Breastfeeding Infants With Craniofacial Anomalies

Introduction

Birthing is an overwhelming natural process that is followed by the development of organs. The process of development of organs is called organogenesis. A fully matured human baby is nourished in the mother's womb for nine months. During this time, certain miscommunication of genes or familial traits or otherwise acquired chromosomal variation due to external factors together gives rise to defects in the formation of the head and face, which are known as craniofacial anomalies.

What Is a Craniofacial Anomaly?

These are rare disorders occurring in 1 in every 2000 births. These defects involve the facial structures or cranial structures like the skull, the brain, the eye sockets, the nose, the upper and lower jaw, the mouth, and the ear structure. Craniofacial anomalies could involve any of these structures or all of these structures. Certain craniofacial anomalies affect airway passage and cause breathing difficulties. And physical defects of the orofacial region affect speech and the masticatory process. Anomalies of the face are caused due to gene mutations and environmental and folic acid deficiency. Craniofacial anomalies can involve the brain or occur by dysostosis and synostosis processes.

• Dysostosis: It is a congenital defect seen in the bones and teeth. The organ formation will be completely absent or partially present.

• Synostosis: The fusion of more than one bone is called synostosis. In craniofacial anomalies, the skull bone fuses with adjacent bone.

How Is Breast Feeding Carried Out in Infants With Craniofacial Deformities?

Lactation in patients with craniofacial anomalies is very challenging due to deformed structures. First and foremost, the size of the breasts and the child's weight is evaluated. Three components are considered while breastfeeding, they are:

• The latch at the breast.

• Sustaining a seal at the breast.

• The suction inside the baby’s mouth.

The best method of breastfeeding such infants is by sitting upright. This prevents the milk from seeping inside the nose or lungs. The milk gets accumulated sideways where there is no cut present. The mother should assist the child in feeding milk by giving an episodal sucking of the breasts. The sucking should be done in small force with limited secretion according to the child's capacity for swallowing. Any sign of leaking milk from the mouth should be immediately stopped. In complicated cases, where feeding through breast is not possible, the mother can collect her milk in a closed container and feed the child from that using a spoon or using a special customized feeding device.

What Are the Complications of Cleft Lip and Palate?

Children with cleft lip and palate have the following difficulties:

• Speech difficulty.

• Drooling milk and food outside the mouth and nose.

• Chest infections followed by cough and cold.

• Ear infections.

• Getting frequent illnesses.

• Air sucked through the open passages.

• Milk enters the lungs through false openings of the mouth.

• Frequent burping is present.

• Natal teeth are present in infants and can be removed.

What Are the Different Types of Craniofacial Anomalies Seen?

Studies have found around fifty craniofacial syndromes. The most frequent citation of facial anomalies is the cleft lip and palate. It is estimated that 0.33 per 1000 births are born with this anomaly. Males are most commonly affected by cleft palate. Scientists and doctors make many different classifications for the study of craniofacial anomalies. A few congenital anomalies are listed below for referral. There are certain syndromes associated with craniofacial anomalies, such as:

• Craniosynostosis: It is a disorder seen in the growth plates of the skull. The bones are fused and developed early, blocking the further growth of the brain. Lack of brain growth causes deformity of facial structures. It is treated surgically.

• Apert Syndrome: It is also known as acrocephalosyndactyly. This is caused by bone deformation in the skull, face, hands, and feet. The skull is seen as a tall peaked structure, and the hands and feet are fused together. The sutures of the skull fuse early, causing abnormal cranial and facial features. The cranium tends to grow in a parallel or lateral direction. When the frontal and backbone of the skull grow shorter, it is known as Turribrachycephaly. The facial features observed are midfacial hypoplasia or swollen face, a big nose, and protruding eyes. In the oral cavity, it is seen as crooked teeth, cleft palate, absence of teeth, narrow jaw, and other features. Such patients show breathing difficulty, sleep apnea, and eye problems. In some cases, all the fingers of the hands fuse together with a single digit protruding, also known as a rose-bud formation. The gene responsible for Apert syndrome is FGFR2, seen in chromosome 10.

• Crouzon Syndrome: This is caused by premature fusion of the skeletal base bone. It is followed by blindness, anxiety, and twelve percent of patients show mental retardation. Other clinical features are protruding eyes, crowding of teeth, cleft palate, and deviated nasal septum. One in twenty-five thousand children are born with Crouzan syndrome.

• Muenke Syndrome: This is also caused by premature closure of skull bones.

• Pfeiffer Syndrome: This is caused by premature fusion of bones. The symptoms include a high, prominent forehead, bulgy eyes, an abnormally large distance between the eyes, dental problems, hearing loss, and wide far away thumbs. It is caused by the FGFR1 (fibroblast growth factor receptor) gene mutation seen in chromosome 8 and the FGFR2 gene mutation seen in chromosome 10.

• Saethre-Chotzen Syndrome: It is caused due to premature closure of one or more skull sutures. The symptoms are mental retardation, bulging eyes, and flat faces due to underdeveloped facial features.

• Hemifacial Microsomia: It is also known as hemifacial microsomia and is the second most common condition affecting the head and neck in children. It is caused by underdevelopment of one side of the face, and twenty percent of the cases happen on both sides. It is marked by the absence of soft tissues like the ear, lower jaw, upper jaw, and nervous system. It can be surgically treated by placing a graft.

Some of the anomalies seen due to dysplasia are:

• Mandibular Dysostosis: This is also known as Treacher-Collins syndrome or Franceschitti - Zwahlen -Klein syndrome. It is caused by COFFEE, POLRIC, and POLRID gene mutations seen in the fifth chromosome. It is followed by symptoms like palpebral fissures below the eye line and deficiency of eyelids with other facial deformities.

Facial deformities are also caused due to dysplasia seen in the cleft palate and lips. It is caused due to abnormal fusion of nasal, orbital, and lip structures. It is a congenital condition originating from the top of the lip and can extend anywhere to the eyes, nose, and chin in the open section. Different types of cleft are present such as median nasal cleft, median cleft lip, premaxilla, oblique facial cleft, cleft lip, and palate.

How Are the Dental Procedures Carried Out in Cleft Lip and Palate Infants?

Though many surgical and orthodontic appliances have been made to restore the normal anatomy and physiology of facial structures, the process is challenging.

• Impression Techniques: For any impression procedures, three things have to be considered - first, the patient position, second tray selection, and finally, the impression material to be used.

• Patient Positioning: An accurate patient position helps deliver a precise negative replica of the area of interest. Various positions have been proposed, but the ideal positions are prone, with the face placed down, upright, and even upside down. Some prefer the hospital crib position.

• Tray Selection: First, the shape of the dental arch is taken, and the tray size is selected. Rimming entire tray borders with utility wax is also done to provide additional bulk of the material laterally. A posterior dam is also used to prevent the extra seeping of impression material posteriorly. Prefabricated trays are also available in markets (Coe laboratories, Chicago) Shatkin and Stark have described the use of wax for impression trays in cleft lip or cleft palate patients.

• Impression Material: Materials such as heavy-body silicone impression material, polyvinyl siloxane impression material, low-fusing impression compound, and alginate have been routinely employed.

What Are the Methods of Management Advised for Craniofacial Anomalies?

The following points can be referred to for the management of the condition:

• The child should be fed regularly with thick milk for proper nutrition.

• The baby should be constantly monitored for weight.

• Prosthodontic appliances like obturators should be practiced against open palatal openings to give a proper seal.

• The mouth should be inspected twice daily to inspect for food debris areas.

• The mouth should be constantly cleaned and evaluated for conditions like oral thrush. They generally appear as white patches on the tongue, which do not go away on wiping. Oral thrush is fungal infection, and pediatricians will further prescribe medications for their treatment.

• Areas of deposition can be wiped away using a wet cotton wipe.

• Areas of the lip can be lubricated with products like Acyclovir.

Conclusion

Diagnosis of craniofacial anomaly occurs either during birth or before birth. The ultrasonography captures the facial view of the baby based on its appearance. The revelation of such a diagnosis often takes away parenthood's joy. This fear and worry for tomorrow, the newborn's survival, can be assured under the best pediatric and prosthetic care. Prenatal counseling of the couples is done to prepare for the delivery and ensure a brighter future filled with hope.