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Clinical experience with infants with Robin sequence: a prospective study
I L Marques1, T V de Sousa, A F Carneiro
1Hospital de Reabilitação de Anomalias Craniofaciais da Universidade de São Paulo, Bauru, SP, Brazil. ilza_marques@uol.com.br
Insights
Robin sequence (RS) patients, particularly those with probable isolated RS and type 1 obstruction, often improve without surgery. Nasopharyngeal intubation (NPI) is recommended for significant respiratory and feeding issues in RS types 1 and 2.
Area of Science:
- Craniofacial Medicine
- Pediatric Surgery
- Neonatology
Background:
- Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and airway obstruction.
- Early identification and management are crucial for improving outcomes in infants with RS.
Purpose of the Study:
- To investigate the clinical progression of infants diagnosed with Robin sequence during their first six months of life.
- To evaluate the effectiveness of various treatment modalities for airway obstruction in RS patients.
Main Methods:
- A prospective longitudinal study was conducted involving 62 infants with RS.
- Patients were categorized based on RS presentation: probable isolated RS (PIRS), syndromic RS, and RS with neurological involvement.
- Respiratory obstruction severity was assessed via nasopharyngoscopy, guiding interventions such as nasopharyngeal intubation (NPI), glossopexy, and tracheostomy.
Main Results:
- Prone position treatment (PPT) or NPI served as definitive management for 75.8% of PIRS cases and 52% of syndromic RS cases.
- A significant proportion of infants with type 1 obstruction (51.1%) improved with PPT alone, while 25.5% required NPI.
- Infants treated exclusively with NPI demonstrated positive weight, length, and neuromotor development.
Conclusions:
- The majority of PIRS patients and those with type 1 obstruction experience favorable outcomes without surgical intervention.
- Nasopharyngeal intubation (NPI) is indicated as a primary treatment for significant respiratory and feeding challenges in Robin sequence patients with type 1 and type 2 obstruction.
Objective:
To study the clinical course of patients with Robin sequence (RS) during the first 6 months of life.
Design:
A longitudinal prospective study of children with RS.
Setting:
Hospital de Reabilitação de Anomalias Craniofaciais, Universidade de São Paulo, Bauru-SP, Brazil, 1997 and 1998.
Patients:
Sixty-two children were studied from hospital admission to 6 months of age. Thirty-three (53.2%) presented with probable isolated RS (PIRS), 25 (40.3%) presented with syndromes or other malformations associated with RS, and 4 (6.5%) presented with RS with neurological involvement.
Interventions:
The type of respiratory tract obstruction was defined by nasopharyngoscopy. The patients with type 1 and type 2 obstruction underwent nasopharyngeal intubation (NPI), and glossopexy was indicated in patients with type 1 obstruction who did not show clinical improvement with this procedure. Tracheostomy was indicated in patients with type 2 obstruction who did not show a good course after NPI, in patients with type 1 obstruction who did not show good course after glossopexy, and in patients with type 3 and type 4 obstruction.
Results:
Prone position treatment (PPT) or NPI was the definitive treatment in 25 cases (75.8%) of PIRS and in 13 cases (52%) of syndromes or other malformations. Among the children with type 1 obstruction, 24 (51.1%) were submitted exclusively to PPT and 12 (25.5%) to NPI. With the type 2 groups, only one (12.5%) received PPT, and three (37.5%) were treated exclusively with NPI. All 15 infants treated exclusively with NPI (24.4%) presented with good weight, length, and neuromotor development.
Conclusions:
Most patients with PIRS and type 1 obstruction improved without surgical intervention. NPI should be the initial treatment in all patients with RS with type 1 and type 2 obstruction who present with important respiratory and feeding difficulties.