Freeman-Sheldon syndrome (whistling face syndrome) and cranio-vertebral junction malformation producing dysphagia and weight loss

Hae Ryong Song, John F. Sarwark, Sarah Sauntry, John Grant

Research output: Contribution to journalArticle

5 Citations (Scopus)

Abstract

The whistling face syndrome has been reported, with complications of the respiratory system and feeding problems due to microstomia, since 1938. Dysphagia, however, has not previously been reported as a complication of whistling face syndrome. We report a 13-year-old with the whistling face syndrome and dysphagia of late onset who has the typical whistling face, scoliosis and multiple joint contractures. Dysphagia occurred as a result of compression at the cervicomedullary junction by a cranio-vertebral junction malformation and impingement by the tip of the odontoid process. This child had undergone multiple surgical procedures for cleft palate, clubfeet, contractures of the hip and knee and scoliosis. Halo traction and posterior occipital-cervical decompression and fusion successfully treated the dysphagia.

Original languageEnglish
Pages (from-to)272-274
Number of pages3
JournalPediatric Neurosurgery
Volume24
Issue number5
Publication statusPublished - 1996 May 1
Externally publishedYes

Fingerprint

Deglutition Disorders
Weight Loss
Scoliosis
Hip Contracture
Microstomia
Odontoid Process
Clubfoot
Singing
Traction
Cleft Palate
Contracture
Decompression
Respiratory System
Knee
Joints
Freeman-Sheldon syndrome

Keywords

  • Cranial-cervical malformation
  • Dysphagia
  • Whistling face syndrome

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Freeman-Sheldon syndrome (whistling face syndrome) and cranio-vertebral junction malformation producing dysphagia and weight loss. / Song, Hae Ryong; Sarwark, John F.; Sauntry, Sarah; Grant, John.

In: Pediatric Neurosurgery, Vol. 24, No. 5, 01.05.1996, p. 272-274.

Research output: Contribution to journalArticle

@article{d6804aad85eb449f9d57e21f8052e368,
title = "Freeman-Sheldon syndrome (whistling face syndrome) and cranio-vertebral junction malformation producing dysphagia and weight loss",
abstract = "The whistling face syndrome has been reported, with complications of the respiratory system and feeding problems due to microstomia, since 1938. Dysphagia, however, has not previously been reported as a complication of whistling face syndrome. We report a 13-year-old with the whistling face syndrome and dysphagia of late onset who has the typical whistling face, scoliosis and multiple joint contractures. Dysphagia occurred as a result of compression at the cervicomedullary junction by a cranio-vertebral junction malformation and impingement by the tip of the odontoid process. This child had undergone multiple surgical procedures for cleft palate, clubfeet, contractures of the hip and knee and scoliosis. Halo traction and posterior occipital-cervical decompression and fusion successfully treated the dysphagia.",
keywords = "Cranial-cervical malformation, Dysphagia, Whistling face syndrome",
author = "Song, {Hae Ryong} and Sarwark, {John F.} and Sarah Sauntry and John Grant",
year = "1996",
month = "5",
day = "1",
language = "English",
volume = "24",
pages = "272--274",
journal = "Pediatric Neurosurgery",
issn = "1016-2291",
publisher = "S. Karger AG",
number = "5",

}

TY - JOUR

T1 - Freeman-Sheldon syndrome (whistling face syndrome) and cranio-vertebral junction malformation producing dysphagia and weight loss

AU - Song, Hae Ryong

AU - Sarwark, John F.

AU - Sauntry, Sarah

AU - Grant, John

PY - 1996/5/1

Y1 - 1996/5/1

N2 - The whistling face syndrome has been reported, with complications of the respiratory system and feeding problems due to microstomia, since 1938. Dysphagia, however, has not previously been reported as a complication of whistling face syndrome. We report a 13-year-old with the whistling face syndrome and dysphagia of late onset who has the typical whistling face, scoliosis and multiple joint contractures. Dysphagia occurred as a result of compression at the cervicomedullary junction by a cranio-vertebral junction malformation and impingement by the tip of the odontoid process. This child had undergone multiple surgical procedures for cleft palate, clubfeet, contractures of the hip and knee and scoliosis. Halo traction and posterior occipital-cervical decompression and fusion successfully treated the dysphagia.

AB - The whistling face syndrome has been reported, with complications of the respiratory system and feeding problems due to microstomia, since 1938. Dysphagia, however, has not previously been reported as a complication of whistling face syndrome. We report a 13-year-old with the whistling face syndrome and dysphagia of late onset who has the typical whistling face, scoliosis and multiple joint contractures. Dysphagia occurred as a result of compression at the cervicomedullary junction by a cranio-vertebral junction malformation and impingement by the tip of the odontoid process. This child had undergone multiple surgical procedures for cleft palate, clubfeet, contractures of the hip and knee and scoliosis. Halo traction and posterior occipital-cervical decompression and fusion successfully treated the dysphagia.

KW - Cranial-cervical malformation

KW - Dysphagia

KW - Whistling face syndrome

UR - http://www.scopus.com/inward/record.url?scp=0029857791&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0029857791&partnerID=8YFLogxK

M3 - Article

VL - 24

SP - 272

EP - 274

JO - Pediatric Neurosurgery

JF - Pediatric Neurosurgery

SN - 1016-2291

IS - 5

ER -