Pediatric Otolaryngology Challenges in Multi-System Disease, An Issue of Otolaryngologic Clinics

Preface: Treating Common Disorders in Complex Children Austin S. Rose xi

Current Management of Infantile Hemangiomas and Their Common Associated Conditions Larry D. Hartzell Lisa M. Buckmiller 545

This article reviews the most current practice guidelines in the diagnosis and treatment of infantile hemangiomas. Several systemic conditions that can be associated with hemangiomas, such as PHACES syndrome, are also discussed. Propranolol has become an effective first-line treatment, and protocols for its use as well as its potential risks are outlined.

Otolaryngologic Manifestations of Craniofacial Syndromes Laura H. Swibel Rosenthal Nadieska Caballero Amelia F. Drake 557

This review describes important aspects of the most commonly encountered craniofacial syndromes. The goal is to provide otolaryngologists and other health care providers with critical information necessary to manage these patients appropriately. The algorithm provided in this article should be helpful in guiding the treatment of craniofacial patients based on their unique otolaryngologic characteristics. The principles highlighted in the algorithm can be applied to other craniofacial syndromes not addressed here, including Pierre Robin sequence and Down syndrome.

Otolaryngologic Manifestations of Skeletal Dysplasias in Children Sofia Lyford-Pike Julie Hoover-Fong David E. Tunkel 579

This article reviews some of the otolaryngologic manifestations of skeletal dysplasias. Achondroplasia is discussed most comprehensively. Skeletal dysplasias are bone and cartilage disorders that disrupt the development of the long bones, craniofacial skeleton, and vertebral column, with the most notable characteristic being short stature. Children with skeletal dysplasias have various medical problems. These children often develop head and neck manifestations of their disorders. Hearing loss, middle ear disease, and respiratory difficulties are seen in these children. Otolaryngologists must be knowledgeable about these disorders to diagnose, treat, and appropriately refer children with skeletal dysplasias.

The Otolaryngologist's Approach to the Patient with Down Syndrome Regina Rodman Harold S. Pine 599

As more patients with Down syndrome are living into adulthood, attention has focused on health factors that affect the quality of the patient's life and their ability to reach full potential. Patients with Down syndrome have several morphologic abnormalities that predispose them to problems with the ear, nose, and throat, and appropriate treatment can have a significant impact on the quality of life of these patients. Otolaryngologists are likely to see many patients with Down syndrome throughout their careers. This article reviews the literature to provide information and recommendations regarding management of Down syndrome.

Allergic Fungal Sinusitis in Children Brian D. Thorp Kibwei A. McKinney Austin S. Rose Charles S. Ebert Jr 631

Allergic fungal sinusitis (AFS) is a subtype of eosinophilic chronic rhinosinusitis (CRS) characterized by type I hypersensitivity, nasal polyposis, characteristic computed tomography scan findings, eosinophilic mucus, and the presence of fungus on surgical specimens without evidence of tissue invasion. This refractory subtype of CRS is of the great interest in the pediatric population, given the relatively early age of onset and the difficulty in managing AFS through commercially available medical regimens. Almost universally, a diagnosis of AFS requires operative intervention. Postoperative adjuvant medical therapy is a mainstay in the treatment paradigm of pediatric AFS.

Multisystem Disease and Pediatric Laryngotracheal Reconstruction Jeremy D. Meier David R. White 643

Laryngotracheal reconstruction can be technically challenging. Successfully managing the patient's medical comorbidities is essential. Children undergoing laryngotracheal reconstruction rarely present with isolated subglottic stenosis; many have associated multisystem disorders. Effectively managing the patient enables successful outcomes after airway reconstruction.

A Review of the Evaluation and Management of Velopharyngeal Insufficiency in Children James M. Ruda Paul Krakovitz Austin S. Rose 653

This article highlights the most common causes of velopharyngeal insufficiency (VPI), and discusses routine evaluation and treatment algorithms for the managment of VPI in children. VPI is a multifactorial condition that occurs commonly in syndromic and non-syndromic children. The most common features of VPI are audible hypernasal speech, facial grimacing, decreased speech intelligibility, nasal regurgitation, and nasal emission from failure to produce oronasal separation. Work-up of VPI typically involves radiologic and endoscopic testing performed with the assistance of a speech-language pathologist. Management of VPI involves initial speech therapy followed by operative repair with sphincter or pharyngeal flap pharyngoplasty, if needed.

Recurrent Respiratory Papillomatosis Naren N. Venkatesan Harold S. Pine Michael P. Underbrink 671

Recurrent respiratory papillomatosis (RRP) is a rare, benign disease with no known cure. RRP is caused by infection of the upper aerodigestive tract with the human papillomavirus (HPV). Passage through the birth canal is thought to be the initial transmission event, but infection may occur in utero. HPV vaccines have helped to provide protection from cervical cancer; however, their role in the prevention of RRP is undetermined. Clinical presentation of initial symptoms of RRP may be subtle. RRP course varies, and current management focuses on surgical debulking of papillomatous lesions with or without concurrent adjuvant therapy.

Pierre Robin Sequence: Evaluation, Management, Indications for Surgery, and Pitfalls Andrew R. Scott Robert J. Tibesar James D. Sidman 695

This article reviews the various treatment methods for airway obstruction and feeding difficulty in infants with Pierre Robin Sequence (PRS), and highlights the benefits and limitations of early mandibular distraction osteogenesis in particular as a way of managing both airway obstruction and feeding difficulty in these children.

Endoscopic Skull Base Techniques for Juvenile Nasopharyngeal Angiofibroma Adam M. Zanation Candace A. Mitchell Austin S. Rose 711

This review focuses on the anatomy and techniques for endoscopic resection of juvenile nasopharyngeal angiofibroma (JNA), as well as adjunctive and potential alternative treatments. JNA is a benign but locally aggressive vascular tumor that primarily affects adolescent boys. Traditionally, these tumors have been removed via open surgical approaches. Recent advances in endoscopic equipment and techniques have enabled the endoscopic surgical excision of these tumors with favorable outcomes and decreased morbidity. At our institution as at others, we have achieved successful outcomes with transnasal endoscopic resection of JNA, including tumors with significant extension into adjacent compartments.

Index 731