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A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment algorithm.

Paes EC, van Nunen DP, Speleman L, Muradin MS, Smarius B, Kon M, Mink van der Molen AB, van der Molen AB, Niers TL, Veldhoen ES, Breugem CC - Clin Oral Investig (2015)

Bottom Line: Forty-four infants (59%) were found to have been treated conservatively.RS is a heterogenic group with a wide spectrum of associated anomalies.As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable.

View Article: PubMed Central - PubMed

Affiliation: Department of Plastic, Reconstructive and Hand Surgery, Wilhelmina Children's Hospital, Utrecht, The Netherlands. emmapaes@gmail.com.

ABSTRACT

Objectives: Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution's approach and a review of the current literature.

Material and methods: A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996-2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS.

Results: Forty-four infants (59%) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25%, p = .014). A mandibular distraction was conducted in 24% (n = 18) of cases, a tracheotomy in 9% (n = 7), and a tongue-lip adhesion in 8% (n = 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described.

Conclusions: RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended.

Clinical relevance: We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics.

No MeSH data available.


Related in: MedlinePlus

Use of a nasopharyngeal airway as conservative treatment option in a 1-month-old infant with RS
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Related In: Results  -  Collection


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Fig1: Use of a nasopharyngeal airway as conservative treatment option in a 1-month-old infant with RS

Mentions: The majority (59 %, n = 44) of the infants admitted to our hospital could be successfully managed conservatively (Table 2). This consisted of side/prone positioning, temporary supplemental oxygen or usage of continuous positive airway pressure (CPAP), a mayotube or nasopharyngeal airway (NPA) (Fig. 1). In 41 % (n = 31), a surgical intervention was pursued, at a mean age of 50 days (SD, 55). Until 2006, this consisted of TLA whenever possible. If TLA failed, or there was a (sub)glottic pathology, a tracheotomy was performed. Since 2006, the primary surgical intervention for UAO caused by a supraglottic obstruction is MDO. During the study period, in more than half of the surgically treated cases (58 %, n = 18), MDO was pursued; in 19 % (n = 6) TLA and in 23 % (n = 7), a tracheotomy. Average duration until decannulation after a tracheotomy was 13.4 months (range, 4.1–36.5). More than half of the nonisolated RS infants, compared to only a quarter of the isolated RS, infants needed surgical intervention (53 vs. 25 %, p = .014) (Table 2). Moreover, mean duration of admission was significantly shorter in the isolated group than in the nonisolated group (33 vs. 58 days, p = .018). Two infants with syndromic RS received two interventions: One patient had a tracheotomy prior to MDO; another needed a tracheotomy directly after release of the TLA. Both were successfully decannulated afterwards.Table 2


A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment algorithm.

Paes EC, van Nunen DP, Speleman L, Muradin MS, Smarius B, Kon M, Mink van der Molen AB, van der Molen AB, Niers TL, Veldhoen ES, Breugem CC - Clin Oral Investig (2015)

Use of a nasopharyngeal airway as conservative treatment option in a 1-month-old infant with RS
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC4592702&req=5

Fig1: Use of a nasopharyngeal airway as conservative treatment option in a 1-month-old infant with RS
Mentions: The majority (59 %, n = 44) of the infants admitted to our hospital could be successfully managed conservatively (Table 2). This consisted of side/prone positioning, temporary supplemental oxygen or usage of continuous positive airway pressure (CPAP), a mayotube or nasopharyngeal airway (NPA) (Fig. 1). In 41 % (n = 31), a surgical intervention was pursued, at a mean age of 50 days (SD, 55). Until 2006, this consisted of TLA whenever possible. If TLA failed, or there was a (sub)glottic pathology, a tracheotomy was performed. Since 2006, the primary surgical intervention for UAO caused by a supraglottic obstruction is MDO. During the study period, in more than half of the surgically treated cases (58 %, n = 18), MDO was pursued; in 19 % (n = 6) TLA and in 23 % (n = 7), a tracheotomy. Average duration until decannulation after a tracheotomy was 13.4 months (range, 4.1–36.5). More than half of the nonisolated RS infants, compared to only a quarter of the isolated RS, infants needed surgical intervention (53 vs. 25 %, p = .014) (Table 2). Moreover, mean duration of admission was significantly shorter in the isolated group than in the nonisolated group (33 vs. 58 days, p = .018). Two infants with syndromic RS received two interventions: One patient had a tracheotomy prior to MDO; another needed a tracheotomy directly after release of the TLA. Both were successfully decannulated afterwards.Table 2

Bottom Line: Forty-four infants (59%) were found to have been treated conservatively.RS is a heterogenic group with a wide spectrum of associated anomalies.As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable.

View Article: PubMed Central - PubMed

Affiliation: Department of Plastic, Reconstructive and Hand Surgery, Wilhelmina Children's Hospital, Utrecht, The Netherlands. emmapaes@gmail.com.

ABSTRACT

Objectives: Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution's approach and a review of the current literature.

Material and methods: A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996-2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS.

Results: Forty-four infants (59%) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25%, p = .014). A mandibular distraction was conducted in 24% (n = 18) of cases, a tracheotomy in 9% (n = 7), and a tongue-lip adhesion in 8% (n = 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described.

Conclusions: RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended.

Clinical relevance: We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics.

No MeSH data available.


Related in: MedlinePlus