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\begin{document}

\title{Management of gastrointestinal and nutritional problems in children on
home invasive mechanical ventilation}



\author[1]{Ceyda Tuna Kırsaçlıoğlu}%
\affil[1]{Ankara University Faculty of Medicine}%


\vspace{-1em}



  
  \date{May 25, 2024}


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\selectlanguage{english}
\begin{abstract}
In recent decades, the patient survival is increased due to the advances
in intensive care units and development of modern mechanic ventilators.
Unfortunately, it is not always possible to wean these children from
mechanical ventilation. Recently, after placement a tracheostomy tube,
they can support at home with non-invasive or invasive mechanical
ventilation. Most of the children who need ventilation support at home
have neurological impairment. The nutritional issues and
gastrointestinal complications are well defined in critically ill
patients, but there are very limited studies on the children with
tracheostomy. Considering that majority of the patients has
neuromuscular disorders, the nutritional and gastrointestinal problems
of the children with tracheostomy are discussed, in the light of the
knowledge on critically ill patients.%
\end{abstract}%



\sloppy


\textbf{Management of gastrointestinal and nutritional problems in
children on~home~invasive~mechanical~ventilation}

Associate Professor, M.D. Dr. Ceyda TUNA KIRSA\selectlanguage{ngerman}ÇLIO\selectlanguage{polish}Ğ\selectlanguage{english}LU

Ankara University School of Medicine, Department of Pediatrics, Division
of Pediatric Gastroenterology, Hepatology and Nutrition, Ankara, T\selectlanguage{ngerman}ürkiye

E mail:\emph{ckirsaclioglu@ankara.edu.tr},\emph{ceytun@yahoo.com}

ORCID number: 0000-0002-3551-7267

Keywords:

Children; constipation; feeding; gastroesophageal reflux;
gastrointestinal; mechanical ventilation; nutrition; tracheostomy.

Running head:

Nutrition, gastrointestinal issues and tracheostomy

\textbf{Abstract}

In recent decades, the patient survival is increased due to the advances
in intensive care units and development of modern mechanic ventilators.
Unfortunately, it is not always possible to wean these children from
mechanical ventilation. Recently, after placement a tracheostomy tube,
they can support at home with non-invasive or invasive mechanical
ventilation. Most the children who need ventilation support at home have
neurological impairment. The nutritional issues and gastrointestinal
complications are well defined in critically ill patients, but there are
very limited studies on the children with tracheostomy. Considering that
majority of the patients has neuromuscular disorders, the nutritional
and gastrointestinal problems of the children with tracheostomy are
discussed, in the light of the knowledge on critically ill patients.

\textbf{Key words:} Children; constipation; feeding; gastroesophageal
reflux; gastrointestinal; mechanical ventilation; nutrition;
tracheostomy.

\textbf{Management of gastrointestinal and nutritional problems in
children on~home~invasive~mechanical~ventilation}

Advances in neonatal/pediatric intensive care units and mechanical
ventilation (MV) over the last three decades have increased the patient
survival in worldwide. Despite increased survival, the patients who
couldn't be weaned from MV in intensive care units due to chronic
respiratory failure caused by different etiologies, have been emerged
the need of MV support at home. With the development of more modern and
advanced home ventilators, home invasive mechanical ventilation (HIMV)
has been used widely in children with chronic respiratory failure all
over the world\textsuperscript{1-3}.

The children who have progressive respiratory failure or who could not
weaned from MV in intensive care units need long term mechanical
ventilation. When a non-invasive MV support is not an option, invasive
MV support via tracheostomy can be continued at home, after clinical
stability have been established at hospital\textsuperscript{1,4}. To be
a clinically stable child the followings are required; a stable airway,
stable oxygen requirement (\textless{} 40\%), stable ventilator settings
that haven' been changed over a period of time, the need for infrequent
laboratory testings, a stable home care plan and an adequate nutrition
to maintain growth\textsuperscript{1,4}. A safe and stable enteral
feeding is as important as a stable airway. While preparing the child to
discharge, not just preparation for MV, but also a detailed nutritional
assessment and adjustment should be done. The caregiver (the parents,
the homecare providers) training should be performed not only for MV,
but also for nutritional issues before discharge. Besides giving
information for the acute life-threatening events related to MV and
underlying disease, the importance of nutrition and negative effects of
inadequate nutrition on growth, development and respiratory functions
should be explained\textsuperscript{1,5}.

In childhood HIMV is generally required for neuromuscular, central
nervous system and cardiopulmonary disorders. The indications for HIMV
support in children are given in Table 1. The majority of these children
have neuromuscular disorders, therefore it is not possible to directly
associate the gastrointestinal and nutritional problems with mechanical
ventilation\textsuperscript{1-3}.

\textbf{Nutritional assessment and nutrition management of the children
with tracheostomy}

The global problem of the world, malnutrition leads to loss of lean body
mass, growth and developmental delay, muscle weakness (e.g., decreased
respiratory muscles' and diaphgram strength), immune dysfunction (e.g.,
altered humoral and cellular immunity and increased bacterial adhesions
in the lower respiratory tract, increased infections), delayed wound
healing and prolong hospital stay \textsuperscript{6-8}. To prevent and
improve malnutrition, the children with tracheostomy and HIMV should be
evaluated for the nutritional status at hospital and after discharge
regularly. The frequency of the visits is determined by the patients'
malnutrition degree, weight gain rate, presence of feeding intolerance
or gastrointestinal symptoms. If the patients are stable, visits should
be performed every 1 to 3 months for infants and every 3-6 months for
older children\textsuperscript{1-3,6}.

The nutritional assessment of children consists of a detailed dietary
history, physical examination findings, anthropometric measurements and
basic laboratory tests. During hospitalization and at every visit,
patients' detailed dietary intake (including water) and nutritional
requirements should be determined. The deficiencies of vitamin and trace
elements may reveal some pathological physical findings such as angular
chelitis, keratitis, dermatitis, hair loss. Decubitus skin ulcers and
poor peripheral circulation findings are the important physical
examination findings related to undernutrition particularly in
neurologically impaired patients. The anthropometric measurements should
be recorded during hospitalization and should be routinely monitored in
every visit. The length or height, body weight, head circumference
measurements, weight for length and body mass index values should be
evaluated due to age and gender of the patients and z-scores should be
calculated. The growth charts and z-scores are very useful to monitor
the growth of the children. The segmental measurements such as tibial
and knee-heel length are useful in estimating height of the patients who
cannot be measured due to severe contracture. In addition mid-upper arm
circumference and triceps skin fold measurements are valuable in
detecting acute malnutrition and also determining the effect of
nutritional support on follow-up. Malnutrition definition generally
based on the antropometric measurements. The severity of malnutrition is
defined according to z-scores. Weight for age, weight for height, body
mass index are mostly used indices to define malnutrition. European
Society for Pediatric Gastroenterology, Hepatology, and Nutrition
(ESPGHAN) and Academy of Nutrition and Dietetics/American Society of
Parenteral and Enteral Nutrition (ASPEN) recommendations for definition
moderate-severe acute malnutrion are given in Table 2. Also Academy of
Nutrition and Dietetics/ASPEN defined inadequate nutrient intake as a
risk factor for severe and moderate malnutrition, when nutrient intake
is \selectlanguage{english}[?]25\% and 26\%-50\% of estimated energy/protein requirement,
respectively\textsuperscript{7,9-11}.

Routine laboratory evaluation including hemoglobin levels, renal
function tests, albumin, liver enzymes, fasting blood glucose should be
performed. ESPGHAN recommended routine screening of vitamins (vitamin D,
vitamin B12, folate, vitamin A, vitamin E), macrominerals (calcium,
phosphate, magnesium), trace elements (zinc, iron), ferritin and
parathormon in children with chronic conditions under high risk of
malnutrition. The measurements should be performed in the absence of
systemic inflammation because blood levels of vitamins and trace
elements may be affected from systemic inflammation. In addition, a
simultaneous measurement of acute phase reactans and albumin level is
required \textsuperscript{6,9,12}.

The main purpose of nutritional support is to optimize growth and
development. The nutritional requirement of every children changes due
to own clinical condition and underlying disease. The patients who have
undernutrition need higher energy requirements to catch-up growth.

Energy requirement of the children with neurological impairment (NI)
differs due to the heterogeneity of clinical features. It depends on
severity of the NI, muscle tone, physical activity level, mobility and
presence of malnutrition. ESPGHAN recommended the use of dietary
reference intake (DRI) standarts to estimate the energy need. But
sometimes DRI may overestimate the energy needs of the children with NI.
For instance total energy expenditure (TEE) / resting energy expenditure
(REE) ratio is lower in quadriplegic cerebral palsy compared to children
with normal activity (TEE/REE ratio is 1.1 and 1.5-1.6 respectively).
Additionally the overestimation of energy need may result in obesity or
being overweight. On the other hand the children with athetosis need
more energy. At least in the beginning `The Schofield equation' may be
used to calculate basal metabolism rate (Table 3)\textsuperscript{13}.
On follow-up according to the weight gain velocity, activity and stress
factors further dietary interventions can be made. In children with NI,
recommended DRI for protein is same for healthy children. In case of
decubitus ulcers or severely undernourished patients the protein intake
may be increased to 2-2.4 g/kg/day\textsuperscript{6,9}. The DRI values
for energy and protein for healthy children are given in Table
4\textsuperscript{14}.

In the patients with Duchenne muscular dystrophy (DMD), the Schofield
equation considered as an accurate predictive equation and they should
receive 100\% of DRI for energy. The energy requirement should be
specialized based on ambulation, physical activity and current weight of
the patient. For instance, some reports suggested the estimated energy
need is 80\% of DRI in ambulatory DMD boys, and 70\% of DRI in
non-ambulatory boys with DMD. In the presence of weight loss, energy
intake can be increased to 130\%-150\% of DRI in DMD boys. Similarly
protein intake should meet the DRI for age\textsuperscript{15,16}.

The accurate estimation of REE of the infants with spinal muscular
atrophy (SMA) type 1 should be based on nutritional and ventilation
status (spontaneous, non-invazive or invasive MV) of the patient. The
tracheostomy and invazive MV leads to 50\% of decrease in REE, but
spontaneously breathing infants need more energy. The estimated amount
of energy is recommended to be 9-11 kcal/cm/day by `The International
Standarts of Care for SMA' \textsuperscript{17}.

The patients with cystic fibrosis may have increased REE and increased
nutrient loss due to pulmonary, pancreatic and hepatic involvement To
maintain growth, development and pulmonary functions, daily energy and
protein requirements should be 120-150\% of DRI and 150\%-200\% of
DRI\textsuperscript{16}. The recommended daily energy intake for the
infants with bronchopulmonary dysplasia (BPD) is 120-150
kcal/kg\textsuperscript{18}. Luo J\textsuperscript{19} et al. reported a
significant increase in weight and length z-scores of the infants with
BPD and a decreased energy requirement within 4 weeks after tracheostomy
placement.

The water intake is adjusted based on the healthy children's daily
requirements and can be calculated by Holliday Segar formula (Table
5)\textsuperscript{20}. Daily water need is equal to daily caloric
expenditure and approximately 100 ml/kg water for every 100 kcal/kg is
needed in normal physiologic conditions. The water losses due to
pathological conditions should be given
separately\textsuperscript{15,20}.

Enteral tube feeding is required when the patients can't feed by oral
route or oral intake is not sufficient for nutrition, hydration, growth
and even medication intake. It may be performed through a nasogastric,
nasojejunal, percutaneous endoscopic gastrostomy (PEG), or jejunal tube
{[}PEG with a jejunal extension (PEG-J), percutaneous endoscopic
jejunostomy (PEJ) or surgical jejunostomy{]}. When the patient need
non-oral nutrition support more than 3-6 weeks, PEG is
indicated\textsuperscript{21}. There is no need to investigate the
children for gastroesophageal reflux in the absence of related symptoms
prior to PEG placement\textsuperscript{6,21}. The long term MV requires
a safe enteral feeding route. Goneidy A. et al\textsuperscript{22}.
suggested to insert a gastrostomy tube at time of tracheostomy formation
in children receiving long term ventilation and have been already
feeding with NG tube.

Percutaneous endoscopic gastrostomy placement should be performed under
general anaesthesia by a trained staff. Aseptic cleaning (simple washing
is sufficient) and sterile dry dressing of the wound site is recommended
for one week. While site is healing, a clear discharge may be seen. The
parents or caregivers should be trained for feeding tube and stoma care.
They should be informed for possible complications such as tube removal,
clogging. In daily care, infectious signs should be looked for the
gastrostomy site. The parents or caregivers should be informed for the
importance of nutrition. They should be trained for feeding techniques
before discharge\textsuperscript{21,23}.

Three hours after PEG placement, feeding can be started. Isoosmolar
formulas are the best choice to start feeding. The first choice should
be bolus feeding because it is more physiological. If the patient hasn't
tolerated the bolus feeding via NG tube previously, the alternative will
be continuous feeding. The targeted energy intake is determined by
assessing the nutritional status of the children. The feeding volume
should be increased gradually both in bolus and continuous feeding
because excessive feeding may cause abdominal distension, which may
affect respiratory function. A stepwise increase of feeding is useful to
monitor feeding intolerance or the other gastrointestinal
complications\textsuperscript{21}.

Enteral feeding formulas are chosen according to the patients age,
underlying disease and tolerance. Breast milk, infant formulas,
isocaloric and hypercaloric formulas may be used for enteral feeding.
Formula may be changed depend on calorie requirements or volume
restriction. Also feeding type or content of formula can be changed due
to feeding intolerance in follow-up\textsuperscript{21}. Adjusting a
safe feeding regimen and optimal formula before discharge is essential
for the patient with tracheostomy,

\textbf{Gastrointestinal problems in children
on~home~invasive~mechanical~ventilation}

The gastrointestinal (GI) problems in critically ill patients who were
given MV support in intensive care units have been demonstrated both in
animal and human studies\textsuperscript{24-26}. During MV, splanchic
hypoperfusion seems to play an important role in the pathogenesis of GI
complications due to cardiocirculatory dysfunction. In particular, it
was reported that high positive end-expiratory pressure (PEEP) levels
(PEEP \textgreater{}15 cmH\textsubscript{2}O) cause an increased right
ventricular afterload which lead to an increase in right atrial
pressure, decreased systemic venous return and cardiac output and
splanchic perfusion respectively\textsuperscript{24-27}. Also acute
moderate increase in arterial carbondioxide level has been observed to
increase hepatic and splanchic blood flow in a biphasic manner;
initially reducing of blood flow due to sympathetic stimulation, then
increasing blood flow due to direct vasodilator effect of
carbondioxide\textsuperscript{28}. Despite there are conflicting results
on the effect of PEEP on hepatic blood flow as decreased or no change.
The decrease in portal flow may be compansated with increased arterial
blood flow in order to maintain hepatic blood
flow\textsuperscript{29,30}. The gastro-esophageal reflux disease,
dysphagia, delayed gastric emptying and constipation are the most common
gastrointestinal problems of the children with tracheostomy on
MV\textsuperscript{31-33}.

\textbf{Gastro-esophageal reflux disease}

Gastro-esophageal reflux (GER) is the passage of gastric contents into
the esophagus, with or without regurgitation and vomiting. North
American Society for Pediatric Gastroenterology, Hepatology, and
Nutrition (NASPGHAN) and ESPGHAN defined pediatric gastro-esophageal
reflux disease (GERD) as GER leads to troublesome symptoms which affect
daily functioning and/or complications, such as hematemesis, dysphagia,
erosive esophagitis, poor weight gain, feeding refusal, dystonic neck
posturing, apnea, recurrent aspiration pneumonia, coughing or choking at
the end of feeding in infants and children. The children who had
specific underlying conditions such as neurolgical disease, pulmonary
disease (e.g., cystic fibrosis), congenital gastrointestinal
abnormalities (e.g.,diaphragmatic hernia, esophageal atresia, intestinal
atresia.), obesity or prematurity are more vulnerable for
GERD\textsuperscript{34}. Lower esophageal sphincter (LES), angle of His
(angle between the esophagus and the gastric fundus), the crural
diaphgram and phrenoesophageal ligament are the components of the
anti-reflux barrier. The LES relaxes during swallowing and allows the
pass of esophageal contents to the stomach. The postprandial gastric
distension triggers transient LES relaxations (TLESRs) and the gastric
contents move upward into the esophagus. Despite TLESRs are considered
to be the major cause of GER episodes, delayed gastric emptying,
decreased LES pressure, increase in the His angle, increased
intraabdominal pressure may lead GER. Coughing, straining, increased
respiratory effort, medications, obesity, lying on right side position
may cause GER due to anti-reflux barrier
impairement\textsuperscript{35-38}.

In the study of mechanical ventilated critically ill patients, the basal
LES pressure was found to be low and a minimal increase in abdominal
pressure due to such as suctioning, straining, coughing may cause
frequent reflux episodes. Also medications like anti-cholinergic drugs,
calcium canal blockers, barbiturates may relax LES\textsuperscript{39}.
Enteral nutrition related gastric distension or delayed gastric emptying
may increase the frequency of TLESRs and causes increased GER episodes
in these patients\textsuperscript{31}. Newton M et
al\textsuperscript{40}. reported that GERD was diagnosed in 20\% of the
children with tracheostomy and of these children 12.4\% had tracheostomy
related complications, with odds ratio of 1.5 for developing
tracheostomy related complications. Ertu\selectlanguage{polish}ğ\selectlanguage{english}rul A et
al\textsuperscript{41}. demonstrated GERD was the major co-morbidity
(\%28) in children dependent HIMV, most of them had an underlying a
neuromuscular disease. In the study of Blanchi ET et
al.\textsuperscript{42}, the prevalence of reflux in the adults with
tracheostomy was reported as 45.2\% and there was no typical symptoms
most of 24-hour pHmetry and manometry measurement.

In the presence of the GERD related symptoms in infants and children
with MV, red flag symptom and signs should be investigated (Table 6). In
the presence of these findings the appropriate tests and radiologic
evaluation should be performed to rule out other pathological conditions
(Table 7)\textsuperscript{34-38}.

The treatment of GERD in otherwise healthy infants and children were
detailed in ESPGHAN and NASPGHAN recommendations\textsuperscript{34}. In
suspicion of GERD and if there is no red flag symptom and/or signs, the
first step in treatment should be feeding modification. The head
elevation and positioning is not recommended in sleeping infants due to
the risk of sudden infant death syndrome\textsuperscript{34}, but there
is no clear data for the infants with tracheostomy.

The nutritional interventions should be made according to patients
underlying illness, concomitant symptoms or disorders, age and
nutritional status. Food thickeners may be given to orally feeding
patients. Reducing feeding volumes, increasing feeding frequency and
avoiding overfeeding may relief the symptoms. If the infant fails to
these modifications, an extensively hydrolyzed protein-based formula and
in breastfed infants elimination of cow's milk from maternal diet should
be considered for 2- to 4-weeks. If there is no response to extensively
hydrolyzed protein based formula, an amino-acid based formula may be
given. In children with NI who fed with tube whey based formula may be
given, it also help to gastric emptying. If there is no improvement,
acid suppression treatment should be considered for 4-8 weeks. The use
of alginates may slightly improve the GERD symptoms and signs but
ESPGHAN and NASPGHAN do not recommend use antacids/alginates for chronic
treatment of infants and children with GERD. Also proton pump inhibitors
(PPIs) may be considered in the treatment. If the symptoms improve in
4-8 weeks period, PPIs can be ceased\textsuperscript{6,34-38}.

If there is no response to the optimal medical treatment in 4-8 weeks
(refractory GERD) or weaning attempts of PPIs are failed within 6-12
months period, possible underlying conditions which may lead to GERD
symptoms and efficacy of treatment (e.g., drug usage, dosage, adherence,
interactions) should be evaluated.

In suspect of congenital gastrointestinal abnormalities such as hiatal
hernia, pyloric stenosis, malrotation, duodenal or antral web, duodenal
or esophageal stenosis, or esophageal stricture, achalasia, extrinsic
compression of esophagus barium/water soluble radiopaque swallowing
follow-through studies should be performed. Abdominal ultrasound also
may be helpful to detect the congenital abnormalities. Follow-through
studies and abdominal ultrasound is not recommended to diagnose
GERD\textsuperscript{34-38}.

In the suspicion of GERD due to persistent troublesome symptoms, the
association of the symptoms and gastroesophageal reflux events (both
acid and non-acid) should be identified by pH-multichannel intra-luminal
impedance (MII). If it is not available only pH-metry can demonstrate
the relation of symptoms only with acid GER events.

Esophagogastroduodenoscopy with biopsies should be performed to clarify
the etiology of esophagitis (ie, eosinophilic esophagitis) or identify a
mucosal disease as a complication of the GERD such as erosive
esophagitis and Barrett esophagitis\textsuperscript{34-38}.

In the presence of reflux-related erosive esophagitis in infants and
children, the first-line treatment is proton pump inhibitors (PPIs). If
PPIs are not available histamine-2 receptor antagonists (H2RAs) may be
used. Baclofen may be considered before surgery in refractory GERD.
Kawai et al\textsuperscript{43}. reported a decrease in vomiting, number
of acid refluxes with the gamma-aminobutyric acid type B receptor
agonist baclofen. The use of domperidon, metoclopramide or any other
prokinetics (e.g.,,erythromycin) in infants and children with GERD is
not recommended\textsuperscript{34}.

Since the incidence of GERD in neurologically impaired children is high
(up to 70\%) for these clinically fragile patients, ESPGHAN recommended
using whey-based formulas, thickening the enteral formulas, and PPIs
treatment before diagnostic tests.. Despite GERD symptoms cannot be
expressed by severely neurologically impaired patients, objective
diagnostic tests such as esophageal pH-metry or pH/multichannel
intraluminal impedance monitoring, and/or upper GI endoscopy will be
benefical to determine the futher need of PPIs
treatment\textsuperscript{6,34}.

In the treatment of infants and children with GERD who are refractory to
optimal medical treatment, transpyloric or jejunal feedings may be an
alternative treatment to fundoplication, but there is no strong
evidence. In neurologically impaired children both procedures didn't
prevent aspiration pneumonia. This condition may be independent of the
reflux events and related to their swallowing
dysfunction\textsuperscript{34,44}. The complication rates of
transpyloric or jejunal feeding reported to be considerably high about
85\%; including clogging, dislodgement, intussusception, and
perforation\textsuperscript{45,46}.

Anti-reflux surgery is recommended as foloows; life threatening
complication under optimal medical treatment, refractory GERD symptoms,
underlying a chronic condition (e.g.,neurologically impaired, cystic
fibrosis), need for chronic pharmacotherapy to control the signs and/or
symptoms of GERD. Fundoplication leads to decrease the baseline pressure
of LES, the number of TLESRs and increase the length of intra-abdominal
esophagus. Despite different anti-reflux surgical procedures, Nissen
fundoplication is accepted as gold standart technique, with shorter
hospital stay and low incidence of perioperative complications and also
morbidity. Unfortunately, no reduction of extraesophageal symptoms such
as pneumonia and mechanical ventilation requirement was demonstrated in
neurological impaired children after
fundiplication\textsuperscript{44,47,48}. Also, more than 75\% of the
patients couldn't cease acid suppression medications for at least one
year after fundoplication\textsuperscript{49}\emph{.} After
fundoplication gas-bloat, early satiety or pain after feeding,
dysphagia, retching, worsening aspiration risk from esophageal stasis,
unwrapping and redo fundoplication (2.2\%-12.2\%) are the other reported
side effects\textsuperscript{50,51}.

Total esophagogastric disconnection may be considered as a rescue
procedure for neurologically impaired children with a failed
fundoplication Since these complications may inversly effect the
patients with tracheostomy, the parents should be carefully informed for
the risks and benefits of the surgical
interventions\textsuperscript{34}.

\textbf{Dysphagia}

The children with tracheostomy may be fed by oral route and/or by tube,
depending on underlying conditions and swallowing abilities. Mainly, the
severity and characteristics of neurological impairment are
determinative of feeding route. For instance, the lack or diminished gag
reflex, the disorders with bulbar involvement require tube feeding.
Factors that may affect feeding of the children with tracheostomy are
given in Table 8 \textsuperscript{33,52}.

Swallowing is a complex process which is controlled by brain stem,
cortex and enteric nervous system that innervates esophagus smooth
muscles. There are three phases of swallowing. The oral phase begins
with opening of the mouth and continues with sucking, biting, chewing.
The tongue movements and addition of the saliva allow the food bolus to
pass into oropharynx. In pharyngeal phase, the velum elevates to close
the nasopharynx. The hyoid and larynx elevate with anterior moving,
epiglottis descends and vocal cords are closed. The laryngeal elevation
protects the airway and anterior movement of the larynx helps open the
upper esophageal sphincter. The contraction of the pharyngeal muscles
moves the bolus from the pharynx to the esophagus. In esophageal phase,
bolus passes into the stomach by esophagus peristaltism, gravity and LES
relaxation\textsuperscript{53}. The effect of tracheostomy tube on
increasing the risk of aspiration and penetration is defined but there
is no strong evidence. Tracheostomy tube fixes the larynx and prevents
laryngeal elevation. Also it desensitizes larynx and leads an
ineffective cough mechanism, because a positive subglottic pressure
cannot be maintained during swallowing. The cuff of the tracheostomy
tube may impringe on the esophagus during
deglutition\textsuperscript{8,53}. Streppel M et al\textsuperscript{54}.
reported 70\% of the children with tracheostomy had swallowing
disorders. The aspiration rate in the study group was 43\% and half of
them had silent aspiration. The underlying diseases may also contribute
to the development of swallowing disorders\textsuperscript{54}.

The aspiration, penetration and other swallowing problems can be
detected by various diagnostic approaches. Penetration is passage of
material into the laryngeal lumen, but remaining above the vocal cords,
not passing to the glottis. Aspiration is the passage of material below
the vocal cords. The Modified Blue Dye Test is a screening test for
aspiration. After the patient swallow a methylene blue mixed drink or
food, suctioning a colored secretion is considered as aspiration.
Flexible Endoscopic Evaluation of Swallowing (FEES) and Videofluoroscopy
(VFS) are the gold standart methods to evaluate swallowing
dysfunction\textsuperscript{33,53,54}. Pharyngeal phase of swallowing,
penetration and aspiration can be detected by FEES. A speech and
language therapist (SLT) and a pediatric Ear-Nose-Throat (ENT)
specialist can perform FEES. A VFS demonstrate oral, pharyngeal and
esophageal phases of swallowing, penetration, aspiration, obstruction,
fistulas and motility problems can be observed. VFS is usually performed
by a SLT and a radiologist. In the suspicion of an abnormality in the
esophageal phase of swallowing, manometry may be considered. Swallowing
the different consistencies of foods such as liquids, thickened liquids,
purees and solids can be determined \textsuperscript{33,53,54}.

Aspiration is a major problem that complicates the clinical status and
respiratory condition of these children. Aspiration may lead hypoxemia,
cough, respiratory distress or it may be silent. Untreated aspiration
may result in bronchiectasis and decreased lung
function\textsuperscript{33}. On contrary, in recent years many centers
change their feeding strategy to giving heavy-thickened liquids even if
there is aspiration and/or penetration on VFSS. Particularly in patients
with tracheostomy it is considered to be safer due to the chance of
removing aspirates through the tracheostomy tube. Furthermore,
improvement of the swallowing function depending on oral stimulation
over time is possible\textsuperscript{55,56}.

In the first 2-4 months of life, swallowing and sucking are reflexive,
subsequently swallowing becomes more voluntary and it progressively
develops in the first years of life. The development of proper oral
feeding skills is negatively affected in premature infants who had long
term intubation in intensive care units. Oral-motor and sensory feeding
difficulties such as weak or incoordinate suck and swallow, oral
aversion, hypersensitivity to oral stimuli (may be due to repetitive
aspirations), refusal to taste food may occur\textsuperscript{32,53,54}.

The early experience of oral feeding in tube fed children will be
beneficial as follows; facilitates the oral motor skills such as chewing
and swallowing, improves sensory skills such as taste and texture of the
foods, and reduces oral aversion. A team work should be required to
decide and carry out to transit oral feeding in tube-feeding children
with tracheostomy. The team should be consist of an occupational
therapist, speech language pathologist, physical therapist, respiratory
therapist, psychologist, dietitian and clinicians as pediatric
pulmonologist, pediatric ear-nose-throat specialist and pediatric
gastroenterologist\textsuperscript{32,53,54}.

Following the instrumental tests, the patients who have a medical
stability, swallow their oral secretions, need infrequent tracheal
suctioning are seem to be ready to oral feeding. Oral feeding should be
attempted under the actual mode of ventilation with following oxygen
saturation. The aspiration and oral aversion risk may be reduced by
tasting small amounts of food in extreme caution. Coughing, chocking,
increased oxygen demand or worsening pulmonary functions, presence or
suctioning of food or liquid around the stoma or in the tracheostomy
tube, sudden changes in the amount, constituency or color of the
secretions are significant findings of unsuccessful
swallowing\textsuperscript{32,55}.

\textbf{Gastrointestinal hypomotility}

The critically ill positive pressure ventilated patients had decreased
gastric and duedonal contractility. This may be due to Cajal cell (GI
motor activity control unit) dysfunction, pre-existing disease,
endotoxin secretion, corticotropin releasing factor secretion during
stress and drugs\textsuperscript{27,31}.

D\textbf{elayed gastric emptying}

Delayed gastric emptying is a common problem in intensive care units in
the patients with MV. In the absence of mechanical obstruction,
vomiting, bloating feeding intolerance and demonstrating the delayed
emptying with `gastric emptying scintigraphy',
`\textsuperscript{13}C-octanoate acid gastric emptying breath test' or
`acetaminophen absorption test' is sufficient to diagnose.

Supine position, infections, obesity, frequent suctioning, hiatal
hernia, increased intracranial pressure and gastrotomy tube placement
may lead delayed gastric emptying. Also electrolyte disturbances and
hyperglycemia may worsen gastric
emptying\textsuperscript{27,31,57-59}.In tube fed children with
neuromuscular disorders delayed gastric emptying has been reported. The
children with DMA, the delay reported to be related to gastric smooth
muscle involvement\textsuperscript{60,61}. Also delayed gastric emptying
reported in patients with SMA type 1\textsuperscript{62}. In enterally
fed children diagnosed mitochondrial disease gastric delay impairment
was reported\textsuperscript{63}.

Gastric decompression should be performed via nasogastric tube or
gastrostomy tube. If it doesn't resolve a jejunal tube placement or
total parenteral nutrition can be considered to achieve
feeding\textsuperscript{31}. Small volume, frequent, low-fat and low
fiber content feeding may be given in stable
patients\textsuperscript{58}. The gastric emptying is significantly
faster with whey based formula compared to casein based
formula\textsuperscript{64}. The use of prokinetic drugs such as
erythromycin, domperidone, cisapride and metoclopramide is contraversial
because of their serious side effects and conflicting
efficiacy\textsuperscript{58}.

\textbf{Constipation}

Constipation is a common gastrointestinal complication in children with
neuromuscular disorders. The cause of the constipation may be
immobility, inadequate fluid taking, abdominal muscle weakness, smooth
muscle involvements in neuromusculary disease, low fiber diet,
gastrointestinal dysmotility (may be related to underlying disease). A
careful history taking, together with abdominal and rectal examination
will be sufficient to diagnose. Abdominal examination may reveal
fecaloid masses. It is important to evaluate the perianal site for
fissures, skin tags, anal tonus and sensation. Rectal digital
examination should be performed to identify the placement and size of
rectum, and also the consistency and amount of stool. In the suspicious
of the surgical abdomen, abdominal radiography and ultrasound may be
performed. The colonic transit time assessment may be used in treatment
resistance patients. A slow colonic transit time was reported in
patients with demonstrated in the children with brain lesions, DMD and
tube feeding children\textsuperscript{6,15,17,65}.

Simons JP et al\textsuperscript{66}. hypothesed that the Valsalva
maneuver increases in subglottic pressure, but tracheostomy tube may
lead loss of this pressure and may predispose constipation. But they
found no relation with tracheostomy and constipation in their
study\textsuperscript{66}.

Treatment of constipation should be individualized to the patient. If
there is stool in the rectum, previously a fecal impaction should be
performed. Enamas and polyethylene glycol (per oral in dosage of 1.5
g/kg/day) are given for 3 consecutive days and continue till a clear and
liquid defecation. The fecal impaction treatment may be prolonged to 6
days. In maintenance treatment, polyethylene glycol 0.8 g/kg/day or
lactulose 1-2 ml/kg/day is required. The risk of aspiration pneumonia
due to aspiration of mineral oil and polyethylene glycol should kept in
mind. Tube feeding patients should be reviewed for water intake and
formula content. Fiber content formulas may be preferred in tube feeding
patients\textsuperscript{65}.

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