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Cerebro-costo-mandibular syndrome

Lim CT, Koh MT

Australas Radiol, 1992 May;36(2):158-9.
PMID: 1520180

Cerebro-costo-mandibular syndrome (CCM) is a very rare entity with oro-facial features closely resembling Pierre-Robin anomaly (1,2). We describe a patient with typical features: severe micrognathia, glossoptosis, central cleft soft palate and multiple posterior rib-gap defects seen on chest radiograph. Respiratory distress which was evident soon after birth was relieved by an oro-pharyngeal airway. He suffered from a cardiorespiratory arrest and succumbed, presumably secondary to aspiration of milk feeds, before a tracheostomy could be performed.

Matched MeSH terms: Pierre Robin Syndrome/epidemiology*; Pierre Robin Syndrome/radiography
Feeding and reflux in children after mandibular distraction osteogenesis for micrognathia: A systematic review

Breik O, Umapathysivam K, Tivey D, Anderson P

Int J Pediatr Otorhinolaryngol, 2016 Jun;85:128-35.
PMID: 27240511 DOI: 10.1016/j.ijporl.2016.03.033

Mandibular distraction osteogenesis (MDO) is becoming increasingly more commonly used as in neonates and infants with upper airway obstruction secondary to micrognathia. A significant number of these children are dependent on nasoenteric feeding or gastrostomies after birth for adequate nutrition and often suffer from gastro-esophageal reflux (GERD).
OBJECTIVE: This analysis is a subset of a larger systematic review. The objective of this study is to determine the effects of MDO on feeding and GERD.
DATA SOURCES: The databases searched included PubMed, Embase, Scopus, Web of Knowledge and grey literature sources.
STUDY SELECTION: The inclusion criterion included studies in children with clinical evidence of micrognathia/Pierre Robin Sequence (PRS) who have failed conservative treatments, including both syndromic (sMicro) and non-syndromic (iPRS) patients. 21 studies relevant to feeding and 4 studies relevant to GERD outcomes were included. All studies included were case series and case reports.
RESULTS: MDO leads to a significant improvement in feeding, with 82% of children feeding exclusively orally after surgery. The overall percentage of children with iPRS who were feeding orally was 93.7% compared with only 72.9% in the sMicro group (p<0.004). A growth decline within the first six weeks after surgery was observed in multiple studies. Overall, out of 70 patients with pre-operative GERD, only four had evidence of GERD after surgery.
CONCLUSIONS: Considering the limitations of this systematic review, this study found that successful relief of airway obstruction by MDO leads to improvement of feeding and improvement in symptoms of GERD in children with upper airway obstruction secondary to micrognathia. Clinicians need to be aware of the risk of growth decline in the initial post-operative period.

Matched MeSH terms: Pierre Robin Syndrome/complications; Pierre Robin Syndrome/surgery*
Projection the long-term ungauged rainfall using integrated Statistical Downscaling Model and Geographic Information System (SDSM-GIS) model

Tukimat NNA, Ahmad Syukri NA, Malek MA

Heliyon, 2019 Sep;5(9):e02456.
PMID: 31687558 DOI: 10.1016/j.heliyon.2019.e02456

An accuracy in the hydrological modelling will be affected when having limited data sources especially at ungauged areas. Due to this matter, it will not receiving any significant attention especially on the potential hydrologic extremes. Thus, the objective was to analyse the accuracy of the long-term projected rainfall at ungauged rainfall station using integrated Statistical Downscaling Model and Geographic Information System (SDSM-GIS) model. The SDSM was used as a climate agent to predict the changes of the climate trend in Δ2030s by gauged and ungauged stations. There were five predictors set have been selected to form the local climate at the region which provided by NCEP (validated) and CanESM2-RCP4.5 (projected). According to the statistical analyses, the SDSM was controlled to produce reliable validated results with lesser %MAE (<23%) and higher R. The projected rainfall was suspected to decrease 14% in Δ2030s. All the RCPs agreed the long term rainfall pattern was consistent to the historical with lower annual rainfall intensity. The RCP8.5 shows the least rainfall changes. These findings then used to compare the accuracy of monthly rainfall at control station (Stn 2). The GIS-Kriging method being as an interpolation agent was successfully to produce similar rainfall trend with the control station. The accuracy was estimated to reach 84%. Comparing between ungauged and gauged stations, the small %MAE in the projected monthly results between gauged and ungauged stations as a proved the integrated SDSM-GIS model can producing a reliable long-term rainfall generation at ungauged station.

Matched MeSH terms: Pierre Robin Syndrome
Femoral-facial syndrome

Samuel, K.F., Rasat, R.

Malaysian Journal of Paediatrics and Child Health, 1998;10(2):59-61.
MyJurnal

A Malay baby boy with the femoral-facial syndrome is presented. The phenotype included bilateral femoral hypoplasia, short nose with a broad nasal tip, long philtrum with a thin upper lip, micrognathia, bilateral cryptorchidism and hypoplastic penis.

Matched MeSH terms: Pierre Robin Syndrome
Fulltext Mandibular advancement by distraction Osteogenesis for obstructive sleep apnea in patient with Pierre Robin Sequence: a case report

Mohamed Hisham Mohamed Jali @ Yunos, Shaifulizan Abdul Rahman, Ramizu Shaari

International Medical Journal Malaysia, 2017;16(21):28-28.
MyJurnal

Pierre Robin Sequence (PRS) is a condition consists of set of anomalies, which are
cleft palate, micrognathia and glossoptopsis. Management of patients with PRS addresses two main
problems, namely airway obstruction and feeding difficulties. Airway obstruction may lead to
obstructive sleep apnea (OSA). Treatment modalities for OSA are based on the causes. There are
surgical and non-surgical methods. Non-surgical methods such as diet, medication, oral appliances
and continuous positive airway pressure (CPAP) can only be employed in moderate cases. Surgical
method such as maxillo-mandibular advancement or expansion can be achieved by orthognathic
surgery or distraction osteogenesis. (Copied from article).

Matched MeSH terms: Pierre Robin Syndrome
Mandibular distraction osteogenesis for the management of upper airway obstruction in children with micrognathia: a systematic review

Breik O, Tivey D, Umapathysivam K, Anderson P

Int J Oral Maxillofac Surg, 2016 Jun;45(6):769-82.
PMID: 26867668 DOI: 10.1016/j.ijom.2016.01.009

Mandibular distraction osteogenesis (MDO) is increasingly used for neonates and infants with upper airway obstruction secondary to micrognathia. This systematic review was conducted to determine the effectiveness of MDO in the treatment of airway obstruction. The databases searched included PubMed, Embase, Scopus, and grey literature sources. The inclusion criteria were applied to identify studies in children with clinical evidence of micrognathia/Pierre Robin sequence (PRS) who had failed conservative treatments, including both syndromic and non-syndromic patients. Overall 66 studies were included in this review. Primary MDO for the relief of upper airway obstruction was found to be successful at preventing tracheostomy in 95% of cases. Syndromic patients were found to have a four times greater odds of failure compared to those with isolated PRS. The most common causes of failure were previously undiagnosed lower airway obstruction, central apnoea, undiagnosed neurological abnormalities, and the presence of additional cardiovascular co-morbidities. MDO was less effective (81% success rate) at facilitating decannulation of tracheostomy-dependent children (P<0.0001). Failure in these patients was most commonly due to severe preoperative gastro-oesophageal reflux disease, swallowing dysfunction, and tracheostomy-related complications. The failure rate was higher when MDO was performed at an age of ≥24 months. More studies are needed to evaluate the long-term implications of MDO on facial development and long-term complications.

Matched MeSH terms: Pierre Robin Syndrome