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Enfermedad de

Hirschprung
o
Megacolon Congenito
5to C

Que es?????
Consiste en una ausencia de
clulas
Ganglionares
Ganglios nerviosos
En la pared
Muscular
Submucosa

Porque??
Detencin en la migracin
caudal de las clulas
procedentes de la cresta neural
antes de llegar al ano
Desarrollo embrionario

Incidencia
1 de cada 5,000
70% a 80%

Masculinos

Menos frecuente en la
raza negra
Antecedente +
Mayor riesgo de
padecerla

Malformaciones
agregadas

25% de los casos con


antecedes familiares
10% no familiar (sin
antecedentes)

Factores Genticos
Se descubri una paciente
femenina con aganglionosis
total del colon
Sindrome de Zuelzer-Wilson

portaba una delecin en el


cromosoma
10:46,XX de q110.11.21-q21.2

Tres genes

1.

2.

Identificados en el
hombre
Gen de RET

Cromosoma 10

Gen EDNRB

Gen del receptor de la


endotelina B

3.

Autosmicos
dominante

Cromosoma 13

Autosmicos Recesivo

Gen EDN3

Gen de endotelina 3

Cromosoma 20

Autosmicos recesivo

Fisiopatologa
Intestino
Falta de
propagacin de las
ondas de propulsin

Esfnter anal
interno
Relajacin anormal
Ausencia de la
relajacin

Fisiopatologa

Intestino

Esfnter anal Interno

Falta de ondas
peristalticas

Relajacin anormal
O ausencia de la
relajacin

Aganlionosis
Hipoganglionosis
Disganglionosi

Cuadro Clinico

3 Puntos Cardinales
1. Ausencia de evacuacion en las
primeras 24 hrs de vida
2. Distension Abdominal
3. Vomito

Examen Rectal
Con sonda rectal,
termometro o
lavados
Induce a la salida
explosiva de heces
liquidas y gas
sospechoso a
enterocolitis

Se nota hipertona
del esfnter rectal y
casi siempre esta
vaco

Se ha visto que la
ENTEROCOLITIS se presenta en
un 12% a 58% de los pacientes
con megacolon congnito

Enterocolitis

Hipotesis Etiologicas

1.

Estasis fecal

2.

Alteracion de las de
la composicion de la
mucina y
mecanismos de
defensa de la
mucosa
Aumento de la
actividad de la
prostaglandina E1 y
la infeccion por
Clostridium difficile

3.

Isquemia en mucosa
Invasion y
traslocacion de
bacteriana.

Forma grave de
enterocolitis

Megacolon toxico

Caracterizado por:
1.
2.
3.
4.
5.
6.

Fiebre
Vomito teido de bilis
Diarrea explosiva
Distensin abdominal
Deshidratacin
Choque

Diagnostico
Radiologico

Radiografia
abdominal
Posicion Supina
y verticla

Muestran niveles
Hidroaereos en
colon

Hallazgo tipico

Imagen transoperatoria

Ecografia que muestra


EH ultra corta

EH con segmento largo


sndrome de ZuelzerWilson

Electromanometria
Rectal
Precisin diagnostica
85%

Normalmente

Se produce relajacion
del esfnter anal
interno

EH

Muestran cambios
caractersticos
durante el estimulo
En la presin del
conducto anal
Parte inferior del
recto

Biopsia Rectal
Tincin de hematoxilina y eosina

Se toman
muestras de
2, 3, 5 cm

Muestra optima
3.5 cm de
diametro
Incluye
submucosa

Examen histoquimico
Celulas ganglionares gigantes y heterotopicas en el
plexo submucoso, en un paciente con displasia
neuronal intestinal

Tratamiento

Descompresin
1. Instalacin de una sonda nasogastrica.
2. Vaciamiento repetido del recto con
sonda rectales e irrigaciones.

Despus de hacer todos los mtodos


diagnsticos
Se procede a establecer un estoma, si es
necesario.

Tratamiento

Colostoma

Antes de realizarla
es necesario
1. Lavado
intestinal
2. Administracion
de antibioticos
30 minutos
antes de la
operacin
3. Instalacin de
una sonda
ureteral

Procedimientos
Definitivos
Tcnica de
Swenson

Tcnica

For Swensons pull-through operation the


patient is positioned on the operating
table to provide simultaneous exposure
of the perineum and abdomen. The pelvis
is allowed to drop back over the lower
end of the table and the legs are
strapped over sandbags. A Foley catheter
is inserted into the bladder. The abdomen
is opened via a paramedian incision.
Some surgeons prefer a Pfannenstiel
incision when performing a Swensons
pull-through operation in the neonate.
Extramucosal biopsies are taken at
intervals along the antimesenteric border
and assessed by frozensection to
determine the level of
ganglionatedbowel. The sigmoid colon is
mobilized by dividingthe sigmoid vessels
and retaining the marginalvessels.It may
be necessary to mobilize the splenic
flexureto obtain adequate length. The
proximal level ofresection above the
ganglionated level, previouslydetermined
by frozen section, is selected and the
bowel is divided between intestinal
clamps or staples. The peritoneum is
divided around its lateral andanterior
reflection from the rectum, exposing
themuscle coat of the rectum.At this
point, the bowel isdivided at the
rectosigmoid junction and
removed.Dissection extends around the
rectum, keeping veryclose to the bowel
wall. It is essential to maintain
thedissection close to the muscular wall
in order to preventdamage to the pelvic
splanchnic innervation.All

vessels are electro-coagulated under


direct vision. Sufficient tension-free
length is obtained by dividing the
inferior mesenteric pedicle, carefully
preserving the marginal vessels.
Dissection is carried down to the level
of the external sphincter posteriorly
and laterally, but does not extend as
deeply anteriorly,
leaving around 1.5 cm of intact rectal
wall abutting against the vagina or
urethra. The mobilized rectum is
intussuscepted through the anus by
passing a curved clamp or a Babcock
forceps through the anal canal; an
assistant places the closed rectal
stump within the jaws of the clamp.
When the dissection has been
completed, it should
be possible to evert the anal canal
completely when traction is applied to
the rectum.An incision is made
anteriorly through the rectal wall about
1 cm from the dentate line, extending
halfway through the rectal
circumference.A clamp is inserted
through this incision to grasp multiple
sutures placed through the cut end of
the proximal colon. An outer layer of
interrupted 4-0 absorbable sutures is
placed through the cut muscular edge
of the rectum and the muscular
wall of the pull-through
colon.When the outer layer has been
completed, the proximal bowel is
opened and an inner layer of
interrupted 4-0 absorbable
suturesis placed.When anastomosis is
completed, the sutures are cut,
allowing the anastomosis to
retractwithin the anus.

Procedimientos
definitivo
Tcnica de
Duhamel-Grob

Tecnica
The advantage of the Duhamel pull-through is that very little
manipulation of the rectum is performed anteriorly thus avoiding
injury to the genitourinary innervation. The rectum is divided
and closed just above the peritoneal reflection. The redundant
aganglionic bowel is resected. The retrorectal space is created
by blunt dissection down to the pelvic floor. The posterior rectal
wall is incised 1.5 to 2 cm above the dentate line and sponge
holding forceps is inserted into the retrorectal space and
ganglionic bowel pulled through. The anterior half of the
pulledthrough ganglionic bowel is anastomosed to the posterior
wall of the aganglionic rectum and remainder of the colo-rectal
anastomosis completed by approximating the aganglionic
rectum to the posterior wall of the pulled-through ganglionic
bowel. Finally an extra long automatic stapling device is used to
complete the side to side anastomosis between the aganglionic
rectum and the ganglionic pulled-through bowel. Some surgeons
complete the side to side anastomosis prior to closing the rectal
stump, thereby preventing any residual septum.

Procedimientos
definitivos
Descenso
endorectal

Tcnica
In Soave or endorectal pull-through the first steps of
the operation are similar to those described for
Swensons or Duhamel operation. The colon is
mobilized and resected about 4 cm above the
peritoneal reflection. The endorectal dissection
begins 2 cm below the peritoneal reflection. The
seromuscular layer is incised circumferentially and
the mucosal-submucosal tube is freed distally. The
mucosal dissection iscontinued distally to the level
of the dentate line. Themucosa is incised
circumferentially 1 cm above thedentate line. A
Kelly clamp is inserted from belowand the
ganglionic bowel is pulled
through.Coloanalanastomosis is completed using
4/0 absorbable sutures.

Procedimientos
definitivos
Reseccin
anterior segn
Rehbein

Tcnica
Rehbeins technique differs from
the Swensons procedure,in that
the anastomosis is a low,
anterior colorectal
anastomosis. In this procedure,
3 to 5 cm of the terminal
aganglionic rectum is left
behind,which is anastomosed to
the ganglionic bowel.

Bibliografa
CIRUGIA PEDIATRIA
ASCHRAFT Murphy, Sharp,
Sigalet, Snyder
SPRINGER SURGERY ATLAS
SERIES Series Editors: J. S. P.
Lumley J. R. Siewert