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[Neonatal trismus]
V Abadie1, G Chéron, A Madjiidi
1Département de pédiatrie, Hôpital des Enfants-Malades, Paris, France.
Insights
Neonatal trismus, a rare condition causing jaw muscle contraction, is a poor prognostic sign. It often indicates underlying developmental defects and requires specialized investigations and resuscitation techniques.
Area of Science:
- Neonatology
- Pediatric Neurology
- Developmental Biology
Context:
- Trismus, characterized by sustained jaw-closing muscle contraction, is uncommon in neonates.
- It can be associated with various congenital anomalies and neurological dysfunctions.
- This study investigates a cohort of neonates presenting with trismus.
Purpose:
- To characterize the clinical presentation and associated conditions in neonates with trismus.
- To evaluate the diagnostic investigations employed in these patients.
- To determine the prognostic implications of neonatal trismus.
Summary:
- Twenty-four neonates (1-12 days old) with trismus were studied, exhibiting severe sucking/swallowing difficulties and apnea. Twenty-one had facial anomalies and brainstem dysfunction, including Robin sequence, Hanhart syndrome, and Moebius syndrome. Gastroesophageal reflux and increased vagal reactivity were common. Five patients had central nervous system anomalies.
- Specialized investigations included polysomnography, Holter monitoring, and imaging. Eight patients died within the first year of life.
- Neonatal trismus is a significant indicator of poor prognosis, necessitating specialized diagnostic approaches and often resuscitation.
Impact:
- Highlights the association between neonatal trismus and severe congenital anomalies and neurological deficits.
- Emphasizes the need for comprehensive, specialized investigations in affected neonates.
- Underscores the critical prognostic value of trismus in the neonatal period, guiding clinical management and parental counseling.
Background:
Trismus is caused by sustained contraction of the jaw-closing muscles. It is rare in the neonate and can be a part of developmental defects.
Patients And Methods:
Twenty-four neonates, aged 1 to 12 days, were admitted from 1980 to 1992 because they suffered from trismus. All of them had severe difficulties of sucking and/or swallowing, some (12/24) had repeated episodes of apnoea requiring tracheotomy. Specialized investigations included transcutaneous PO2 and PCO2 monitoring, polygraphic recordings during sleep, 24-hour Holter monitoring, ocular compression test, barium swallow, manometry and 24-hour pH monitoring of the distal esophagus, electromyography of muscles involved in swallowing and several imaging techniques.
Results:
Twenty-one patients had anomalies of the facies and brain stem dysfunctions. They were classified as Robin sequence (14 patients) with (five patients) or without (nine patients) micrognathia, cleft palate and glossoptosis, as Hanhart syndrome (four patients) and Moebius syndrome (three patients). The last three patients had arthrogryposis (two patients) and Stuve-Wiedeman syndrome. Twenty-two of 24 patients had severe gastroesophageal reflux, 15 of 20 had increased vagal reactivity and five of 24 had anomalies of the central nervous system. Eight patients died during the first year of life.
Conclusion:
Neonatal trismus is a poor prognostic sign, requiring specialized investigations and frequently resuscitation techniques.