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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

Algorithm of the institutional approach to infants with Robin sequence
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Fig2: Algorithm of the institutional approach to infants with Robin sequence

Mentions: Our treatment algorithm is presented in Fig. 2. Infants diagnosed with RS are initially treated in prone or side position when their condition allows it. Prior to any decision making, the patient is observed for at least 24 h. Monitoring of vital parameters, measurements of oxygen saturation by continuous pulse oximetry, capillary blood gas analysis, and more recently transcutaneous carbon dioxide measurements (Tosca®) are performed [50–52]. Observation of clinical signs of respiratory distress during sleep and awake, as well as feeding ability, are documented by experienced nursing and medical staff. We consider oxygen saturations of <90 % for >5 % of the monitored time and/or any single desaturation <80 % as a sign of UAO [39, 41, 42]. Blood gas analysis revealing respiratory acidosis (pCO2 > 50 mmHg, HCO3 > 30 mmHg) or transcutaneous CO2 > 50 mmHg during >25 % of the total sleep time is indicative of hypoventilation [53]. Results are discussed in a multidisciplinary setting consisting of at least a pediatrician, plastic surgeon, otolaryngologist, and a pediatric intensive care specialist after 24–48 h of monitoring. A clinical geneticist is always consulted. Based on the observations and measurements, patients are divided into mild UAO or moderate/severe UAO. These characteristics are described in Fig. 2.Fig. 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)

Algorithm of the institutional approach to infants with Robin sequence
© Copyright Policy - OpenAccess
Related In: Results  -  Collection

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

Fig2: Algorithm of the institutional approach to infants with Robin sequence
Mentions: Our treatment algorithm is presented in Fig. 2. Infants diagnosed with RS are initially treated in prone or side position when their condition allows it. Prior to any decision making, the patient is observed for at least 24 h. Monitoring of vital parameters, measurements of oxygen saturation by continuous pulse oximetry, capillary blood gas analysis, and more recently transcutaneous carbon dioxide measurements (Tosca®) are performed [50–52]. Observation of clinical signs of respiratory distress during sleep and awake, as well as feeding ability, are documented by experienced nursing and medical staff. We consider oxygen saturations of <90 % for >5 % of the monitored time and/or any single desaturation <80 % as a sign of UAO [39, 41, 42]. Blood gas analysis revealing respiratory acidosis (pCO2 > 50 mmHg, HCO3 > 30 mmHg) or transcutaneous CO2 > 50 mmHg during >25 % of the total sleep time is indicative of hypoventilation [53]. Results are discussed in a multidisciplinary setting consisting of at least a pediatrician, plastic surgeon, otolaryngologist, and a pediatric intensive care specialist after 24–48 h of monitoring. A clinical geneticist is always consulted. Based on the observations and measurements, patients are divided into mild UAO or moderate/severe UAO. These characteristics are described in Fig. 2.Fig. 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