L. Maestri

Ospedale dei Bambini “V. Buzzi”
Milano
SC di Chirurgia Pediatrica
(Direttore dott. G. Riccipetitoni)
SS di Chirurgia Pediatrica Gastroenterologica
(Responsabile dott. L. Maestri)
Servizio di Endoscopia Digestiva
(Referente dott. G. Fava)
DISFAGIA E RGE:
VALUTAZIONE E TRATTAMENTO
Congresso intersocietario SIMFERSIMFER-SINPIA
Bosisio Parini, 88-9-10 ottobre 2008
Chirurgo pediatra e
SIMFER-SINPIA? Che
c’azzecca?
THE NERVOUS SYSTEM AND GASTROINTESTINAL
FUNCTION
M.A. Altaf and M.R. Sood DEV DISABIL RES REV, 2008
GASTROINTESTINAL DISORDERS IN CHILDREN WITH
NEURODEVELOPMENTAL DISABILITIES
P.B. Sullivan DEV DISABIL RES REV, 2008
ORAL- MOTOR IMPAIRMENT
Symptoms from oral-motor impairment include feeding
problems,
drooling
and
gagging
as
well
as
dysarthria. Uncoordinated swallowing increases the
rysk of pulmonary aspiration, which may or may not
heralded by recurrent coughing and choking with
feeds.
P.B. Sullivan, 2008
ORAL- MOTOR IMPAIRMENT
Feeding problems can easily lead to malnutrition in children with CP
and seriously adversely effect the quality of life of theirs mothers.
Those children with the severest general motor deficit are also
those with the most severe degree of oral-motor impairment. This
is because development of oral-motor skills mirrors general
neurological maturation.
P.B. Sullivan, 2008
ORAL- MOTOR IMPAIRMENT
This development requires the coordination of the movement of a
total of 31 pairs of striated muscles in the mouth, pharyng and
oesophagus by six cranial nerves, the brain stem and the
cerebral cortex. When the central control of this mass of
musculature is severly impaired there is little chance of getting
sufficient quantities of food and drink safely into the
esophagus
P.B. Sullivan, 2008
GER
The high incidence of GER (15-75%) in neurologically
impaired children is well recognized. Several reasons have
been proposed to account for this high incidence…including
hiatus hernia, adoption of a prolonged supine position,
and increased intrabdominal pressure secondary to
spasticity, scoliosis or seizures
P.B. Sullivan, 2008
GER
Nevertheless, CNS dysfunction is likely to be the prime cause
of GER. As a result of neuromuscolar incoordination, the
antireflux function of LES mechanism and esophageal
motility are significantly impaired.
Thus, food and acid and pepsin reflux rostrally from the
stomach into the esophagus
P.B. Sullivan, 2008
Mechanisms of GER
Pharynx
•
Transient LES relaxation
•
Intra-abdominal pressure
•
Reduced esophageal capacitance
•
Gastric compliance
•
Delayed gastric emptying
UES
Esophagus
Mechanisms of Esophageal Complications
Crural
diaphragm
Pylorus
Angle
of His
•
Impaired esophageal clearance
•
Defective tissue resistance
•
Noxious composition of refluxate
LES
Mechanisms of Airway Complications
Stomach
•
Vagal reflexes
•
Impaired airway protection
GERD
GER is a pathological process and GERD refers to the symptom
complex that arises as a result of that process.
It is ironic that the learning deficit that usually accompanies
neurological impairement also impairs the ability of the affected
individual to communicate the main symptom of GERD which is
pain
P.B. Sullivan, 2008
GERD
Chronic
peptic
esophagitis=>mucosal
ulceration=>stricture
formation
Pain=>irritability and crying=>distonic movement of face and neck
Chronic dysphagia=>behavioral food avoidance or aversion
Vomiting, hematemesis, anemia, rumination and regurgitation
Dental problem
P.B. Sullivan, 2008
RESPIRATORY
CONSEGUENCE OF GERD
The association between GERD
and respiratory complications
(apnea,
laryngitis,
asthma/wheezing, chronic cogh,
chronic
pulmonary
aspiration,
recurrent
pneumonia
and
progressive
lung
has
injury)
been recognized for decades
P.B. Sullivan, 2008
ORALMOTOR
IMPAIRMENT
GERD
La diagnosi
=> pHmetria esofagea
=> pasto baritato
=> esofagogastroduodenoscopia
=> Manometria esofagea
pHmetria esofagea
Monitoraggio prolungato del pH esofageo
dimostra
•per quanto tempo vi è acido nell’esofago (IR in %)
•relazione tra i sintomi e l’evento RGE
accessi di tosse
FKT
Pasto baritato
Consente una precisa valutazione della
anatomia del tubo digerente (frequenti
le anomalie anatomiche nei bambini
con danno neurologico)
Pasto baritato
Consente una valutazione (grossolana) della funzione di
deglutizione che, tuttavia,viene meglio studiata con
=> Videofluoroscopia
=> Valutazione logopedica
=> Valutazione foniatrica
In casi selezionati, la funzione di deglutizione viene studiata dal
punto di vista manometrico
Deglutizioni secche
Deglutizioni umide
Esofagogastroduodenoscopia
Valuta le condizioni della mucosa esofagea,
permettendo di diagnosticare la presenza di
lesioni esofagitiche più o meno gravi
Terapia medica
Risultati spesso deludenti
=> procinetici (domperidone)
=> antiacidi (idrossido di MG e Al)
=> acido alginico
=> antisecretivi (ranitidina, omeprazolo,
esomeprazolo)
In pediatria va seguito lo schema “step up”
(dal farmaco più maneggevole e con minor effetti collaterali al
farmaco meno maneggevole e con più effetti collaterali)
PPI
antiH2
Ac. alginico
procinetico
Domperidone: 0.2 mg/Kg/dose 4 volte/die, 20’ prima dei pasti
Ac. Alginico: 0.5 ml/Kg/dose 4 volte al giorno. 20’ dopo i pasti
Ranitidina: 10 mg/Kg in 2 dosi al giorno
PPI: 1-2 mg/Kg al giorno alla mattina
Terapia medica
Farmaci “non gastroenterologici”
=> baclofen
=> ondansetron
Effect of baclofen on emesis and 24 hour
esophageal pH in NI children with GER
The GABA type B receptor agonist baclofen was
recently reported to reduce reflux in adult
patients with GERD reducing the incidence of
transient lower esophageal sphincter relaxations
8 NI children with GER was studied. Baclofen
(0.7 mg/Kg/die) was administered…in 3 divided
doses 30’ before meals for 7 days
J Pediatr Gastroenterol Nutr. 2004 Mar; 38(3):317
Results
Emesis decreased in 6/8 patients (75%)
pH monitoring parameters
¾N of refluxes
193 vs. 124
(39%)
P 0.01
¾RI
17 vs. 11
(40%)
P 0.21
¾N of long acid refluxes
11 vs.
4
(56%)
P 0.02
¾T of longest acid reflux
41’ vs. 27’
(37%)
P 0.13
In conclusions: 1 week baclofen therapy…had significant effects
on GERD in NI children
J Pediatr Gastroenterol Nutr. 2004 Mar; 38(3):317
Ondansetron: a review of its use as an antiemetic in
children
Ondansetron is a selective serotonin 55-HT3 receptor
antagonist.
In dose ranging and large placeboplacebo-controlled trials,
intravenous (0.075 to 0.15 mg/Kg) or oral (0.1 mg/kg)
ondansetron was significantly more effective than
placebo in preventing emesis in children undergoing
surgery associated with a high risk of postop. nausea
and vomiting
Paediatr Drugs 2001; 3(6): 441
DA PEDGI
For this very difficult population, I have noticed
that often Baclofen or Tranzene or some other
med prescribed by neurologist work better for
vomiting or reflux than “our” medicines do (H2
blockers and PPIs)
I use ondansetron 4 mg od – bid
Adrian Jones, Professor of Pediatrics
Edmonton, Alberta
Canada
Trattamenti “operativi“
NO medicina, NO chirurgia
¾sondino ng
¾sondino transpilorico
Trattamenti “operativi“
NO medicina, NO chirurgia
¾Gastrostomia (chirurgica, percutanea
endoscopica, laparoscopica)
¾Digiunostomia (chirurgica, percutanea
endoscopica, laparoscopica)
Gastrostomia
Obiettivi
•migliorare lo stato nutrizionale
•evitare un riempimento eccessivo dello
stomaco
VIA GASTRICA
pallone
VANTAGGI DELLA GASTROSTOMIA
Miglioramento dello stato nutrizionale
Somministrazione sicura di farmaci e di altri
trattamenti (stipsi)
Impact of nutritional rehabilitation on
GER in neurological impaired children
10 malnourished NI children (triceps skin fold thickness
[TSF] below the 5th percentile) treated with aggressive
nutritional rehabilitation,
rehabilitation, fed through a PEG.
When TSF was > or = 50th percentile the 24
24--hour pH probe
study showed marked improvement in 6/10 patients
J Pediatr Surg 1994 Feb; 29(2):167
VANTAGGI DELLA GASTROSTOMIA
Miglioramento dello stato nutrizionale
Somministrazione sicura di farmaci e di altri
trattamenti (stipsi)
Miglioramento della qualità di vita (paziente e
care givers)
This study has quantified for the
first time a significant, measurable
improvement in the quality of life
of carers (mothers predominately)
after overcoming the feeding
problems of children with CP by
insertion of gastrostomy tube
Dev. Med. And Child Neurol. 2004
Gastrostomia
Problema
RGE
Digiunostomia
Obiettivo
“Saltare” lo stomaco
Venting dello stomaco
VIA GASTRICA
pallone
via digiunale
Istituti Clinici di Perfezionamento
Ospedale di rilievo nazionale e di alta specializzazione convenzionato con l’Università degli
Studi di Milano
Ospedale dei Bambini “V. Buzzi”
U.O di Chirurgia Pediatrica
ambulatorio per la riabilitazione dei bambini
incontinenti e stomizzati
AMBULATORIO DI GASTROENTEROLOGIA CHIRURGICA
LA STOMIA NUTRIZIONALE
GUIDA PER I GENITORI
Terapia chirurgica
Risultati assai deludenti: circa 20% di recidive
(5% nei bambini senza danno neurologico)
Alta incidenza di complicanze
Complicanze più frequenti
™“retching”
™ impossibiltà di vomitare (gas bloat syndrome)
™ occlusione intestinale
The failure rate of surgery for gastroesophageal reflux
The main presenting symptoms at the time of the diagnosis of
failure was severe retching, recurrent vomiting and aspiration
and gas bloat syndrome.
The predominant cause of fundoplication failure is herniation
into the posterior mediastinum
mediastinum,, which occured most frequently
in children with hypertonic cerebral palsy.
J Pediatr Surg. 1998 Jan; 33(1):64
GOS
National trends in the use of
antireflux procedures for children
…retrospective cohort study of children
undergoing antireflux surgery in US
from 1996 to 2003…
During the study period, 48665 antireflux
procedures were performed…
Pediatrics, 2006
National trends in the use of
antireflux procedures for children
There was a significant decrease in the
percentages of children undergoing
antireflux procedures who were
neurologically impaired between 1996 and
2003
Pediatrics, 2006
SCOMPARSA O MIGLIORAMENTO SIGNIFICATIVO DEI SINTOMI
(giudizio solo clinico)
94% =>84.6%
(NON NEUROLOGICI/ NEUROLOGICI)
COMPLICANZE
4.2% =>12.8%
(NON NEUROLOGICI/NEUROLOGICI)
REINTERVENTI
(REDO FUNDO O OCCLUSIONE)
3.6% =>11.8%
(NON NEUROLOGICI/NEUROLOGICI)
Surgical treatment of gastroesophageal reflux in children: a combined
hospital study of 7467 patients
Pediatrics, 1998
INDICAZIONI ALLA CHIRURGIA
=>mancato controllo dei sintomi dopo PEG/PEGJ
=>gravi alterazioni anatomiche della regione esofago-gastrica
Razionale dell’intervento chirurgico
Tutte le tecniche chirurgiche adottate si
propongono lo scopo di:
=>ottenere
un segmento di esofago
addominale sufficentemente lungo
=>Fissare meccanicamente lo SEI al di
sotto del diaframma
=>Ricostruire un angolo di His di 0°
Razionale dell’intervento chirurgico
Tutte le tecniche chirurgiche adottate si
propongono lo scopo di:
=>ottenere
un segmento di esofago
addominale sufficentemente lungo
=>Fissare meccanicamente lo SEI al di
sotto del diaframma
=>Ricostruire un angolo di His di 0°
Scelta della tecnica
=>Opzione di Scuola
Risultati della chirurgia
The results of
pediatric
series of
laparoscopic fundoplication suggest that
the results and complications rates are
similar
to
procedure,
those
but
of
the
open
hospitalization
shortened
NASPGHAN
is
Indications must remain identical
for open and laparoscopic
procedures
Journal of Pediatric Surgery Lecture
Juan A. Tovar
Journal of Pediatric Surgery 42, 277, 2007
The laparoscopic Nissen and LAPEG are realizable also in
neurologically impaired children using personalized solutions
adapted to the patient’s deformities and spasticity. The
choice to perform an initially PEG, indicated in cases of
severe malnutrition or moderate GER, doesn’t represent a
formal indication against the laparoscopic fundoplicatio;
SIGENP, 2007
RISULATI
Totale: n°
n° 252 pazienti
9
Nissen: 25%
9
Età media: 4,8 anni (3 m – 14 aa)
9
Floppy Nissen-Rossetti: 63%
9
Sintomi: esofagite (63%(; asma (18%);
infezioni respiratorie (28%);
9
Toupet: 1,7%
9
Fundoplicatio anteriori: 10%
9
Ernia iatale: 29%;
9
9
Patologie associate: 35%;
9
Pz cerebropatici: 25%;
9
Pz operati per AE: 4%
Complicanze i.o.:
1 caso di perforazione esofagea;
1 caso di perforazione gastrica
1 caso di apertura della pleura
Reintervento: 3,8% (stenosi; RGE
recidivo
9
PROPOSTA
Trattamento medico “gastroenterologico”
Trattamento medico “non gastroenterologico”
PEG
PEJ
Lap--fundo+lapeg
Lap
Grazie per l’attenzione!
[email protected]