Pilomatrixoma of the head and neck in children.

OBJECTIVE: Pilomatrixomas are benign skin neoplasms of hair follicle origin. They are one of the most common superficial masses of the head and neck excised in children. Although the entity has been well studied in the literature, few studies have been undertaken to evaluate the clinical characteristics of head and neck pilomatrixomas specifically in children. The purpose of this study was to review the clinical characteristics and management of children presenting with pilomatrixomas of the head and neck at a large tertiary care pediatric hospital. STUDY DESIGN: A retrospective chart review was performed of all patients with histologically confirmed pilomatrixoma of the head and neck excised during a 6-year period (1992-1997) at the Children's Hospital of Philadelphia. RESULTS: Ninety-one cases of pilomatrixoma were confirmed in 86 patients. The age range was 5 months to 17 years. The median age at time of excision was 6.0 years. The most common sites of occurrence were the cheek (36%), neck (20%), periorbital region (14%), and scalp (9%). The male to female ratio was 1:1.5. Multiple lesions were found in 8.2% of patients. Surgical excision was curative in all cases. CONCLUSION: Pilomatrixoma is a cutaneous neoplasm that is one of most common causes of superficial head and neck masses in children. Although the presurgical diagnosis may be difficult in some cases, pilomatrixoma must be kept in the differential of superficial head and neck masses in children. Surgical excision is almost always curative.

Traumatic perilymphatic fistulas in children: etiology, diagnosis and management.

Post-traumatic perilymphatic fistulas have been described following ear and temporal bone injury, particularly in the setting of temporal bone fractures. However, indications for exploratory surgery in cases of trauma without temporal bone fracture are vague and not well described. We describe three children who presented with symptoms suggestive of perilymphatic fistula (PLF) without an associated temporal bone fracture: two with penetrating tympanic membrane injuries and one with blunt temporal bone trauma. All had symptoms of hearing loss and vestibular disturbance. Two of the children cooperated with ear-specific audiologic assessment, which demonstrated sensorineural hearing loss (SNHL) on the traumatized side. The third child showed audiometric evidence of a SNHL on the injured side, but due to his age, the degree of severity of the SNHL was unable to be appropriately addressed prior to the patient being surgically managed. All three children underwent exploratory surgery and were found to have bony defects in the region of the oval window. All were repaired with fascial grafts to the oval and round windows with complete resolution of vestibular symptoms. However, two of the three patients with documented post-operative audiograms suffered from persistent SNHL on the injured side. We conclude that exploratory middle ear surgery is indicated in patients suffering from blunt or penetrating temporal bone or middle ear trauma who demonstrate persistent vestibular symptoms, sensorineural hearing loss or radiographic evidence of oval window pathology. As this is a limited number of patients, a larger series may be warranted to study the actual incidence of post-traumatic PLF in the child with persistent hearing loss and vertigo after head or ear trauma.

Medial medullary injury during adenoidectomy.

We report medullary injury during adenoidectomy in two children who received injections of local anesthetic agents into the operative bed. Initial manifestations included hemiparesis, nystagmus, and ataxia. Magnetic resonance imaging showed hemorrhagic, paramedian medullary lesions in both patients. The mechanism of injury is likely to be injection of fluid into the medulla.

Melanotic neuroectodermal tumor of infancy (MNTI) of the hard palate: presentation and management.

OBJECTIVE: To discuss the presentation and management of melanotic neuroectodermal tumor of infancy (MNTI) of the hard palate. METHOD: Case presentation and literature review. CASE: A 6-month-old girl presented with a slow growing, non-tender anterior oral hard palate mass. Radiologic imaging revealed a well-circumscribed cystic lesion containing teeth. After excision, histopathologic and electron microscopic evaluation revealed MNTI. No recurrence was seen at 12-month follow-up. CONCLUSIONS: This case and a review of the literature reveal MNTI to be a rare, benign hard palate tumor, which may present as a smooth, firm, painless, slow-growing anterior palatal lesion. Imaging reveals a well-circumscribed cystic lesion. Complete excision should be curative. Management requires attention to the potential need for palatal reconstruction, orthodontic care and correction of secondary nasal deformities.

Communication disorders in the 22Q11.2 microdeletion syndrome.

The 22q11.2 microdeletion syndrome is a genetic disorder that is being recognized with increasing frequency. Confirmation of the diagnosis can be made using fluorescence in situ hybridization. Many medical and developmental problems are present in children with this syndrome. Communication disorders are among the most common features of this syndrome and include articulation, language, resonance, and voice problems. The purpose of this paper is to provide a description of the communicative and developmental features in a sample of children with the 22q11.2 microdeletion syndrome seen for evaluation. Because communication and feeding disorders may be presenting features of this syndrome, speech and language pathologists must be familiar with this syndrome and its various characteristics. Awareness of these features and a multidisciplinary approach are necessary for the identification and treatment of the complex communicative and medical problems present in this population.

Doxycycline sclerotherapy of benign lymphoepithelial cysts of the parotid: a minimally invasive treatment.

Benign lymphoepithelial cysts (BLCs) of the parotid gland are associated with human immunodeficiency virus infections in both children and adults. These cysts may become painful and unsightly, often initiating a request for therapeutic intervention. There are several treatment options described in the literature. We report the use of doxycycline sclerotherapy in the treatment of a child with BLCs of the parotid.

Nontuberculous mycobacterial cervical adenitis.

Granulomatous inflammation is a common finding in pathologic evaluation of surgically excised chronic lymphadenopathy in children. Confusion exists regarding diagnosis and management of these lesions. Over a 10-year period at The Children's Hospital of Philadelphia, a total of 81 children were identified with biopsy-confirmed granulomatous lesions of the head and neck, with nontuberculous mycobacteria (NTM) accounting for 67 of the cases. The typical presentation was that of a nontender mass in the cervicofacial area present for weeks to months, unresponsive to antimicrobials. All underwent surgical excision, which was curative in 54 patients; 13 children required additional procedures. This paper reviews NTM, its typical clinical presentation, difficulty in diagnosis, and the methods of treatment.

Periauricular cysts and sinuses.

Periauricular cysts, sinuses, and fistulas occur commonly in the pediatric population. They arise from developmental defects of the first branchial cleft and first branchial arch. In most instances the diagnosis and management of these conditions are straightforward, but exceptional presentations sometimes occur. Failure to recognize these unusual cases may result in inadequate treatment and subsequent recurrence, and even if the correct diagnosis is made, surgical management of these lesions may be complicated. A series of 15 cases of periauricular congenital lesions is reviewed, of which three cases illustrating a diagnostic or surgical challenge are presented. The embryology, presentation, and management of these anomalies are discussed. This is one of the largest series of first branchial cleft anomalies reported in the literature, and our paper uniquely discusses first branchial cleft anomalies and preauricular sinuses together, with an emphasis on the surgical management of facial nerve, external ear, and middle ear involvement.

The effects of gelatin film stents in the middle meatus.

Controversy exists regarding the management of the middle meatus after pediatric functional endoscopic sinus surgery (FESS). To prevent adhesions following pediatric FESS, gelatin film stenting of the middle meatus has been recommended. The effects of stenting, however, have not been established. Fifty-one children with similar degrees of bilateral sinus disease had a gelatin film stent placed in one middle meatus on completion of FESS, while the opposite meatus was not stented. Two to three weeks later at the time of a second, staged procedure, the sides were compared for the presence of the stent, adhesions, granulaion tissue, and patency of the maxillary sinus ostia. In 11 children the postoperative findings were more severe in the side without the stent, whereas in 29 children they were more severe in the stented side. There was no difference between the sides in 11 children. Although gelatin film stenting benefits some children, it should not be used routinely following pediatric FESS but should be reserved for children who are predisposed to develop adhesions or have poor prognostic factors, such as immunodeficiency and ciliary dyskinesia.

Effect of otitis media with effusion on gross motor ability in preschool-aged children: preliminary findings.

OBJECTIVE: To investigate whether gross motor skills in preschool-aged children 24 to 60 months old with otitis media with effusion (OME) are different from those of preschool children without OME. CHILDREN AND METHODS: The gross motor portion of the Peabody Developmental Motor Scales (PDMS-GM) was used to compare 13 children with OME before and after tympanostomy tube placement to 12 children without OME. RESULTS: The children with OME had significantly reduced scores preoperatively compared to those without OME on the PDMS-GM. After surgery, the children with OME had higher scores than those without OME, indicating an accelerated rate of development. CONCLUSIONS: Balance and motor development are additional factors to be considered in the medical and surgical management of the young child with chronic OME. Chronic OME may represent an additional problem for young children with existing motor deficits or other disabilities.

Imaging of granulomatous neck masses in children.

Non-tuberculous mycobacterial (NTM) infection is the most common cause of granulomatous inflammation in pediatric neck masses. Diagnosis relies upon culture, acid-fast bacilli (AFB) staining, chest radiograph, purified protein derivative (PPD) test, and clinical features. Computed tomography (CT) and magnetic resonance (MR) imaging may provide valuable information in the work-up of children with cervical masses. We reviewed 11 CT and 5 MR studies of children with a clinical diagnosis of NTM infection. Specific findings included stranding of the subcutaneous fat, thickening and enhancement of the overlying skin, obliteration of the tissue palnes, and multichambered masses. One patient had calcifications within the mass. MR with contrast better demonstrated the soft tissues and is our recommended imaging modality, although CT is more likely to detect calcifications within the neck mass.

Maturational influences on suppression of vocal cord function by anesthesia.

Vocal cord abduction is a complex action that occurs with inspiration and is controlled by the respiratory center in the brainstem. A study was conducted to evaluate the maturational influences on the effect of anesthesia on vocal cord movement. Rabbits aged 15, 30, 45, and 60 days were used to study this problem. The animals were equilibrated to varying concentrations of halothane, and vocal cord movements were documented. In all age groups the vocal cord motion increased with increasing concentration of halothane. The amount of vocal cord movement also increased with the increasing age of the animal. These effects, however, were independent of each other. There did not appear to be any interaction between the concentration of halothane and the age of the animal. These findings are discussed in light of the apparent absence of active vocal cord motion observed in some children under a general anesthetic.

Direct laryngoscopy in children: rigid and flexible fiberoptic.

Examination of the child's larynx is mandatory in the management of pediatric airway problems. The techniques used to perform this examination have evolved over time and now present the airway specialist with choices that can be tailored to each specific situation. Traditionally, rigid direct laryngoscopy (RDL) has been used to evaluate the pediatric larynx. More recently, flexible fiberoptic laryngoscopy (FFL) has been used to visualize the child's airway. RDL and FFL each play a role in the evaluation of the pediatric larynx. Expertise with both techniques is mandatory for the otolaryngologist managing respiratory disorders in children. FFL has become our technique of choice to evaluate the pediatric larynx especially when airway dynamics are of concern. RDL remains the preferred technique when laryngeal/tracheal surgery is planned or establishment and protection of the airway with intubation or bronchoscopy is required.

Vocal cord injection in children with unilateral vocal cord paralysis.

Unilateral vocal cord paralysis (UVCP) in children is uncommon and rarely leads to serious sequelae. However, on rare occasions, it can present with severe aspiration and dysphonia. Several therapeutic techniques have been used in adults with UVCP, but the reported alternatives in children have been much more limited. Observation and speech therapy are the standard treatment. We describe three children with UVCP and severe aspiration who were treated with vocal cord injection. The treatment indications, clinical courses, and outcomes of the three cases are detailed. The injection of vocal cords in children is discussed, with an emphasis on those aspects unique to the management of UVCP in pediatric patients. Alternative surgical treatment modalities are also presented. Vocal cord injection is an effective and viable therapeutic option for the management of UVCP in certain pediatric patients with severe aspiration and dysphonia.

Familial occurrence of pleomorphic adenoma.

Pleomorphic adenoma is the most common neoplasm found in the parotid gland. The familial occurrence of any salivary gland neoplasm is rare. There are 8 previous reports in the literature of familial occurrence of salivary gland tumors. Two of these report the occurrence of pleomorphic adenoma of the parotid gland in siblings; all 4 patients were adults. We report here an 11-year-old male who presented with a left parotid mass and underwent parotidectomy. The mass proved to be a pleomorphic adenoma. His sister presented 9 years later at the age of 15 years with a similar mass also in the left parotid area. She underwent superficial parotidectomy which revealed the mass to be a pleomorphic adenoma. We believe these are the youngest siblings reported to date in which familial pleomorphic adenoma has occurred.

Salivary gland neoplasms in children: a 10-year survey at the Children's Hospital of Philadelphia.

Fifteen salivary gland tumors were treated at The Children's Hospital of Philadelphia between 1982 and 1991. Eight of these lesions were malignant and 7 were benign. All of the benign tumors were pleomorphic adenomas. For these, superficial parotidectomy or excision of the submandibular gland was the treatment of choice. One child had recurrence 2 years after her initial surgery. Among the malignant lesions, mucoepidermoid carcinoma was diagnosed in 5 children, and acinic cell carcinoma in 3. Six malignant tumors involved the parotid gland, while 2 originated in the submandibular salivary gland. Superficial or total parotidectomy, or excision of the submandibular gland was performed, according to the nature and the location of the lesion. The facial nerve was sacrificed in one patient because of extensive involvement of the nerve. A 2-year survival rate of 100% was achieved, and all the patients were free of disease at the end of the follow-up period. Successful management of salivary gland lesions in children requires a high index of suspicion of possible malignancy and complete surgical removal as the initial treatment. Radiation therapy is recommended in the management of those patients with microscopic residual tumor and/or nodal involvement.

Congenital tracheal stenosis. The otolaryngologist's perspective.

Congenital tracheal stenosis is a rare congenital anomaly, with less than 70 reported cases in the literature. The presenting signs and symptoms of stridor, recurrent pneumonia, and respiratory distress are commonly seen in other conditions. The rarity of congenital tracheal stenosis and the diverse presentations make accurate early diagnosis difficult and frequently lead to inappropriate treatment. We treated three patients with congenital tracheal stenosis who presented with different sites of stenosis. Each patient displayed different symptoms and required individualized management. The treatment of congenital tracheal stenosis depends on identifying the site and extent of the stenosis. We reviewed the embryogenesis and treatment of this abnormality and developed a new classification system that will aid in the management of congenital tracheal stenosis.