Severe Feeding Difficulties Research Articles

Early feeding problems in children with cerebral palsy: weight and neuro‐developmental outcomes

Using prospectively collected data from 13 971 births enrolled in a large population‐based cohort study (Avon Longitudinal Study of Parents and Children: ALSPAC), the prevalence of feeding difficulties at 4 weeks and 6 months of age in 33 children subsequently diagnosed with cerebral palsy (CP) were investigated. It was also assessed whether early feeding difficulties could be predictive of functional and growth outcomes at age 8 years. Weak sucking at 4 weeks of age was reported in 11 of 23 children with CP and in 2206 of 12299 (18%) of the remaining population (p<0.0001), and great difficulties feeding at 6 months of age was reported in two of 21 (10%) of the group with CP and in 373 of 10941 (3.3%) of control participants (p=0.017). Feeding difficulties at 4 weeks of age were associated with the pattern of functional impairment at age 4 years (p=0.009) and at 8 years (p=0.068), being clinically underweight (p=0.01), and having speech and swallowing difficulties (p=0.005) at 8 years of age. Early, persistent, and severe feeding difficulties are a marker for subsequent poor growth, feeding, and developmental outcomes and can identify children with CP who will benefit from gastrostomy feeding.

Identifying feeding problems in mentally retarded persons: development and reliability of the screening tool of feeding problems (STEP)

Feeding problems are extremely common among individuals with mental retardation. Palmer, Thompson, and Linscheid (1975) estimate that 33% of persons with mental retardation have severe feeding difficulties or problems. Furthermore, the consequences of untreated feeding problems can be severe if not fatal. Despite these numbers, little has been done to systematically identify these problems. The Screening Tool of Feeding Problems (STEP) was developed as a means to identify feeding problems presented by persons with mental retardation, and thus facilitate the process of identifying who would benefit from some type of behavioral or medical intervention. Items included in the STEP target feeding problems identified in the literature, in the areas of risk of aspiration, food selectivity, feeding skills deficits, food refusal and associated behavior problems, and nutrition related behavior problems. The current study describes the construction of this scale, provides psychometric data including test-retest and cross rater reliability, and factor analysis data.

Esophageal dysmotility in brothers with an FG-like syndrome

We present 4 brothers with developmental delay, minor anomalies, and symptoms due to gastrointestinal dysmotility. There was some resemblance with FG syndrome, although none of the brothers had sufficient findings to make this diagnosis. The index case presented with at age 1 month with screaming episodes, mild gastro-esophageal reflux (GER), and severe constipation. Esophageal manometry studies were consistent with the diagnosis of "nutcracker esophagus." Symptomatic and manometric improvement followed treatment with oral calcium channel blockers. Two older and less severely affected brothers had similar manometric findings but did not require treatment. A fourth brother with symptoms in infancy now has normal esophageal manometry findings. These boys in all likelihood have an X-linked syndrome with manifestations of FG syndrome, in which treatment with calcium channel blockers, produces clinical and manometric improvement. The FG syndrome is an X-linked syndrome of multiple congenital anomalies/mental retardation with facultative manifestations of gastrointestinal dysmotility, including gastro-esophageal reflux, severe feeding difficulties, and constipation. Esophageal dysmotility, in particular "nutcracker esophagus," should be suspected in infants with the FG syndrome and screaming attacks.

“Outbreak” of Hand Injuries During Hajj Festivities in Saudi Arabia

Micrognathia and glossoptosis may cause severe respiratory and feeding difficulties in newborns with Pierre Robin syndrome. The growth of the mandible is alleged to more or less “catch up” during the first year of life. We could not objectify such a catch-up growth using the noninvasive and harmless measurement of the jaw index. The jaw index is defined as alveolar overjet x maxillary arch/mandibular arch. The alveolar overjet is the frontodorsal distance between the most anterior points of the upper and lower alveolar arches, whereas the maxillary arch is measured from the left tragus to the right tragus via the subnasal point, and the mandibular arch is measured from the left to the right tragus via the pogonion point. The growth of the mandible in 7 Pierre Robin patients was found to be proportionately similar to that observed in healthy control subjects age 0 months (N = 100), 6 months (N = 42), and 1 year (N = 32).

The jaw index: new guide defining micrognathia in newborns.

Newborns with glossoptosis due to micrognathia can suffer from life-threatening respiratory distress and severe feeding difficulties. These characteristic features are found in newborns with Pierre Robin sequence. In the relevant literature, no concensus concerning diagnosis for these children can be found. A guide defining micrognathia can be helpful in the diagnosis of glossoptosis and possible airway obstruction. The jaw index is obtained in newborns by measuring three facial dimensions. In a series of 100 healthy neonates the standard measures were defined. The average jaw index in children with Pierre Robin sequence differs significantly from the reference group and was found to be over 3.6 times the normal value. Micrognathia can be defined with the jaw index.

Subperiosteal release of the floor of the mouth in Pierre Robin sequence: experience with 12 cases.

Between 1983 and 1995, 12 children with Pierre Robin sequence were treated with a subperiosteal release of the floor of the mouth (SRFM) to correct severe upper-airway obstruction (group 1: n = 10) and prolonged feeding difficulties (group 2: n = 2). Eight children had isolated Pierre Robin sequence, while four had associated anomalies. The age at surgery was 2 to 13 weeks (mean, 5.2 wk) in group 1, and 8.5 months to 5 years in group 2. Postoperative endotracheal intubation was maintained for 1 to 14 days, and nasopharyngeal intubation for 3 to 13 days. One child with a tracheostomy prior to SRFM was decanulated 11 weeks postoperatively. Postoperative course was uneventful and control polysomnograms (group 1) showed a marked improvement of the obstructive phenomenons and O2 saturation levels. Children were discharged 2 to 6 weeks postoperatively (mean, 4 wk) except for the child with a tracheostomy (19 wk). The only complication was a wound infection treated with systemic antibiotics. Oral feedings were initiated shortly after extubation: six children were discharged with oral feedings only, one child required supplemental gavages for 6 weeks at home, and three children had persistent inadequate oral intake. We have found SRFM to be an effective surgical procedure for the management of infants with Pierre Robin sequence.

Subperiosteal Release of the Floor of the Mouth in Pierre Robin Sequence: Experience With 12 Cases

Between 1983 and 1995, 12 children with Pierre Robin sequence were treated with a subperiosteal release of the floor of the mouth (SRFM) to correct severe upper-airway obstruction (group 1: n = 10) and prolonged feeding difficulties (group 2: n = 2). Eight children had isolated Pierre Robin sequence, while four had associated anomalies. The age at surgery was 2 to 13 weeks (mean, 5.2 wk) in group 1, and 8.5 months to 5 years in group 2. Postoperative endotracheal intubation was maintained for 1 to 14 days, and nasopharyngeal intubation for 3 to 13 days. One child with a tracheostomy prior to SRFM was decanulated 11 weeks postoperatively. Postoperative course was uneventful and control polysomnograms (group 1) showed a marked improvement of the obstructive phenomenons and O2 saturation levels. Children were discharged 2 to 6 weeks postoperatively (mean, 4 wk) except for the child with a tracheostomy (19 wk). The only complication was a wound infection treated with systemic antibiotics. Oral feedings were initiated shortly after extubation: six children were discharged with oral feedings only, one child required supplemental gavages for 6 weeks at home, and three children had persistent inadequate oral intake. We have found SRFM to be an effective surgical procedure for the management of infants with Pierre Robin sequence.

Juvenile systemic hyalinosis – a rare cause of gingival hypertrophy: a case report

A 5-year-old boy was referred because of gross gingival hypertrophy which caused severe feeding difficulties in addition to obvious aesthetic concern. The patient also suffered from frequent upper respiratory tract infections and diarrhoea. In addition, he had pigmentation on bony prominences of his hands, elbows, knees and ankles, cutaneous nodules behind his ear and granulomatous tissue adjacent to his nose. Excess gingival tissue was removed under general anaesthesia. Histological features suggested a diagnosis of juvenile hyaline fibromatosis, which is considered to represent the same underlying pathological condition as infantile systemic hyalinosis. It is suggested that systemic hyalinosis should be preceded by 'infantile' or 'juvenile' depending on the clinical presentation.

Ohdo-like blepharophimosis syndrome with distinctive facies, neonatal hypotonia, mental retardation and hypoplastic teeth

We report four children with unusual facial features including severe blepharophimosis, ptosis, and a distinctive nose with a broad flat tip and a depressed bridge. All four patients were markedly hypotonic and had severe feeding difficulties and developmental delay. Two had congenital heart defects and all three who survived had hypoplastic teeth. Both of the male patients had cryptorchidism. These four children have a distinctive syndrome which is similar to that reported by Biesecker (J Med Genet (1991) 28: 131-134).

Gastrooesophageal reflux in children with cerebral palsy.

Feeding difficulties, vomiting and recurrent chest infections associated with poor growth and nutrition are common in children with cerebral palsy (CP). However, the role of gastrooesophageal reflux as a possible cause has been little studied. We therefore investigated 23 children with cerebral palsy (median age 2.3 years, range 0.6-11.8) whose symptoms were consistent with gastrooesophageal reflux (severe feeding difficulties 70%, failure to thrive 52%, anaemia 31% and recurrent chest infections 31%). Using 24-hour ambulatory oesophageal pH monitoring, we determined the relationship between gastrooesophageal reflux and: (a) developmental age (Griffith's scales); (b) feeding behaviour skills score; (c) nutritional status and (d) clinical assessment of cerebral palsy (type and severity). Abnormal gastrooesophageal reflux (i.e. reflux index > 5%) was detected in 16 (70%) patients (median reflux index 11.4%; range 5.4-59%). Reflux was unrelated to chronological or developmental age, but there was a significant, unexplained association with male gender (P < 0.01). No correlation was found with feeding behaviour skills score, malnutrition (which was commonly severe) and type and severity of cerebral palsy. We conclude that gastrooesophageal reflux is common and should be sought in symptomatic, neurologically handicapped children as effective treatment is likely to improve quality of life.

Characteristics and management of feeding problems of young children with cerebral palsy.

The nature and extent of feeding difficulties associated with cerebral palsy was assessed in 12 infants with moderate to severe oral-motor dysfunction, compared with a control group. Data were gathered at the infants' homes by interview and by direct observation of mealtimes. The results revealed a range of long-standing problems, for which little management advice had been given. Most case infants were poorly positioned; specially designed seats were not used. The mean duration of mealtimes for case and comparison infants did not differ significantly. Case infants ate and were offered less food than the control infants. Feeding problems usually had persisted since birth and were associated with marked failure to thrive. Multidisciplinary assessments of the severe feeding difficulties of these children are indicated.

Central Nervous System Malformations and White Matter Changes in Pseudo-Neonatal Adrenoleukodystrophy*

Clinical, biochemical and morphological findings in a 16-month-old infant girl with pseudo-neonatal adrenoleukodystrophy are reported. The parents were first cousins and the baby was born at term, small for gestational age. The neonatal period was characterized by convulsions resistant to treatment, generalized, severe muscle hypotonus, feeding difficulties and poor weight gain. Developmentally she remained at a neonatal level. A CT-scan showed low density of cerebral white matter and MR examination white matter changes, a thin corpus callosum, cerebellar malformation and dorsal displacement of the brainstem. There was an accumulation of very long chain fatty acids (VLCFA) in serum lipids and cultured skin fibroblasts but plasmalogen and phytanic acid levels were normal. A liver biopsy revealed enlarged peroxisomes staining for catalase. Three similar cases have been reported previously; in two of these there was a deficiency of acyl-CoA oxidase. MR evidence of leukodystrophy combined with gross cerebral and cerebellar morphologic changes have not been reported earlier.

Neuropathologic Observations in Congenital Phrenic Nerve Palsy

A right phrenic nerve palsy with severe respiratory and feeding difficulties throughout the patient's 2 1/2 years of survival was found at autopsy to relate to grossly complete atrophy of the muscle of the right diaphragm. An oblique fibrotic scar transected the right phrenic nerve at the level of the brachial plexus. There was marked reduction of the number of anterior motor neurons in the right central portion of cervical segments 2, 3, and 4. The lesion in the phrenic nerve is what would be expected following stretching during delivery.

Apparent G syndrome presenting as neck and upper limb dystonia and severe gastroesophageal reflux

We have studied a 3-month-old boy with severe gastroesophageal reflux, feeding difficulties, neck and upper limb dystonia, abnormal ears, normal genitalia, and anatomically apparently normal larynx and trachea. Initially diagnosed as suffering from Sandifer "syndrome," he was treated with a gastrostomy and Nissen fundoplication. However, his characteristic facial appearance subsequently led to the diagnosis of G syndrome.

Severe feeding difficulty in infants with increased pulmonary blood flow : A. R. C. Dobell, P. P. Reddy, R. E. Kavey and P. B. Pencharz. J Thorac Cardiovasc Surg 72:303–305 (August), 1976

Severe feeding difficulty in infants with increased pulmonary blood flow : A. R. C. Dobell, P. P. Reddy, R. E. Kavey and P. B. Pencharz. J Thorac Cardiovasc Surg 72:303–305 (August), 1976

Severe feeding difficulty in infants with increased pulmonary blood flow

Three infants with significant left-to-right intracardiac shunts and moderate cardiac disability failed to thrive primarily because of a complete distaste for food. They refused to feed, spat out food introduced into their mouths, and vomited food that reached their stomachs. These severe feeding difficulties were relieved by intracardiac repair, and we conclude that in some unusual instances such feeding disorders constitute the indication for operation.

Left aortic arch with right descending aorta and right ligamentum arteriosum: A rare form of vascular ring

The combination of left aortic arch, right descending aorta, and right patent ductus arteriosus is a rare form of aortic arch anomaly. Only 11 such cases have been previously reported. A 6-month-old infant with this anomaly manifested severe respiratory difficulty and feeding problems in the early newborn period. The aortic arch abnormalities were confirmed by angiocardiography, and operative correction was successful. This is the first case reported in which the patient developed severe respiratory and feeding difficulties due to this type of a vascular ring.

Fetal face syndrome with mental retardation.

Two cases of a variant of the fetal face syndrome are described. The affected babies were born from two consecutive pregnancies to the same gypsy parents. In addition to the classical syndrome, both cases showed a severe feeding difficulties, failure to thrive and severe psychomotoric retardation and one of them cleft lip and palate.