Abstract
Patients with craniofacial microsomia are at higher risk of developing obstructive sleep apnoea (OSA), as described in the previous article entitled “Obstructive sleep apnoea in craniofacial microsomia: a systematic review”. These patients are also more likely to develop feeding difficulties. The present systematic review provides an overview of the literature on the prevalence, treatment, and follow-up of feeding difficulties in children with craniofacial microsomia (CFM). A search was performed in PubMed, Embase, Cochrane Library, and Web of Science for articles on CFM and feeding difficulties. The following data were extracted from the articles: number of patients, patient characteristics, presence of feeding difficulties, and the treatment and outcomes of feeding difficulties. Eight articles on CFM and feeding difficulties were included, two of which reported the prevalence of feeding difficulties (range 42–83%). Treatment mostly consisted of tube feeding. No information regarding follow-up was found in these articles. According to the literature, feeding difficulties are related to CFM. However, as there have been no prospective studies and few studies have presented objective measurements, no definitive conclusions can be drawn. Prospective studies are needed to determine the prevalence of feeding difficulties in patients with CFM.
Craniofacial microsomia (CFM) is the result of a disturbance in the embryological development of the first and second branchial arches and is characterized by asymmetric underdevelopment of the facial structures, including the mandible, maxilla, ears, soft tissues, and facial nerves. CFM is most often regarded as a unilateral malformation, however the facial structures are involved bilaterally in 10% of cases and several recent publications have suggested that the contralateral side is abnormal in most cases as well, although not truly hypoplastic. The reported incidence rate ranges from 1 in 3500 to 1 in 20,000, which makes CFM the second most common facial birth defect after cleft lip and palate. CFM in combination with epibulbar dermoid and extra-craniofacial anomalies, such as heart, renal, and vertebral anomalies, is known as Goldenhar syndrome.
The most typical deformity of CFM is mandibular hypoplasia, which occurs in 89–100% of these patients. The severity of the mandibular hypoplasia can be classified into four types based on the Pruzansky classification, modified by Kaban. Type I is a small mandible with normal morphology. Type IIa is a mandibular ramus abnormal in both size and shape. Type IIb is a mandibular ramus and temporomandibular joint (TMJ) abnormal in size, morphology, and location. Type III deformity consists of an absent ramus, condyle, and TMJ.
Huisinga-Fischer et al. evaluated the relationship between underdevelopment of the masticatory muscles and hypoplasia of the craniofacial skeleton in CFM. Craniofacial bony abnormalities were found to be associated with underdevelopment of the masticatory muscles, which might lead to problems with mastication and therefore feeding difficulties.
Other studies have suggested that sucking efficiency is limited in patients with CFM as a result of mandibular hypoplasia, which restricts the excursion of the mandible. Facial nerve weakness might lead to limited active cheek and lip movements, and anomalies in the structure and function of the tongue might play a role in feeding difficulties in patients with CFM as well.
Additionally, anomalies at the level of the oropharynx and larynx may play a role in feeding difficulties in patients with CFM, and more specifically in patients with Goldenhar syndrome, as movement of the lateral pharyngeal wall of the affected side is diminished compared to the unaffected side during swallowing. This does not appear to be related to the severity of the facial anomalies.
Not only do oropharyngeal and laryngeal deformities, and more specifically Goldenhar syndrome, increase the risk of feeding difficulties in patients with CFM, but extra-craniofacial anomalies, such as gastrointestinal malformations and congenital heart disease, also have an influence on this risk.
As previously mentioned, mandibular hypoplasia is the most common deformity in patients with CFM. As well leading to malocclusion and therefore to feeding difficulties, mandibular hypoplasia can also lead to obstructive sleep apnoea (OSA). With a prevalence of 7–67%, as described in the previous review “Obstructive sleep apnoea in craniofacial microsomia: a systematic review”, patients with CFM are more likely to develop OSA than patients in the healthy population. As feeding difficulties are closely correlated to OSA, patients with CFM could, for this reason, be more at risk of feeding difficulties as well.
Thus, feeding difficulties in patients with CFM might be the result of underdevelopment of the mandible, facial nerve, and/or masticatory muscles, but could also be the result of lateral pharyngeal wall anomalies or OSA. Although several studies have stated that feeding difficulties are more likely to occur in patients with CFM, not much is known about the prevalence, treatment, and follow-up of feeding difficulties in patients with CFM.
The aim of this review is to give an overview of the literature regarding CFM and the prevalence and treatment of feeding difficulties based on the following key questions: (1) What is the prevalence of feeding difficulties in patients with CFM and what types of feeding difficulties are reported? (2) How are feeding difficulties treated in patients with CFM?
Methods
Search strategy
A search of public domain databases was performed to identify articles focusing on CFM and feeding difficulties. The search was conducted in the following databases: PubMed, Embase, Cochrane Library, and Web of Science (all searched up to 12 September 2014). In addition, we performed a manual search of secondary sources including references of the articles initially identified. The goal was to identify all studies addressing CFM in relation to feeding difficulties.
The following search terms were used: (((facial[tiab OR face[tiab] OR hemifacial[tiab] OR orbitocranial[tiab] OR facies[tiab] OR cranial[tiab] OR mandibulofacial[tiab] OR otomandibular[tiab] OR craniofacial[tiab] OR faciocranial[tiab] OR hemimandibular[tiab]) AND (microsom*[tiab] OR asymmetr*[tiab] OR dysosto*[tiab] OR dysplasia[tiab OR anomal*[tiab] OR deformit*[tiab] OR hypoplasia[tiab] OR syndrom*[tiab] OR malformation*[tiab])) OR (treacher collins[tiab] OR goldenhar[tiab]) OR (oculoauriculovertebral*[tiab] OR facioauriculovertebral*[tiab] OR (auriculo vertebral*[tiab]))) AND (dysphagia[tiab] OR ((feeding[tiab] OR swallow*[tiab] OR deglutition[tiab] OR eat*[tiab] OR chew*[tiab] OR masticat*[tiab] OR bite[tiab] OR biting[tiab]) AND (problem*[tiab] OR difficult*[tiab] OR abnormal[tiab] OR disabilit*[tiab] OR disorder*[tiab] OR impair*[tiab] OR normal*[tiab] OR disturb*[tiab] OR unable[tiab] OR unabilit*[tiab])) OR nutrition*[tiab] OR malnutrition*[tiab] OR failure to thrive[tiab]) AND publisher[sb].
Data extraction and analysis
Two investigators (C.J.J.M.C. and B.I.P.) selected the studies independently. All articles on the prevalence and treatment of patients with CFM and feeding difficulties were included. Expert opinions were excluded. The full texts of articles that met the inclusion criteria and of articles for which the abstract was lacking information were obtained.
Articles were graded on quality of evidence using the Oxford Centre for Evidence-Based medicine (CEBM) criteria. Data on the number of patients, patient characteristics such as gender, age, and severity of the CFM, and the presence of feeding difficulties were tabulated when available.
Results
The initial search retrieved 1604 articles. After removing duplicate articles and including additional articles identified from secondary sources, 1057 abstracts were assessed. After examination of the title and abstract, 39 articles were retrieved for further examination. Eight articles were included in the analysis ( Fig. 1 ).
What is the prevalence of feeding difficulties in patients with CFM and what types of feeding difficulties are reported?
Feeding difficulties are strongly related to OSA and could therefore be present more often in patients with CFM. Furthermore, the anatomical deformities in CFM could lead to feeding difficulties as well. The prevalence of feeding difficulties in the studies included varied from 42% to 83%. However, data on the prevalence of feeding difficulties in CFM were scarce and no firm conclusions can be drawn based on these studies ( Tables 1 and 2 ).
| Reference | CEBM level of evidence | Methodology | Aim of the study |
|---|---|---|---|
| Cohen et al. | III | Cross-sectional study | To examine the neurodevelopmental profile of children with Goldenhar syndrome and to determine if physical manifestations are indicative of poor developmental outcomes |
| Strömland et al. | III | Retrospective study | To survey the systemic and functional defects in patients with Goldenhar syndrome |
| Shokeir | III | Retrospective study | To delineate the natural history of the disorder |
| Reference | No. of patients with CFM | Classification of CFM | Extra-craniofacial malformations | No. of patients with FD and cleft lip and/or palate | Age range | Gender, M/F | No. of patients with FD | Type of FD |
|---|---|---|---|---|---|---|---|---|
| Cohen et al. | 24 | NR | Congenital heart defect, scoliosis, genitourinary abnormality, limb defect | NR | Birth to 57 m | 16/8 | 10 | Feeding disturbances, swallowing dysfunction, failure to thrive |
| Strömland et al. | 18 | NR | Cardiovascular, respiratory system, gastrointestinal | 5 | 8 m to 17 y | 11/7 | 12 | Dysphagia |
| Shokeir | 24 | NR | Congenital heart defect, hiatus hernia | 6 | Birth to 58 y | 11/13 | 20 | Incoordination of deglutition, choking spells |
Cross-sectional study on the prevalence of feeding difficulties in CFM
Ten of the 24 patients (42%) studied by Cohen et al. were diagnosed with feeding difficulties, mainly caused by a decreased tone in the orofacial musculature. Details of the classification of the CFM were not mentioned.
Retrospective studies on the prevalence of feeding difficulties in CFM
Of the 18 patients included in the study of Strömland et al., 12 reported feeding problems in infancy. Half of the group had complaints such as difficulty chewing and swallowing, and six patients complained of drooling. Oral impairment and orofacial deformities could explain the dysphagia in most cases, but in some cases feeding difficulties were mainly the result of other symptoms affecting the general condition of the patient, e.g. heart defects, breathing difficulties, and gastrointestinal problems.
According to Shokeir, one of the four major problems in the neonatal period of patients with Goldenhar syndrome is feeding difficulties such as dysphagia and choking due to anatomical deformities at the level of the mandible, larynx, and/or oesophagus, necessitating gavaging, nasogastric feeding, or surgical treatment. Feeding difficulties occurred in 20 of the 24 patients included. For the other four patients, nothing was mentioned regarding feeding difficulties.
How are feeding difficulties treated in patients with CFM?
The treatment of feeding difficulties was described in a few retrospective studies and case reports and mainly consisted of tube feeding. Patient numbers were low and no objective outcome measurements were reported, except in the case series of Clawson et al. Firm conclusions cannot be drawn based on these studies and case reports ( Tables 3 and 4 ). Unfortunately, follow-up of the patients with feeding difficulties was not described in these studies.
| Reference | CEBM level of evidence | Methodology | Aim of the study |
|---|---|---|---|
| Strömland et al. | III | Retrospective study | To survey the systemic and functional defects in patients with Goldenhar syndrome |
| Shokeir | III | Retrospective study | To delineate the natural history of the disorder |
| Hoch and Hochban | IV | Retrospective CR | Presentation of a case with CFM |
| Clawson et al. | IV | Retrospective CS | To discuss the effectiveness of a behavioural-based feeding programme to improve feeding abilities in Goldenhar syndrome |
| Yokochi et al. | IV | Retrospective CS | Presentation of cases with CFM including videofluorographic findings |
| Zanardi et al. | IV | Retrospective CR | To describe the treatment of a patient with a severe facial deformity due to CFM |
| Mellor et al. | IV | Retrospective CS | Presentation of cases |
| Reference | No. of patients with CFM | No. of patients with CFM and FD | Age range, or mean age | Feeding difficulties | Cleft palate in patients with FD? | Treatment of the FD |
|---|---|---|---|---|---|---|
| Strömland et al. | 18 | 12 | 8 m to 17 y | Chewing, swallowing, drooling, dysphagia | Yes, 5 out of 12 | Tube feeding, gastrostomy, nasogastric tube |
| Shokeir | 24 | 20 | Birth to 58 y | Incoordination of deglutition, choking spells | Yes, 5 out of 20 | Tube feeding, gastrostomy and reconstruction of cleft |
| Hoch and Hochban | 1 | 1 | Birth | Sucking, failure to thrive | Yes | Tube feeding, reconstruction of cleft, treatment of OSA |
| Clawson et al. | 3 | 3 | 15 m to 42 m | Gastroesophageal reflux, dysphagia | Yes, 2 out of 3 | Tube feeding |
| Yokochi et al. | 3 | 3 | 2 y to 4 y | Sucking, drooling | Yes, 3 out of 3 | Tube feeding |
| Zanardi et al. | 1 | 1 | 12 y | Chewing | No | Not further specified |
| Mellor et al. | 3 | 2 | Birth | Not further specified | Yes, 1 out of 2 | Tube feeding |
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