Pierre Robin syndrome (or Pierre Robin sequence) (PRS) is a rare congenital disease that occurs with a prevalence of 1 in 10,000 births.

Its characteristics are a set of three anomalies of the craniofacial area: glossoptosis, retrognathia, and a median velopalatine fissure. It is called a sequence because the morphological alterations produced are a consequence of each other. This sequence can occur alone or together with other morphological alterations, forming a more complex syndrome. These anomalies can cause dysfunctions in the feeding and breathing of the newborn.

This is a process that improves with time as the individual grows and the jaw develops. If the abnormality in the palate is treated correctly, the condition has a good prognosis in most cases.

However, these are measures that require time to pass, and when a mother expresses her desire to breastfeed a newborn with PRS, many questions arise that unfortunately do not have an easy answer. Due to the low incidence of this disease in the population, there is little evidence on the subject. And even more so when it comes to breastfeeding a baby with this syndrome. Therefore, we will report here the information provided by our daily practice. The following is a list of correct actions, in our opinion, in relation to breastfeeding in the presence of PRS.

First of all, it is important to emphasize that it is essential to consider each case individually due to the high variability of symptom presentation together with the characteristics of each individual and each mother-infant dyad. Therefore, the possibility of breastfeeding should never be affirmed or denied, not even when there is a disease that directly affects the sucking ability of an infant.

In this context, the initiation of a breastfeeding support process with a mother-infant dyad should focus on the fact that it is a process that can involve many complexities, but, at the same time, there are elements and resources that can be used to try to achieve exclusive breastfeeding. Depending on the case, these can be discussed in detail so that they can be applied if necessary.

Once the mother has been informed and is familiar with the specific disorder presented by the infant, it is very informative to assess whether the infant is able to maintain the latch onto the breast, if he/she performs a nutritive sucking pattern, and if he/she can make an intraoral vacuum. For this, the mother should be proposed to offer the breast in conditions that may favor a safe feed:

• Carry out the feed at the breast accompanied by professionals.
• Use upright positioning to facilitate swallowing.
• Consider the use of nipple shields.
• Supplementing the baby with a syringe while breastfeeding to facilitate the learning process

In the event that this feed is unsuccessful, it is time to consider the option of placing a palatal obturator until the time of the intervention, which is usually around one year of life. The function we are looking for is that the palatal obturator manages to limit as much as possible this nasopharyngeal communication in order to help to make the vacuum in the oral cavity and also to prevent more effectively the possibility of the appearance of broncho-aspiration of milk by the infant.

Placement of a palatal obturator within hours of birth is encouraged in some hospitals but is still uncommon in many other hospitals. Therefore, we need to keep in mind that while proceeding with measurements and other logistical issues regarding the obturator, feeding options for the infant need to be proposed if the newborn is unable to maintain the vacuum at the breast and suckle effectively. These methods for initiating feeding with breastmilk are:

• Finger-syringe: a technique that can also help to close the cleft with the finger to obtain a better oral seal.
• Use of bottles with special nipple teats, which make it easier for the mother to squeeze the bottle when the baby tries to suck.
• Placement of a nasogastric tube: this is used in circumstances where the infant’s feeding and/or safety cannot be assured. It can sometimes be used in conjunction with direct breastfeeding or other supplementing methods.

An appropriate pumping pattern should be discussed with the mother, she should be advised on the use of the breast pump and be provided with a correct and detailed explanation of how to express and give breast milk in a bottle.

Finally, and despite the fact that by pumping breast milk the baby can be fed, if the mother wishes to do so, it is advisable that you try to encourage the mother to directly breastfeed from the breast as well. The stimulation from the mechanisms involved in the suction, swallowing, and breathing process improves the development of the orofacial structures and improves the infant’s recovery rates after cleft palate surgery.

Resources

https://www.orpha.net/en/disease/detail/436003?name=pierre%20robin&mode=name