Oral Feeding Difficulties Research Articles

Caregiver experiences of oral feeding in infants on long-term high flow nasal cannula support

Purpose Navigating the feeding journey of medically complex infants on long-term high flow nasal cannula support can be challenging for caregivers, especially given inconsistencies in clinical practice for oral feeding on high flow nasal cannula support. The aim of this study was to explore the perspectives of caregivers who have experienced this journey, to inform best practice care. Method This study used a generic qualitative inquiry approach. Semi-structured interviews were conducted with seven caregivers of children who had been on long-term high flow nasal cannula support as infants during 2021. Reflexive thematic analysis was used to generate meaning-united stories. Result All caregivers reported oral feeding difficulties that persisted beyond hospitalisation, resulting in demanding mental loads and care arrangements. Caregivers described how these difficulties were often exacerbated by complex medical needs and the strategies they experimented with to support oral feeding. Caregiver’s experiences in the healthcare system were characterised by inconsistencies in care pathways and variable access to specialist feeding services. The importance of a collaborative relationship with healthcare professionals and a conducive hospital environment were also highlighted. Conclusion The oral feeding journey for families with infants on long-term high flow nasal cannula support was characterised by multifaceted challenges and implications. Further research is required to establish and provide consistent, family-centred, and evidence-based care for this population.

Turkish adaptation of the Neonatal Eating Assessment Tool-Bottle-Feeding in preterm infants discharged to home.

Preterm infants often continue to have feeding difficulties after hospital discharge. Parental use of assessment tools and collaboration with health professionals are important for the early diagnosis of postdischarge feeding difficulties. This methodological study examined the validity and reliability of the Turkish version of the Neonatal Eating Assessment Tool (NeoEAT)-Bottle-feeding in postdischarge preterm infants in Türkiye. A Turkish version of the NeoEAT-Bottle-feeding was developed and applied to 321 mothers of preterm infants younger than 7 months of corrected age between August 2021 and December 2022. Cronbach's alpha, exploratory factor, confirmatory factor, item-total correlation, test-retest, and known-groups validity analyses were performed. The Turkish NeoEAT-Bottle-feeding has 60 items in five factors explaining 55.785% of the total variance. Exploratory factor analysis indicated that the item factor loading ranged from 0.320 to 0.792. The known-group validity analysis confirmed that preterm infants with diagnosed feeding problems had higher total and subscale scores than those without (p = 0.001). The Cronbach's alpha (α) of the entire scale was 0.96. The item-total correlation coefficients were between 0.31 and 0.77 (p = 0.001). There was excellent agreement between test values and retest values obtained after a 2-week interval (intraclass correlation coefficient: 0.930-1.000). The Turkish NeoEAT-Bottle-feeding was shown to be a reliable and valid parent-reported assessment tool for oral feeding skills and difficulties after neonatal intensive care unit discharge in bottle-fed preterm infants younger than 7 months of corrected age. Healthcare professionals can use this assessment tool during the initial evaluation of risk factors contributing to problematic feeding and to determine the effectiveness of planned interventions in preterm infants.

Weight Gain of Infants with Robin Sequence Treated Nonsurgically Using the Stanford Orthodontic Airway Plate (SOAP): 1-Year Follow-Up.

To identify weight gain trends of infants with Robin sequence (RS) treated by the Stanford Orthodontic Airway Plate treatment (SOAP). Retrospective longitudinal cohort study. Single tertiary referral hospital. Eleven infants with RS treated with SOAP. Nonsurgical SOAP. Body weight, Weight-for-age (WFA) Z-scores, and WFA percentiles at birth (T0), SOAP delivery (T1), SOAP graduation (T2), and 12-months old (T3). Between T0 and T1, the weight increased but the WFA percentile decreased from 36.5% to 15.1%, and the Z-score worsened from -0.43 to -1.44. From T1 to T2, the percentile improved to 22.55% and the Z-score to -0.94. From T2 to T3, the percentile and the Z-scores further improved to 36.59% and -0.48, respectively. SOAP provided infants experiencing severe respiratory distress and oral feeding difficulty with an opportunity to gain weight commensurate with the WHO healthy norms without surgical intervention.

Prevalence and Risk Factors for Tube-Feeding at Discharge in Infants following Early Congenital Heart Disease Surgery: A Single-Center Cohort Study.

Oral feeding difficulty is common in infants after congenital heart disease (CHD) surgical repair and is associated with prolonged hospital stay and increased risk for tube-feeding at discharge (TF). The current understanding of the enteropathogenesis of oral feeding difficulty in infants requiring CHD surgery is limited. To determine the prevalence and risk factors for TF following CHD surgery in early infancy. This was a 6-year single-center retrospective cohort study (2016-2021) of infants under 6 months who had CHD surgery. Infants required TF were compared with infants who reached independent oral feeding (IOF). Of the final sample of 128 infants, 24 (18.8%) infants required TF at discharge. The risk factors for TF in univariate analysis include low birth weight, low 5-minute Apgar score, admitted at birth, risk adjustment in congenital heart surgery categories IV to VI, presence of genetic diagnosis, use of Prostin, higher pre- and postsurgery respiratory support, lower weight at surgery, lower presurgery oral feeding, higher presurgery milk calory, delayed postsurgery enteral and oral feeding, higher pre- and postsurgery gastroesophageal reflux disease (GERD), need for swallow study, abnormal brain magnetic resonance imaging (p < 0.05). In the multivariate analysis, only admitted at birth, higher presurgery milk calories, and GERD were significant risk factors for TF. TF had significantly longer hospital stay (72 vs. 17 days) and lower weight gain at discharge (z-score: -3.59 vs. -1.94) compared with IOF (p < 0.05). The prevalence of TF at discharge in our study is comparable to previous studies. Infants with CHD admitted at birth, received higher presurgery milk calories, and clinical GERD are significant risk factors for TF. Mitigating the effects of identified risk factors for TF will have significant impact on the quality of life for these infants and their families and may reduce health care cost. · Oral feeding difficulty in infants after congenital heart disease surgical repair is common.. · Such infants require prolonged hospital stay and higher risk for tube-feeding at discharge.. · Identifying modifiable risk factors associated with tube-feeding can enhance clinical outcomes..

Risk of dysphagia in a population of infants born pre-term: characteristic risk factors in a tertiary NICU

ObjectiveTo examine the prevalence and characteristics of dysphagia and suck-swallow-breath incoordination as phenotypes of oral feeding difficulties. MethodA cross-sectional study with secondary data collected consecutively over 2 years from October 2020 to October 2022 to measure the prevalence of swallowing and oral feeding difficulty in preterm infants using Flexible endoscopic evaluation of swallowing examination at the tertiary Integrated Dysphagia Clinic. ResultsThe prevalence of swallowing disorders was 25 % and the prevalence of suck-swallow-breath incoordination was 62.5 %. The significant risk factor that may show a possible correlation with oral feeding difficulty was mature post-menstrual age (p = 0.006) and longer length of stay (p = 0.004). The dominant percentage of upper airway abnormality and disorder were retropalatal collapse (40 %), laryngomalacia (42.5 %), paradoxical vocal cord movement (12.5 %), and gastroesophageal reflux disease (60 %). The dominant characteristic of oral motor examination and flexible endoscopic evaluation of swallowing examination was inadequate non-nutritive sucking (45 %), inadequate postural tone (35 %), and inadequate nutritive sucking (65 %). ConclusionDysphagia in preterm infants is mostly observed in those with mature post-menstrual age, longer length of stay, and the presence of gastroesophageal reflux disease with inadequate non-nutritive sucking and nutritive sucking abilities. Suck-swallow-breath incoordination is primarily observed in those with immature post-menstrual age, a higher prevalence of cardiopulmonary comorbidity, and a higher prevalence of upper airway pathologies (laryngomalacia, paradoxical vocal cord movement) with inadequate nutritive sucking ability.

Assessment and Rehabilitation of Oral Feeding Difficulties in Preterm Infants

Assessment and Rehabilitation of Oral Feeding Difficulties in Preterm Infants

Safe and secure laparoscopy-assisted jejunostomy tube placement using a percutaneous loop needle device in an infant.

We, herein, report a surgical technique for laparoscopy-assisted jejunostomy tube placement in an infant using a loop needle device to fix the jejunum and abdominal wall. A 3-year-old boy with Down's syndrome underwent nutritional management by gastrostomy due to oral feeding difficulty after radical surgery for congenital duodenal stenosis and following bile duct stenosis. However, intractable gastrostomy site leakage emerged; hence, laparoscopy-assisted gastrostomy takedown and simultaneous laparoscopy-assisted jejunostomy tube placement were planned. After laparoscopy-assisted gastrostomy closure was performed, the jejunum was extracted through the umbilical trocar wound. A jejunostomy tube kit was inserted at the left side of the umbilicus. After tube insertion into the jejunum, the jejunostomy tube was wrapped with four interrupted sutures using the Witzel technique. Suture threads were extracted by percutaneous insertion of a loop needle device, and then, the jejunum was fixed to the abdominal wall. The post-operative course was uneventful. Percutaneous insertion of a loop needle device is useful for fixing a jejunostomy tube to the abdominal wall using the Witzel technique. Our technique is safe, secure and expected to reduce the risk of dislodgement of tube and peritonitis associated with jejunostomy tube placement.

THE ATTITUDES OF CHINESE AMERICAN FAMILY CAREGIVERS OF OLDER PEOPLE WITH DEMENTIA TOWARD TUBE FEEDING

Abstract The aim of this study is to examine the attitudes toward feeding in Chinese American dementia family caregivers. 53 Chinese American dementia caregivers were interviewed in 2021-2022. The survey included their anticipated decisions for tube feeding if their care recipients are at the end of life, goals of care, and knowledge of tube feeding. 32.7% of them would choose tube feeding for individuals with dementia at the end of life; however, 53.9% are confident that the patient would not choose tube feeding if patients are able to make decisions. 92.3% prepared to relieve pain and discomfort as much as possible. 42.3 % of them thought tube feeding are recommended to use if oral feeding difficulties arise. More than 60% thought tube feeding can improve survival and nutrition status. More education is needed for the decision-making regarding tube feeding at the end of life for Chinese American dementia caregivers.

Multiple hamartomas of the oral cavity with bifid tongue and cleft palate: A unique observation

Background: Hamartomas are developmental tumor-like malformations, comprising a mixture of cytologically mature tissues that are indigenous to their anatomical location. Within the oral cavity, these lesions are usually multifocal, relatively small, and asymptomatic, and have been frequently linked to present as a manifestation of the oro-facial-digital-syndrome. Case Presentation: A 2-day-old female newborn was referred with two masses protruding from the oral cavity, feeding difficulties, and dribbling of milk during feeding. The patient had no dysmorphic features and no obvious associated anomalies. On careful examination, there was one mass protruding from the undersurface of a bifid tongue with another larger mass that seemed to originate from the hard palate. Complete surgical excision of both masses and repair of the bifid tongue were done to relieve the baby’s feeding difficulties. Histopathological examination of both specimens confirmed the diagnosis of multiple hamartomas. Conclusion: The occurrence of multiple and large oral hamartomas in newborns might result in feeding difficulties that necessitate urgent surgical intervention. The association of lingual and palatal hamartomas, with bifid tongue and cleft palate, is a unique observation that is not reported in the English literature.

The Evaluation of Facial Muscles by Surface Electromyography in Very Preterm Infants.

It is reported that 40% of preterm infants have problems with eating. Neonatal feeding disorders may be one of the factors increasing neonatal mortality. The aim of our study was to evaluate the muscles involved in suckling and swallowing in premature newborns using surface electromyography (sEMG). We would like to objectively describe the tension of muscles engaged in feeding in order to properly plan the therapy. Another aim was to compare sEMG measurements to gestational age, birth weight, and umbilical blood pH to show which parameters put children at risk of feeding problems. Sixteen preterm neonates with gestational age less than 32 weeks, birth weight less than 1500 g, and oral feeding difficulties were analyzed for muscle response and electrical activity of nerves using sEMG (surface electromyography). We found a negative correlation indicating that preterm infants with a younger gestational age had higher suprahyoid muscle tension, and a positive correlation was found between pH value and suprahyoid muscles. The lower the pH value, the lower the tension in the suprahyoid muscles. sEMG may be a helpful diagnostic tool in the evaluation of the masticatory system of premature infants. Due to the abnormal tone of the muscles responsible for swallowing, it is advisable to rehabilitate as early as possible.

A Unique Clinical Tool for the Evaluation of Oral Feeding Skills in Infants.

Background. Occupational therapy practice for oral feeding assessment is based on clinical observation of infants' sucking, swallowing, and breathing ability, which is influenced by clinical experience and provides poor evidence on explanatory factors. Purpose. To test the clinical utility and safety of a nipple monitoring device for the quantitative evaluation of oral feeding skills. Method. Sixteen infants, with no severe medical complications, participated in a pre-experimental pilot study. Oral feeding performances (duration, intake volume, and rate of transfer), and occurrence of adverse events (apnea, bradycardia, and oxygen desaturations) were recorded to ensure the tool does not interfere with infant's feeding ability or does not create any adverse effects. Findings. There was no significant difference in duration, intake volume, rate of transfer between the two monitored sessions, and no occurrence in adverse events. Implications. The findings suggest that the nipple monitoring device may be used for quantitative assessment and intervention planning of oral feeding difficulties in infants.

Effectiveness of multistimulation approach on feeding habits of low-birth-weight babies-A randomized control trial.

IntroductionLow- birth- weight neonates face oral feeding difficulties due to hemodynamic instability, immaturity of central nervous systems, and incomplete development of oral functions. Use of several interventions might help in improvement of the feeding ability of neonates. The objective of the study was to evaluate the effect of the multistimulation approach in low-birth-weight babies on the oral feeding performance, oral intake volume, weight gain and transition time from tube to total oral intake.MethodsA Randomized, parallel-group, multiple arm trial study was conducted, and a total of 44 low birth weight babies were randomized into three parallel groups with a 2:1:1 ratio. Babies who are Hemodynamically stable were included in the trial. In two Intervention groups, one received an oral stimulation program, another intervention group received tactile stimulation, and the control group received routine newborn procedures for the same duration of time. Oral feeding performance was determined by Oral Feeding Skills (OFS) on a daily basis for five days after providing ten days of intervention. Neonates were monitored until hospital discharge.ResultsInfants in the stimulation groups had significantly better oral feeding performance than infants in the control group in terms of mean proficiency, transfer rate and overall transfer of feeding volume. There was a substantial increase in mean feeding score, daily weight, oral intake volume, and early transition time in both intervention groups compared to control. There was no significant difference in feeding behaviours between the oromotor and multistimulation groups, but the multistimulation group gained more weight compared to the oromotor group.ConclusionsInfants exposed to the stimulation programme had better feeding skills and a shorter transition period from tube feeding to oral feeding; however, the babies who received multistimulation gained greater weight than babies who received only oromotor stimulation. The study recommends multi stimulation in the form of oromotor, and tactile stimulation can be used as an effective NICU procedure for maintaining an infant's ability to take feeds orally before being discharged from the hospital.

EP200: Noonan syndrome associated with focal occipital alopecia in a patient with RAF1 variant: A case report and literature review

Noonan syndrome (NS) is a heterogenous condition characterized by distinctive craniofacial features, cardiac defects, and neurodevelopmental changes and is caused by variants in one of seven genes coding for proteins within the Ras-MAPK pathway. Pathogenic variants in RAF1 account for approximately 3-17% of cases and are associated with a high prevalence of severe hypertrophic cardiomyopathy (HCM) and pulmonary hypertension (PHTN). Cerebrovascular and Chiari I malformations, although rare, have also been described in individuals with RAF-1 NS. To our knowledge, the presence of congenital occipital alopecia in an individual with NS has not been previously reported. We report a patient with a de novo pathogenic heterozygous RAF1 gene sequence variant, c.770C>T (p.Ser257Leu), with atypical scalp findings. Our proband was a 4-week-old female dichorionic, diamniotic twin born at 35 weeks 2 days gestation to nonconsanguineous parents. At birth, her length was 45.7 cm (14th centile), weight was 2656 g (69th centile), and head circumference was 34.5 cm (97th centile). The mother was 38 at the time of birth. There was no history of maternal or gestational diabetes. Prenatal ultrasound was concerning for cystic hygroma. The immediate neonatal course was complicated by acute respiratory failure, left ventricular outflow tract obstruction with concern for coarctation on echocardiogram, poor feeding, and failed newborn hearing screen which prompted transfer to a tertiary NICU for further management. At the time of consultation, the following problems had been identified: bilateral cataracts, significant redundant posterior neck folds that showed fatty soft tissue neck mass without a cystic component, and hypertrophic cardiomyopathy with elevated pulmonary arterial pressures. Physical exam was notable for relative macrocephaly with turricephaly, lowset, posteriorly rotated ears, redundant posterior neck folds, and a focal, 1cm circular area of alopecia at the midline occipital region. These findings were concerning for a RASopathy. Imaging of her superficial midline scalp defect with brain MRI showed a suspected atretic encephalocele, but CT did not reveal any occipital bony defects. After re-evaluation, it was confirmed that there was no intracranial connection or bony defect at the area of the congenital alopecia nor at the level of the suboccipital skin thickening/redundancy. Chromosomal microarray was nondiagnostic and thus exome sequence analysis was preformed which detected the pathogenic RAF1 variant. The diagnosis of RAF1 NS accounts for nearly all of the physical changes seen in our patient, particularly the severe degree of PHTN and HCM, with the exception of her occipital alopecia. Since our initial consultation, the patient continues to grow along NS-specific curve, has persistent mild bilateral hearing loss, and has had interval placement of gastric tube secondary to oral feeding difficulties. There has been mild improvement in cardiac function, septal hypertrophy, and PHTN. She remains seizure-free. This report expands the phenotype of RAF1 Noonan syndrome to include congenital focal alopecia. We recommend that NS should be added to the differential when this same scalp finding is present. Although the presence of this physical finding in our patient was not definitively associated with any underlying structural CNS or bony changes, this case highlights the importance of investigating with detailed cranial and spinal imaging to better characterize cerebral morphology and guide genetic testing in suspected RASopathy cases. Additional cases with this finding are needed to better understand potential associations.

Associations Between Infant Feeding, BMI Trajectory, and Long-Term Clinical Outcome in Patients With a Functional Single Ventricle

Infants with a functional single ventricle (FSV) commonly experience growth faltering and oral feeding difficulties, necessitating tube feeding. We aimed to assess the association between prolonged tube feeding in infants with FSV and long-term growth and post-Fontan clinical outcomes.

The Effect of Oral Motor Therapy on Feeding Difficulties and Eating Behaviors in Younger ASD Children

Background: A variety of feeding difficulties like aversion of food, food selectivity, complete food refusal are the most common problems reported by the Parents of ASD children. Every parent of ASD children struggles to manage their child’s feeding routine as well as undesirable mealtime behaviours, which can cause parental concern and poor family dynamics.&#x0D; Aim: 1) The aim of this study is to analyze the effect of dedicated oral motor work in children with ASD who presented with feeding difficulty. 2) To determine parental concerns related to feeding behavior shown by their child and assess whether an additional home program in parallel with oral motor work by an Occupational Therapist would provide any benefits.&#x0D; Methods: This is a pre and post study where a total number of 18 ASD children at the age of 2-4 year old were participated and selected through BPFAS, out of which 15 copies were considered to be concerned with the oral motor and feeding difficulties of the child. Therefore, 15 participants who were facing feeding difficulties with their children were recruited. Concerned Participants were further assessed by Occupational therapist with the help of ‘The com deall Oro-motor assessment scale for toddlers’.&#x0D; Results: According to The Com Deall Oral-motor assessment scale, some changes observed in the oral motor skills and When parental concern is considered via home plan including mealtime strategies with oral motor activities, the post intervention result showed decrease in Total problem score as well as in Total frequency score.&#x0D; Conclusion: The study concluded that continuous structured direct oral motor work as well as addressing parental concerns via home programs is helpful for the ASD children and gives better understanding of feeding difficulties to their parents. Also, some changes have been seen in oral motor skills through continuous practice of oral motor activities by the therapist.

Risk Factors Associated With Long-term Feeding Problems in Preterm Infants: A Scoping Review.

Infants who are born preterm are at a high risk of encountering feeding problems. The aim of this scoping review is to identify risk factors associated with feeding problems that persist beyond infancy in children (aged 1-6 years) who were born preterm (<37 weeks' gestation). To perform this scoping review, the Preferred Reporting Items for Systematic Reviews and Meta-Analyses framework was applied. To be included in this study, articles had to be published in peer-reviewed journals, had an empirical study design, examined feeding problems relating to the child's feeding ability or refusal to eat certain texture of foods, and assessed risk factors (age, weight, and medical conditions) associated with long-term feeding problems in children who were born preterm. After abstract and full-text screening, a total of 9 articles meeting the inclusion criteria were included in this study. Findings from this study identified that gestational age and birth weight are significant risk factors and that sex, duration of mechanical ventilation, and length of tube feedings are potential risk factors. This study confirms that younger and smaller infants are at a high risk of encountering oral feeding difficulties and early interventions services should be focused on these preterm infants. Evaluation of timing and intensity (dose) of early interventions strategies should be investigated to provide more targeted and effective interventions for younger and smaller preterm infants.

Body adiposity and oral feeding outcomes in infants: a pilot study.

Prevalence of oral feeding difficulties in high-risk infants is increasing. Desire to take orally can be influenced by hunger and satiety, which may influence growth and body fat. To determine the association between body adiposity and infant oral feeding. Retrospective case-control study of infants ≥37-week postmenstrual age (PMA). Infants on tube feeding (cases) compared to birth gestation-matched infants on full oral feeding (controls). Body composition was determined by air displacement plethysmography. Overall, 16 cases vs. 16 controls. At study, cases vs. controls had similar PMA, weight and length z-scores, and calorie intake. The mean oral intake was significantly less in cases vs. controls (66 vs. 168 ml/kg/day, p < 0.001). Cases had significantly higher percentage of fat mass (18.7 vs. 10.9) and fat-mass z-score (1.62 vs. 0.08) (p < 0.05), but similar fat-free mass vs. controls. Five case infants required gastrostomy. Higher body adiposity may worsen the infant oral feeding outcomes.

Oral motor disorder and feeding difficulty during the introduction of complementary feeding in preterm infants.

To investigate the association between oral-motor disorder and feeding difficulty during the process of introducing complementary feeding in preterm infants. This is a cross-sectional, observational, and quantitative study. Preterm infants who had already begun complementary feeding and were up to 24 months corrected gestational age, were followed up at an outpatient clinic for high-risk newborns. The feeding difficulty was assessed using the mother's perception of the presence of defensive behaviors in their child. The measurement of the oral motor skills performance was obtained through the application of an adaptation of the Clinical evaluation protocol of pediatric dysphagia (PAD-PED). The sample consisted of 62 dyads of children with a mean corrected gestational age (CGA) of 13.5 months. Complementary feeding was introduced at the sixth month CGA by almost half of the mothers and most of them reported some sort of difficulty with this activity. Extreme prematurity revealed an association with defensive feeding behavior (p = 0.005), as well as with the initial introduction of food with liquid consistency (p = 0.001), the extended period of time using enteral tube feeding (p = 0.044), the continuous positive airway pressure (CPAP) (p = 0.013) and parenteral nutrition (p = 0.039). There was no significant association between oral motor disorder and feeding difficulty.

Disruptions in the development of feeding for infants with congenital heart disease.

Congenital heart disease (CHD) is the most common birth defect for infants born in the United States, with approximately 36,000 affected infants born annually. While mortality rates for children with CHD have significantly declined, there is a growing population of individuals with CHD living into adulthood prompting the need to optimise long-term development and quality of life. For infants with CHD, pre- and post-surgery, there is an increased risk of developmental challenges and feeding difficulties. Feeding challenges carry profound implications for the quality of life for individuals with CHD and their families as they impact short- and long-term neurodevelopment related to growth and nutrition, sensory regulation, and social-emotional bonding with parents and other caregivers. Oral feeding challenges in children with CHD are often the result of medical complications, delayed transition to oral feeding, reduced stamina, oral feeding refusal, developmental delay, and consequences of the overwhelming intensive care unit (ICU) environment. This article aims to characterise the disruptions in feeding development for infants with CHD and describe neurodevelopmental factors that may contribute to short- and long-term oral feeding difficulties.

Feeding, Communication, Hydrocephalus, and Intracranial Hypertension in Patients With Severe FGFR2-Associated Pfeiffer Syndrome.

Pfeiffer syndrome is associated with a genetic mutation of the FGFR2 (or more rarely, FGFR1) gene, and features the combination of craniosynostosis, midface hypoplasia, broad thumbs and broad great toes. Previous research has identified a wide spectrum of clinical phenotypes in patients with Pfeiffer syndrome. This study aimed to investigate the multifactorial considerations for speech, language, hearing and feeding development in patients with severe genetically-confirmed Pfeiffer syndrome. A 23-year retrospective case-note review of patients attending the Oxford Craniofacial Unit was undertaken. Patients were categorized according to genotype. Patients with mutations located in FGFR1, or outside the FGFR2 IgIII domain-hotspot, or representing known Crouzon/Pfeiffer overlap substitutions were excluded. Twelve patients with severe FGFR2-associated Pfeiffer syndrome were identified. Patients most commonly had pansynostosis (n = 8) followed by bicoronal (n = 3), and bicoronal and sagittal synostosis (n = 1). Seven patients had a Chiari I malformation. Four patients had a diagnosis of epilepsy. Ten patients had with hydrocephalus necessitating ventriculoperitoneal shunt insertion.Feeding difficulties were common (n = 10/12) and multifactorial. In 5/12 cases, they were associated with pansynostosis, hydrocephalus, tracheostomy and tube feeding in infancy.Hearing data were available for 10 patients, of whom 9 had conductive hearing loss, and 8 required hearing aids. Results indicated that 3/4 patients had expressive language difficulties, 3/4 had appropriate receptive language skills. 6/12 patients had a speech sound disorder and abnormal resonance. This study has identified important speech, language, hearing and feeding issues in patients with severe FGFR2-associated Pfeiffer syndrome. Results indicate that a high rate of motor-based oral stage feeding difficulties, and pharyngeal stage swallowing difficulties necessitating regular review by specialist craniofacial speech and language therapists.