Ospedale dei Bambini “V. Buzzi” Milano

SC di Chirurgia PediatricaSC di Chirurgia Pediatrica

(Direttore dott. G. Riccipetitoni)(Direttore dott. G. Riccipetitoni)SS di Chirurgia Pediatrica GastroenterologicaSS di Chirurgia Pediatrica Gastroenterologica

(Responsabile dott. L. Maestri)(Responsabile dott. L. Maestri)Servizio di Endoscopia DigestivaServizio di Endoscopia Digestiva

(Referente dott. G. Fava)(Referente dott. G. Fava)

DISFAGIA E RGE: DISFAGIA E RGE:

VALUTAZIONE E TRATTAMENTOVALUTAZIONE E TRATTAMENTO

Congresso intersocietario SIMFERCongresso intersocietario SIMFER--SINPIASINPIA

Bosisio Parini, 8Bosisio Parini, 8--99--10 ottobre 200810 ottobre 2008

Chirurgo pediatra e SIMFER-SINPIA? Che

c’azzecca?

THE NERVOUS SYSTEM AND GASTROINTESTINAL THE NERVOUS SYSTEM AND GASTROINTESTINAL FUNCTIONFUNCTION

M.A. Altaf and M.R. Sood DEV DISABIL RES REV, 2008

GASTROINTESTINAL DISORDERS IN CHILDREN WITH GASTROINTESTINAL DISORDERS IN CHILDREN WITH NEURODEVELOPMENTAL DISABILITIESNEURODEVELOPMENTAL 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 seizuresP.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

Esophagus

LES

Cruraldiaphragm

Pylorus

Stomach

Angle of His

Pharynx

UES

Mechanisms of GER

• Transient LES relaxation

• Intra-abdominal pressure

• Reduced esophageal capacitance

• Gastric compliance

• Delayed gastric emptying

Mechanisms of Esophageal Complications

• Impaired esophageal clearance

• Defective tissue resistance

• Noxious composition of refluxate

Mechanisms of Airway Complications

• 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 injury) has

been recognized for decadesP.B. Sullivan, 2008

ORAL-MOTOR

IMPAIRMENT

GERD

La diagnosiLa diagnosi

=>=> pHmetria esofageapHmetria esofagea

=> pasto baritato=> pasto baritato

=> esofagogastroduodenoscopia=> esofagogastroduodenoscopia

=> Manometria esofagea=> Manometria esofagea

pHmetria esofageapHmetria esofagea

Monitoraggio prolungato del pH esofageoMonitoraggio prolungato del pH esofageo

dimostradimostra

••per quanto tempo vi è acido nell’esofago (IR in %)per quanto tempo vi è acido nell’esofago (IR in %)

••relazione tra i sintomi e l’evento RGErelazione tra i sintomi e l’evento RGE

accessi di tosseaccessi di tosse

FKT

Pasto baritatoPasto baritato

Consente una precisa valutazione della Consente una precisa valutazione della anatomia del tubo digerente (frequenti anatomia del tubo digerente (frequenti le anomalie anatomiche nei bambini le anomalie anatomiche nei bambini con danno neurologico)con danno neurologico)

Pasto baritatoPasto baritato

Consente una valutazione (grossolana) della funzione di Consente una valutazione (grossolana) della funzione di

deglutizione che, tuttavia,viene meglio studiata condeglutizione che, tuttavia,viene meglio studiata con

=> Videofluoroscopia=> Videofluoroscopia

=> Valutazione logopedica=> Valutazione logopedica

=> Valutazione foniatrica=> Valutazione foniatrica

In casi selezionati, la funzione di deglutizione viene studiata dal punto di vista manometrico

Deglutizioni secche Deglutizioni umide

EsofagogastroduodenoscopiaEsofagogastroduodenoscopia

Valuta le condizioni della mucosa esofagea, Valuta le condizioni della mucosa esofagea,

permettendo di diagnosticare la presenza di permettendo di diagnosticare la presenza di

lesioni esofagitiche più o meno gravilesioni esofagitiche più o meno gravi

Terapia medicaTerapia medica

Risultati spesso deludentiRisultati spesso deludenti

=> procinetici (domperidone)=> procinetici (domperidone)

=> antiacidi (idrossido di MG e Al)=> antiacidi (idrossido di MG e Al)

=> acido alginico=> acido alginico

=> antisecretivi (ranitidina, omeprazolo, => antisecretivi (ranitidina, omeprazolo, esomeprazolo)esomeprazolo)

In pediatria va seguito lo schema “step up”“step up”(dal farmaco più maneggevole e con minor effetti collaterali al farmaco meno maneggevole e con più effetti collaterali)

procinetico

PPI

Ac. alginico

antiH2

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 medicaTerapia medica

Farmaci “non gastroenterologici”Farmaci “non gastroenterologici”

=> baclofen=> baclofen

=> ondansetron=> ondansetron

Effect of baclofen on emesis and 24 hour Effect of baclofen on emesis and 24 hour esophageal pH in NI children with GEResophageal pH in NI children with GER

The GABA type B receptor agonistThe GABA type B receptor agonist baclofen baclofen was was recently reported to reduce reflux in adult recently reported to reduce reflux in adult patients with GERD reducing the incidence ofpatients with GERD reducing the incidence oftransient lowertransient lower esophageal sphincter relaxationsesophageal sphincter relaxations

8 NI children with GER was studied.8 NI children with GER was studied. BaclofenBaclofen(0.7(0.7 mg/Kg/die)mg/Kg/die) was administered…in 3 divided was administered…in 3 divided doses 30’ before meals for 7 daysdoses 30’ before meals for 7 days

J Pediatr Gastroenterol Nutr. 2004 Mar; 38(3):317J Pediatr Gastroenterol Nutr. 2004 Mar; 38(3):317

ResultsResults

Emesis decreased in 6/8 patients (75%)Emesis decreased in 6/8 patients (75%)

pH monitoring parameterspH monitoring parameters

N of refluxesN of refluxes 193 vs. 124 193 vs. 124 (39%)(39%) P 0.01P 0.01

RIRI 17 vs. 1117 vs. 11 (40%)(40%) P 0.21P 0.21

N of long acid refluxesN of long acid refluxes 11 vs. 411 vs. 4 (56%)(56%) P 0.02P 0.02

T of longest acid refluxT of longest acid reflux 41’ vs. 27’41’ vs. 27’ (37%)(37%) P 0.13P 0.13

In conclusions: 1 week baclofen therapy…had significant effects In conclusions: 1 week baclofen therapy…had significant effects on GERD in NI childrenon GERD in NI children

J Pediatr Gastroenterol Nutr. 2004 Mar; 38(3):317J Pediatr Gastroenterol Nutr. 2004 Mar; 38(3):317

Ondansetron: a review of its use as an antiemetic in Ondansetron: a review of its use as an antiemetic in childrenchildren

Ondansetron is a selective serotonin 5Ondansetron is a selective serotonin 5--HT3 receptor HT3 receptor antagonist.antagonist.

In dose ranging and large placeboIn dose ranging and large placebo--controlled trials, controlled trials, intravenous (0.075 to 0.15 mg/Kg) orintravenous (0.075 to 0.15 mg/Kg) or oral (0.1oral (0.1 mg/kg)mg/kg)ondansetron was significantly more effective than ondansetron was significantly more effective than placebo in preventing emesis in children undergoing placebo in preventing emesis in children undergoing surgery associated with a high risk of postop. nausea surgery associated with a high risk of postop. nausea and vomitingand vomiting

Paediatr Drugs 2001; 3(6): 441Paediatr Drugs 2001; 3(6): 441

DA PEDGIDA PEDGI

For this very difficult population, I have noticed For this very difficult population, I have noticed that often Baclofen or Tranzene or some other that often Baclofen or Tranzene or some other med prescribed by neurologist work better for med prescribed by neurologist work better for vomiting or reflux than “our” medicines do (H2 vomiting or reflux than “our” medicines do (H2 blockers and PPIs)blockers and PPIs)

I use ondansetron 4 mg od I use ondansetron 4 mg od –– bidbid

Adrian Jones, Adrian Jones, Professor of PediatricsProfessor of Pediatrics

Edmonton, Alberta Edmonton, Alberta

CanadaCanada

Trattamenti “operativi“Trattamenti “operativi“NO medicina, NO chirurgiaNO medicina, NO chirurgia

sondino ng sondino ng

sondino transpiloricosondino transpilorico

Trattamenti “operativi“Trattamenti “operativi“NO medicina, NO chirurgiaNO medicina, NO chirurgia

Gastrostomia (chirurgica, percutanea Gastrostomia (chirurgica, percutanea endoscopica, laparoscopica)endoscopica, laparoscopica)

Digiunostomia (chirurgica, percutanea Digiunostomia (chirurgica, percutanea endoscopica, laparoscopica)endoscopica, laparoscopica)

GastrostomiaGastrostomia

ObiettiviObiettivi

••migliorare lo stato nutrizionalemigliorare lo stato nutrizionale

••evitare un riempimento eccessivo dello evitare un riempimento eccessivo dello stomacostomaco

VIA GASTRICA

pallone

VANTAGGI DELLA GASTROSTOMIAVANTAGGI DELLA GASTROSTOMIA

Miglioramento dello stato nutrizionaleMiglioramento dello stato nutrizionale

Somministrazione sicura di farmaci e di altri Somministrazione sicura di farmaci e di altri trattamenti (stipsi)trattamenti (stipsi)

Impact of nutritional rehabilitation on Impact of nutritional rehabilitation on GER in neurological impaired childrenGER in neurological impaired children

10 malnourished NI children10 malnourished NI children (triceps skin fold thickness (triceps skin fold thickness [TSF] below the 5th percentile) treated with aggressive [TSF] below the 5th percentile) treated with aggressive nutritional rehabilitationnutritional rehabilitation,, fed through a PEG.fed through a PEG.

When TSF was > or = 50th percentile the 24When TSF was > or = 50th percentile the 24--hour pH probe hour pH probe study showed marked improvement in 6/10 patientsstudy showed marked improvement in 6/10 patients

J Pediatr Surg 1994 Feb; 29(2):167J Pediatr Surg 1994 Feb; 29(2):167

VANTAGGI DELLA GASTROSTOMIAVANTAGGI DELLA GASTROSTOMIA

Miglioramento dello stato nutrizionaleMiglioramento dello stato nutrizionale

Somministrazione sicura di farmaci e di altri Somministrazione sicura di farmaci e di altri trattamenti (stipsi)trattamenti (stipsi)

Miglioramento della qualità di vita (paziente e Miglioramento della qualità di vita (paziente e care givers)care givers)

This study has quantified for the This study has quantified for the first time a significant, measurable first time a significant, measurable improvement in the quality of life improvement in the quality of life

of carers (mothers predominately) of carers (mothers predominately) after overcoming the feeding after overcoming the feeding

problems of children with CP by problems of children with CP by insertion of gastrostomy tubeinsertion of gastrostomy tube

Dev. Med. And Child Neurol. 2004Dev. Med. And Child Neurol. 2004

GastrostomiaGastrostomia

ProblemaProblema

RGERGE

DigiunostomiaDigiunostomia

ObiettivoObiettivo

“Saltare” lo stomaco“Saltare” lo stomaco

Venting dello stomacoVenting dello stomaco

VIA GASTRICA

pallone

via digiunale

Istituti Clinici di PerfezionamentoOspedale di rilievo nazionale e di alta specializzazione convenzionato con l’Università degli

Studi di MilanoOspedale dei Bambini “V. Buzzi”

U.O di Chirurgia Pediatrica

ambulatorio per la riabilitazione dei bambini ambulatorio per la riabilitazione dei bambini incontinenti e stomizzatiincontinenti e stomizzati

AMBULATORIO DI GASTROENTEROLOGIA CHIRURGICAAMBULATORIO DI GASTROENTEROLOGIA CHIRURGICA

LA STOMIA NUTRIZIONALELA STOMIA NUTRIZIONALEGUIDA PER I GENITORIGUIDA PER I GENITORI

Terapia chirurgicaTerapia chirurgica

Risultati assai deludenti: circa 20% di recidiveRisultati assai deludenti: circa 20% di recidive(5% nei bambini senza danno neurologico)(5% nei bambini senza danno neurologico)

Alta incidenza di complicanzeAlta incidenza di complicanze

Complicanze più frequentiComplicanze più frequenti

“retching”“retching”

impossibiltà di vomitare (gas bloat syndrome)impossibiltà di vomitare (gas bloat syndrome)

occlusione intestinaleocclusione intestinale

The failure rate of surgery for gastroesophageal refluxThe failure rate of surgery for gastroesophageal reflux

The main presentingThe main presenting symptomssymptoms at the time of the diagnosis of at the time of the diagnosis of failure wasfailure was severe retching, recurrent vomiting and aspiration severe retching, recurrent vomiting and aspiration and gas bloat syndrome.and gas bloat syndrome.

The predominant cause of The predominant cause of fundoplication failure is herniationfundoplication failure is herniationinto the posterior mediastinuminto the posterior mediastinum, which occured most frequently , which occured most frequently in children with in children with hypertonic cerebral palsy.hypertonic cerebral palsy.

J J Pediatr Surg. 1998 Jan; 33(1):64Pediatr Surg. 1998 Jan; 33(1):64

GOSGOS

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 combinedhospital study of 7467 patients

Pediatrics, 1998

INDICAZIONI ALLA CHIRURGIAINDICAZIONI 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 sipropongono lo scopo di:

=>ottenere un segmento di esofagoaddominale sufficentemente lungo

=>Fissare meccanicamente lo SEI al disotto del diaframma=>Ricostruire un angolo di His di 0°

Razionale dell’intervento chirurgico

Tutte le tecniche chirurgiche adottate sipropongono lo scopo di:

=>ottenere un segmento di esofagoaddominale sufficentemente lungo

=>Fissare meccanicamente lo SEI al disotto 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 aresimilar to those of the openprocedure, but hospitalization isshortened

NASPGHAN

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

RISULATINissen: 25%

Floppy Nissen-Rossetti: 63%

Toupet: 1,7%

Fundoplicatio anteriori: 10%

Complicanze i.o.: 1 caso di perforazione esofagea;1 caso di perforazione gastrica1 caso di apertura della pleura

Reintervento: 3,8% (stenosi; RGE recidivo

Totale: nTotale: n°° 252 pazienti252 pazientiEtà media: 4,8 anni (3 m Età media: 4,8 anni (3 m –– 14 aa)14 aa)

Sintomi: esofagite (63%(; asma (18%); Sintomi: esofagite (63%(; asma (18%); infezioni respiratorie (28%); infezioni respiratorie (28%);

Ernia iatale: 29%; Ernia iatale: 29%;

Patologie associate: 35%;Patologie associate: 35%;

Pz cerebropatici: 25%;Pz cerebropatici: 25%;

Pz operati per AE: 4%Pz operati per AE: 4%

PROPOSTAPROPOSTA

Trattamento medico “gastroenterologico”Trattamento medico “gastroenterologico”

Trattamento medico “non gastroenterologico”Trattamento medico “non gastroenterologico”

PEGPEG

PEJPEJ

LapLap--fundo+lapegfundo+lapeg

Grazie per l’attenzione!

l.maestri@icp.mi.it