acquire great personal experience in dealing with many of the aforementioned lesions. Nevertheless, an appreciation of the usual presentation and natural history of most of the entities will usually permit an accurate diagnosis and guide to management in all but the most obscure conditions.     

Congenital tumours involving the head, neck and central nervous system

Congenital intracranial tumours are uncommon and differ from those occurring in older children in clinical presentation, imaging characteristics and prognosis. These tumours are often detected incidentally on routine prenatal US and/or fetal MRI. Hence, the paediatric radiologist should be familiar with the features of those lesions that should be included in the differential diagnosis. In general, the prognosis of these conditions is poor owing to large tumour size and the limitations of adjuvant therapy at such a young age. Congenital lesions involving the head and neck region require a meticulous imaging approach using both US and MRI techniques to better guide prenatal planning and fetal or neonatal surgical procedures.     

Congenital bilateral plexiform neurofibromas of the cavernous sinuses

We report the CT and MRI findings of congenital bilateral plexiform neurofibromas of the cavernous sinuses in a 2-month-old girl. Contrast-enhanced CT showed enhancement of masses in both cavernous sinuses and enlargement of both superior orbital fissures. On MRI the masses were isointense with muscle on T1-weighted images, hypointense on T2-weighted images and showed strong homogeneous enhancement on contrast-enhanced T1-weighted images. A dural tail sign was observed. The diagnosis was proven by biopsy.     

Thymic cysts of the neck

Thymic cysts of the neck are rare clinical entities. Only 45 children with cervical thymic cysts have been reported in the literature so far; 2 more cases are reported here. Both presented as cystic swellings in the left side of the neck and were pre-operatively diagnosed as a cystic hygroma and a solitary thyroid nodule. Investigations included blood counts, ultrasonograms, thyroid scans, and aspiration cytology. Both lesions could be easily excised by the cervical route. The final diagnosis was made only after histopathological examination. The relevant literature on the aetiology, pathology, clinical features, and treatment is reviewed. Offprint requests to: K. Radhakrishna     

Congenital pyriform sinus fistula: A cause of acute left-sided suppurative throiditis and neck abscess in children

Acute bacterial thyroiditis or neck abscesses in children can be caused by infection through pyriform sinus fistulae which usually originate from the tip of the left pyriform sinus. They are thought to be remnants of either the third or fourth pharyngeal pouches. CT, ultrasound and gastrointestinal contrast studies are all useful in clarifying the pathological process and showing the extent.     

Congenital tracheo-oesophageal fistula of unusual length

Most congenital tracheo-oesophageal fis fistulae are short; the purpose of this report is to describe an unusually long fistula. Despite its length, it had the usually encountered obliquity. Correspondence to: S. K. Mitra     

Congenital rectovestibular fistula. An unusual diagnosis that should not be ignored]

Rectovestibular fistula without anorectal malformation is a rare anomaly. OBSERVATION: A six-week-old baby was hospitalized for a greater labium abscess. Stools leakage was secondary noted in the vagina and in abscess area. Clinical examination under anesthesia concluded to a congenital rectovestibular fistula with secondary drainage in the greater labium. Surgical treatment without colostomy led to recovery. COMMENTS: Congenital recto-vestibular fistula is a difficult diagnosis. It should be evoked when vaginal fecal leakage occurs in the absence of anorectal malformation or perineal injury. Fistula location is often confirmed by clinical examination under anesthesia. Treatment is surgical and allowed recovery without any functional damage.     

Evaluation of head and neck masses

There is always concern for a malignancy or other serious disease when a child presents with a chief complaint of a neck mass. However, a neck mass in child is commonly inflammatory or congenital rather than neoplastic. A complete history and physical exam will often provide enough information to guide management and reassure the concerned parents. In some cases, laboratory tests, radiologic studies or a surgical procedure may be necessary to determine a specific diagnosis and treatment plan. An organized and systematic approach is recommended and detailed in this review.     

Pediatric head and neck rhabdomyosarcoma.

Rhabdomyosarcoma (RMS), the most common malignant soft-tissue tumor in the pediatric population, occurs most often in the head and neck region. Major advancements in the management of these aggressive tumors have been achieved in the last 20 years on three fronts: chemotherapy, radiation therapy, and surgery. Surgery, with the advent of skull-base, craniofacial, and free-tissue-transfer reconstructive techniques, has once again become part of the primary management of these tumors. Prior to 1960, RMS was uniformly fatal; now, with the introduction of multimodality treatment regimens, two thirds of patients can expect long-term survival.     

Vascular anomalies of the head and neck

The head and neck are the most common site of involvement for vascular tumors and malformations, with more than half of all vascular anomalies seen in this region. Lesions in this location can cause significant disfigurement and can be associated with airway obstruction, impairment in vision or hearing, swallowing disorders and hemorrhage. Accurate diagnosis is critical in determining treatment, and interdisciplinary care is essential for optimal management. We review clinical and imaging features that are key to establishing the correct diagnosis, and review treatment modalities, with emphasis on interventional and surgical procedures.     

Malignant neoplasms of the head and neck

Head and neck masses represent a common clinical entity in children. In general, these masses are classified as developmental, inflammatory, or neoplastic. Having a working knowledge of lesions within this region and conducting a thorough history and physical examination generally enables the clinician to facilitate an appropriate workup and establish a diagnosis. The differential diagnosis is broad, and expeditiously distinguishing benign from malignant masses is critical for instituting a timely multidisciplinary approach to the management of malignant lesions. Neoplasms of the head and neck account for approximately 5% of all childhood malignancies. A diagnosis of malignancy may represent a primary tumor or metastatic foci to cervical nodes. In this review, we discuss the general approach to evaluating suspicious masses and adenopathy in the head and neck region and summarize the most common malignant neoplasms of the head and neck with regard to their incidence, clinical presentation, diagnostic evaluation, staging, and management. Thyroid, parathyroid, and salivary gland tumors are discussed elsewhere in this issue of Seminars in Pediatric Surgery.     

Management of common head and neck masses

Head and neck masses are a common clinical concern in infants, children, and adolescents. The differential diagnosis for a head or neck mass includes congenital, inflammatory, and neoplastic lesions. An orderly and thorough examination of the head and neck with an appropriate directed workup will facilitate the diagnosis. The most common entities occur repeatedly within the various age groups and can be differentiated with a clear understanding of embryology and anatomy of the region, and an understanding of the natural history of a specific lesion. Congenital lesions most commonly found in the pediatric population include the thyroglossal duct cyst and the branchial cleft and arch anomalies. The inflammatory masses are secondary to local or systemic infections. The most common etiology for cervical adenopathy in children is reactive lymphadenopathy following a viral or bacterial illness. Persistent adenopathy raises more concerns, especially enlarged lymph nodes within the posterior triangle or supraclavicular space, nodes that are painless, firm, and not mobile, or a single dominant node that persists for more than 6 weeks should all heighten concern for malignancy. In this review, we discuss the current principles of surgical management of the most common head and neck masses that present to pediatricians and pediatric surgeons.     

Midline dermal sinuses and cysts and their relationship to the central nervous system.

Sixty-one children (below 12 years) with midline dermal inclusions of the cranium and spine were operated on at the Red Cross War Memorial Children's Hospital between 1969-1990. The bregmatic area was the most common position for superficial cysts (33). Eight children had sinuses or cysts near the external occipital protuberance, 2 had isolated fourth ventricular cysts and 1 had a cyst in the quadrigeminal plate cistern. Fifteen children had spinal dermal inclusions, 13 of these were in the lumbosacral area, there was 1 sinus in the cervical spine and another in the midthoracic area attached to an intramedullary cyst. Two children had frontal sinuses, one of which was connected to an interhemispheric dermoid cyst and a lipoma of the corpus callosum. A midline swelling or sinus was the most common clinical presentation. Four out of 15 spinal inclusions and 1/11 occipital sinuses had a meningitic history. Five of 11 of the posterior fossa inclusions had raised intracranial pressure and signs suggestive of cerebellar tumor or abscess. Not one of the 33 bregmatic lesions had any connection to the central nervous system. MRI has proved useful in diagnosing both dermal sinuses and cysts, but CT scanning was our standard investigation. Plain x-ray revealed bony abnormalities in only 60% of our patients with spinal sinuses. We feel that all dermal sinuses or cysts in the midline should be surgically explored after CT or MRI scanning. Lesions mistaken for bregmatic cysts have included hemangiomas (4), hamartomas (2), an encephalocele through the anterior fontanelle (1) and lipomas (2).(ABSTRACT TRUNCATED AT 250 WORDS)