Unravelling Robin sequence

Lieshout, Manouk J.S. van; Joosten, Koen; Hoeve, Hans; Mathijssen, Irene M.J.; Koudstaal, Maarten J.; Wolvius, Eppo B.

doi:10.1002/lary.24437

Cited by 35 publications

(8 citation statements)

References 33 publications

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“…In 75–100% of the cases with PRS, a cleft palate is a common finding (Butow et al, 2009 ; van Lieshout et al, 2014 ; Cote et al, 2015 ). PRS patients have a variable mandibular morphology and position depending on the occurrence and type of the associated syndromes (Rogers et al, 2009 ).…”

Section: Resultsmentioning

confidence: 99%

Update on 13 Syndromes Affecting Craniofacial and Dental Structures

Bartzela¹,

Carels²,

Maltha³

2017

Front. Physiol.

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Care of individuals with syndromes affecting craniofacial and dental structures are mostly treated by an interdisciplinary team from early childhood on. In addition to medical and dental specialists that have a vivid interest in these syndromes and for whom these syndromes are of evident interest, experts of scientific background—like molecular and developmental geneticists, but also computational biologists and bioinformaticians—, become more frequently involved in the refined diagnostic and etiological processes of these patients. Early diagnosis is often crucial for the effective treatment of functional and developmental aspects. However, not all syndromes can be clinically identified early, especially in cases of absence of known family history. Moreover, the treatment of these patients is often complicated because of insufficient medical knowledge, and because of the dental and craniofacial developmental variations. The role of the team is crucial for the prevention, proper function, and craniofacial development which is often combined with orthognathic surgery. Although the existing literature does not provide considerable insight into this topic, this descriptive review aims to provide tools for the interdisciplinary team by giving an update on the genetics and general features, and the oral and craniofacial manifestations for early diagnosis. Clinical phenotyping together with genetic data and pathway information will ultimately pave the way for preventive strategies and therapeutic options in the future. This will improve the prognosis for better functional and aesthetic outcome for these patients and lead to a better quality of life, not only for the patients themselves but also for their families. The aim of this review is to promote interdisciplinary interaction and mutual understanding among all specialists involved in the diagnosis and therapeutic guidance of patients with these syndromal conditions in order to provide optimal personalized care in an integrated approach.

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Section: Resultsmentioning

confidence: 99%

Update on 13 Syndromes Affecting Craniofacial and Dental Structures

Bartzela¹,

Carels²,

Maltha³

2017

Front. Physiol.

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“… 33 , 34 A severe obstructive index in neonates has been interpreted as >6, 30 >10, 14 and >24. 12 Our study is not designed to help stratify obstructive severity; however, we observed a significant threshold between the conservative and surgical groups at an OI >20. Combined with other examination findings, this threshold may help guide surgical decision making; surgery should be considered at an OI >20.…”

Section: Discussionmentioning

confidence: 65%

“…In most cases, affected neonates may be treated without surgery, using prone positioning or a palatal obturator to clear the obstructed airway. 11 , 12 Others report good results bypassing the TBAO using long-term nasopharyngeal tubes or continuous positive airway pressure (CPAP) mask therapy. 13 , 14 Surgical interventions are generally reserved for those determined to have failed conservative measures, either with severe, persistent TBAO or inadequate oral feeding.…”

Section: Introductionmentioning

confidence: 99%

Management of Airway Obstruction in Infants With Pierre Robin Sequence

Runyan

Uribe-Rivera

Tork

et al. 2018

Plastic and Reconstructive Surgery - Global Open

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“…In 2017, a systematic review reported feeding difficulties in 80% (range 47–100%) of RS infants, and long-term nasogastric tube feeding in 55% (range 11–100%) [ 11 ]. Mean duration of tube feeding ranged from a few weeks [ 9 , 11 , 12 ] to several months [ 7 , 35 , 36 ].…”

Section: Discussionmentioning

confidence: 99%

Retrospective study on growth in infants with isolated Robin sequence treated with the Tuebingen Palate Plate

Wiechers

Iffländer²,

Gerdes³

et al. 2021

Orphanet J Rare Dis

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Background Children with Robin sequence (RS) are at risk of growth failure, mainly due to their increased work of breathing and feeding difficulties. Various conservative and surgical treatment approaches exist, but their impact on weight gain has not yet been adequately addressed. A functional treatment concept, used in our center for > 20 years, includes a pre-epiglottic baton plate (Tuebingen palatal plate) and intensive feeding training. Objective To investigate the effect of the Tuebingen treatment protocol on growth and weight trajectories during infancy. Methods This retrospective study analyzed longitudinal data from infants with isolated RS admitted to Tuebingen University Children’s Hospital, Germany between 1998 and 2019. Through our electronic patient database, we evaluated anthropometric parameters until reaching 1-year follow-up. Results are shown as median (IQR). Results In 307 infants analyzed, median Z-score for weight decreased from − 0.28 at birth to − 1.12 upon admission to our center at a median age of 22 days. Z-score then remained largely unchanged until discharge (Z-score difference, − 0.08), while the proportion of infants receiving tube feedings decreased from 55.1 to 13.7%. Z-score subsequently increased from − 1.17 at discharge to − 0.44 at the 1-year follow-up (p < 0.001). Conclusion Based on a comparatively large cohort, this functional treatment was associated with better weight gain and improved feeding. As RS infants often show postnatal growth failure, weight monitoring may be a valuable parameter for monitoring treatment effectiveness. Clinical Trial Registration Not necessary due to the retrospective design.

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Unravelling Robin sequence

Cited by 35 publications

References 33 publications

Update on 13 Syndromes Affecting Craniofacial and Dental Structures

Update on 13 Syndromes Affecting Craniofacial and Dental Structures

Management of Airway Obstruction in Infants With Pierre Robin Sequence

Retrospective study on growth in infants with isolated Robin sequence treated with the Tuebingen Palate Plate

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