scholarly journals Muenke syndrome: Medical and surgical comorbidities and long‐term management

2019 ◽  
Author(s):  
Chaya N. Murali ◽  
Donna M. McDonald‐McGinn ◽  
Tara Lynn Wenger ◽  
Carey McDougall ◽  
Bridget M. Stroup ◽  
...  
Keyword(s):  
Muenke Syndrome ◽  
Term Management

Childhood Asthma's Self-Efficacy Scale--Parental Long-Term Management

2012 ◽  
Author(s):  
Misa Iio ◽  
Kosuke Maeba ◽  
Takashi Shimazaki ◽  
Yukihiro Ohya ◽  
Koji Takenaka
Keyword(s):  
Self Efficacy ◽  
Term Management

Long-term management of resistant, fistulazing Crohn's disease by ongoing 4-weekly infliximab therapy (a case report)

2005 ◽  
Vol 43 (05) ◽  
Author(s):  
R Schwab ◽  
P Lakatos ◽  
E Schäfer ◽  
J Weltner ◽  
A Sáfrány ◽  
...  
Keyword(s):  
Crohn’S Disease ◽  
Case Report ◽  
Crohn's Disease ◽  
Infliximab Therapy ◽  
Term Management

Long-Term Management of Meningiomas

Skull Base ◽  
2007 ◽  
Vol 17 (S 1) ◽  
Author(s):  
Tiit Mathiesen
Keyword(s):  
Term Management

Muenke syndrome: long-term outcome of a syndrome-specific treatment protocol

2019 ◽  
Vol 24 (4) ◽  
pp. 415-422 ◽  
Author(s):  
Bianca K. den Ottelander ◽  
Robbin de Goederen ◽  
Marie-Lise C. van Veelen ◽  
Stephanie D. C. van de Beeten ◽  
Maarten H. Lequin ◽  
...  
Keyword(s):  
Treatment Protocol ◽  
First Year ◽  
Ventricular Size ◽  
Term Outcome ◽  
Long Term Outcome ◽  
Skull Growth ◽  
Muenke Syndrome ◽  
First Year Of Life

OBJECTIVEThe authors evaluated the long-term outcome of their treatment protocol for Muenke syndrome, which includes a single craniofacial procedure.METHODSThis was a prospective observational cohort study of Muenke syndrome patients who underwent surgery for craniosynostosis within the first year of life. Symptoms and determinants of intracranial hypertension were evaluated by longitudinal monitoring of the presence of papilledema (fundoscopy), obstructive sleep apnea (OSA; with polysomnography), cerebellar tonsillar herniation (MRI studies), ventricular size (MRI and CT studies), and skull growth (occipital frontal head circumference [OFC]). Other evaluated factors included hearing, speech, and ophthalmological outcomes.RESULTSThe study included 38 patients; 36 patients underwent fronto-supraorbital advancement. The median age at last follow-up was 13.2 years (range 1.3–24.4 years). Three patients had papilledema, which was related to ophthalmological disorders in 2 patients. Three patients had mild OSA. Three patients had a Chiari I malformation, and tonsillar descent < 5 mm was present in 6 patients. Tonsillar position was unrelated to papilledema, ventricular size, or restricted skull growth. Ten patients had ventriculomegaly, and the OFC growth curve deflected in 3 patients. Twenty-two patients had hearing loss. Refraction anomalies were diagnosed in 14/15 patients measured at ≥ 8 years of age.CONCLUSIONSPatients with Muenke syndrome treated with a single fronto-supraorbital advancement in their first year of life rarely develop signs of intracranial hypertension, in accordance with the very low prevalence of its causative factors (OSA, hydrocephalus, and restricted skull growth). This illustrates that there is no need for a routine second craniofacial procedure. Patient follow-up should focus on visual assessment and speech and hearing outcomes.

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