Vito Forte

Juvenile angiofibroma: a review of the literature and a case series report.

Juvenile angiofibroma is a rare, histologically benign tumor which occurs almost exclusively in adolescent boys. The morbidity and mortality associated with this tumor are related to its prominent vascularity and its propensity for aggressive local growth.

The use of expandable metallic airway stents for tracheobronchial obstruction in children

Expandable metallic angioplasty stents (Palmaz stent) have been implanted in the trachea and/or bronchi of seven children. Three children had severe tracheal stenosis after tracheoplasty for congenital tracheal stenosis repair, and four had tracheomalacia or bronchomalacia with or without vascular compression. The mean age at stenting was 9.7 months (range, 2 to 15 months). Balloon expandable stents were inserted into the trachea or bronchus through a 3.5-mm bronchoscope under fluoroscopic control. Initially a single tracheal stent was used for all patients except for one with obstruction in the trachea and both bronchi, in whom three stents were implanted. Three children had recurrent airway obstruction 1 month later; one was cured with a second stent; one child died 1 year later; and the other is being treated for heart disease. The others have no serious respiratory problems. The stents in all have been in place for 1 to 25 (mean, 11) months. No immediate complications were noted. Early and late bronchoscopy showed incomplete epithelialization of the stent and patches of granulation tissue on it. Two stents were removed bronchoscopically, one at the completion of treatment for tracheomalacia and the other at the time of recurrent airway obstruction. This preliminary experience indicates that expandable metallic stents have a useful role in the treatment of selected lower airway obstructions.

Choanal atresia and choanal stenosis.

Congenital narrowing of the nasal airway at the posterior choanae, which can be uni- or bilateral, is an uncommon condition in pediatric patients. The surgical management of choanal atresia varies widely in different centers. This article discusses the different surgical strategies including: dilation and stenting; trans-palatal repair; and transnasal resection utilizing endoscopic sinus surgery (ESS) techniques. The merits of stents, lasers, CT-guided surgery, and the use of additional agents including mitomycin C are reviewed, as well as the particular problems associated with managing bilateral choanal atresia in neonates.

Branchial Sinus of the Piriform Fossa: Reappraisal of Third and Fourth Branchial Anomalies

Objective: The objective of this study was to review clinical and embryologic aspects of third and fourth branchial anomalies.

Encephaloceles of the Anterior Cranial Fossa

From a total of 114 encephaloceles treated surgically at The Hospital for Sick Children in the 15 years to 1994, the case records of 17 patients with sincipital and 5 patients with basal defects were reviewed retrospectively. The condition was evident at birth in 64% of patients, while the remainder presented with either cerebrospinal fluid (CSF) rhinorrhea, nasal obstruction, or feeding difficulty. Hypertelorism affected 73% of patients. All encephaloceles were repaired transcranially, at a mean age of 2 years, usually by means of an intradural pericranial graft. Five children with gross hypertelorism underwent orbital translocation at the time of encephalocele repair. Of those not corrected, primary and secondary hypertelorism regressed in most instances where the encephalocele was treated before the age of 2 years. There were no deaths. The only case of CSF rhinorrhea occurred in a patient with a basal defect, in whom intradural repair was not possible because of adherence of diencephalic structures to the sac wall. Hypertelorism recurred in 1 patient after orbital translocation, requiring recorrection 2 years later. One patient with untreated secondary hypertelorism failed to regress after the encephalocele was excised at the age of 4 months. Developmental outcome was normal in 59% of children, whilst 18% have mild mental or physical disability, and 23% are severely impaired. A child with a sincipital or basal defect and mild hypertelorism should have the encephalocele treated in early childhood to allow the facial skeleton to remodel with growth. When an encephalocele is accompanied by gross hypertelorism or a facial cleft, one-stage correction can be undertaken safely in early childhood with minimal mortality and acceptable morbidity.

Ewing’s sarcoma of the head and neck in children

OBJECTIVE The purpose of this paper was to review our experience with Ewings sarcoma of the head and neck in children. DESIGN Retrospective chart review. SETTING The Hospital for Sick Children, Toronto, Ont., Canada. METHODS Between 1986 and 1996, 70 cases of Ewings sarcoma were identified. The medical records, roentgenographic and pathology reports were reviewed retrospectively. The gender, age of presentation, location and clinical presentation of the tumor were noted in the cases involving the head and neck. The treatment and follow-up of these patients were recorded. RESULTS Of the 70 cases of Ewings, five involved the head and neck (7.1%). The age of presentation ranged from 7.5 to 14 years. An enlarging mass in the mandible was the mode of presentation in three of the five children. Two patients had metastases at initial presentation. All patients received combination treatment regimens with chemotherapy initially, followed by adjuvant surgery and/or radiation. Follow-up ranged from 2 to 11 years. Three of five patients died of metastatic disease. Two are alive and well with no evidence of disease. CONCLUSIONS Ewings sarcoma occurs infrequently in the head and neck in children. An enlarging mass in the mandible is the most frequent mode of presentation. This tumor is treated systemically with high dose chemotherapy and locally with surgical excision where possible. In lesions that are initially unresectable and/or show a poor response to chemotherapy, radiation is used for local control. A good prognosis can be expected if the disease has not metastasized.

A New Classification System for Congenital Laryngeal Cysts

Objectives: A new classification system for congenital laryngeal cysts based on the extent of the cyst and on the embryologic tissue of origin is proposed.

Pediatric aggressive fibromatosis of the head and neck: a 20-year retrospective review

UNLABELLED Aggressive fibromatosis in children is a rare, benign condition that is locally infiltrative and destructive. It often presents as a rapidly growing, painless lump in the head and neck region. To date, only small series and case reports have been reported, and the management of the condition remains unclear. Recently, nuclear beta-catenin expression has been suggested as a tumor-specific marker for aggressive fibromatosis (desmoid). AIM The aims of the study were to review our experience of the presentation, management, and treatment outcome of pediatric aggressive fibromatosis in the head and neck and to identify the presence of the desmoid tumor marker beta-catenin within this population. METHOD The study was conducted as a retrospective case review of children diagnosed with aggressive fibromatosis in the head and neck for a period of 20 years and a review of the literature. Pathologic review of the original tumor specimens was undertaken for evidence of positive tumor margins and presence of nuclear beta-catenin expression. RESULTS A total of 10 patients (6 males, 4 females) were identified. The age at presentation ranged from 12 months to 14 years. In total, 8 patients were treated with surgery alone. This included 7 patients with extension of the tumor to the resection margin; all had good long-term outcomes with no disease progression. Two patients received chemoradiotherapy, one as primary treatment, and the other as adjuvant treatment after gross incomplete resection. Both resulted in poor outcomes requiring further treatments. Within our series of pediatric fibromatosis, only 4 cases (40%) had positive results for any nuclear beta-catenin expression, and 6 (60%) of 10 patients had negative results for beta-catenin. CONCLUSION Our experience is that total gross resection and preservation of form and function is of higher priority than achieving a negative resection margin. Pediatric fibromatosis though aggressive is still a benign condition, and careful thought should be taken before considering adjuvant chemoradiotherapy. Nuclear beta-catenin expression should not be considered a specific tumor marker for pediatric aggressive fibromatosis of the head and neck. Pediatric aggressive fibromatosis in this region may be a distinct subtype of desmoid tumor from its adult form.

Congenital conductive hearing loss

Congenital conductive hearing loss due to ossicular deformities can be treated by either rehabilitation with a hearing aid or surgical reconstruction. We present the results of exploratory tympanotomy performed in a large paediatric otolaryngology centre in 67 patients with non-serous congenital conductive hearing loss. Forty-two children had malformation of one or more ossicles without fixation of the stapes, and 19 had fixed stapes. In 26 cases, the surgeon decided not to perform surgical correction. Seven operated patients were lost to follow-up. As a group, 47 per cent of the patients who underwent reconstruction showed no significant benefit from surgery, with post-operative air-bone gaps (ABG) greater than 30 dB. Assessment of the results by pathology showed that 64 per cent of the patients with mobile stapes had an air-bone gap within 30 dB compared to only 33 per cent of the patients with fixed stapes. One patient sustained severe sensorineural loss after the procedure. Considering that exploratory tympanotomy is a relatively minimal, benign procedure but that findings during exploration may exclude the option of reconstruction (in 39 per cent of our patients), we suggest exploring the ear, but in a more realistic, informed way.

Nasal endoscopy in the treatment of congenital lacrimal sac mucoceles

PURPOSE To determine the incidence of intranasal cysts associated with lacrimal sac mucoceles and the cure rate with nasal endoscopic cyst marsupialization. DESIGN Interventional case series. METHODS SETTING University-affiliated teaching hospital. PATIENT POPULATION Twenty-five infants with non infected or infected lacrimal sac mucoceles or dacrocystitis without obvious mucocele were consecutively enrolled. INTERVENTION PROCEDURES: Management included local lacrimal massage, parenteral antibiotics, and when still symptomatic, nasolacrimal duct probing with concomitant nasal endoscopy. Intranasal cysts identified were marsupialized until the distal end of the nasolacrimal duct probe was visualized. MAIN OUTCOME MEASURES Presence of intranasal cyst identification and cure rate. RESULTS Infants were 4 days to 10 weeks old (mean 19 days). Forty-eight percent had a bluish cutaneous mass inferior and lateral to the lacrimal sac. Twenty percent were bilateral. At presentation, 76 percent had dacrocystitis. Fourteen percent had respiratory distress. Only one child responded to medical management. At endoscopy, 23 of 24 infants had ipsilateral intranasal cysts. The one child without nasal cyst had recurrent dacrocystitis and no mucocele. All children with mucocele were cured except one child with residual nasolacrimal duct obstruction. CONCLUSIONS Lacrimal sac mucoceles were almost always associated with intranasal cysts. Nasal endoscopy is a valuable addition to the treatment plan for lacrimal sac mucoceles not responding to a brief trial of massage or infantile dacrocystitis. To avoid potential complications, we recommend against waiting until infection occurs before proceeding with surgery.