Esophageal
Dysmotility
presented by Dr Shahzad Iqbal
DR. SHAHZAD IQBAL
PGR PAEDS MEDICINE
NISHTER HOSPITAL MULTAN
By the end of this presentation, participants should
be able to:
Learning
Objective 01 Define Esophageal
dysmotiliy
s Classify Esophageal Motility
02 disorders
Identify common motility
03 disoders
Outline the diagnostic
04 approach
Formulate a
05 Management plan
06 Apply knowledge to
clinical Scenarios
01 CASE
SCENARIO
A 13 Year old boy presented to OPD with a h/o
intermittent vomiting and dysphagia since the age
of 3 months. Clinically, he was cachectic with no
syndromic features. On oesophagogastroscopy, the
oesophagus was markedly dilated, with food
residue and a spastic and stiff lower oesophageal
sphincter (LES). No fibrotic rings were identified
and there were no signs of oesophagitis. The
opening was <3 mm and it was not possible to pass
the scope beyond the stiff LES; hence, manometric
studies were not done. A subsequent barium
swallow study (Fig) demonstrated a mega-
oesophagus, with irregular and abnormal
peristalsis in the proximal esophagus. The
oesophagus tapered and narrowed at the
gastroesophageal junction. No aspiration changes
noted in the lung fields.
1) What is the possible diagnosis in this case?
2) What are the management options you can
Esophageal
Dysmotility
Esophageal dysmotility or
Esophageal motility disorders
(EMDS) involve abnormal
movement of the esophagus,
affecting the passage of food from
the pharynx to the stomach.
EMDs, though less common in
children, can cause significant
morbidity, including feeding
difficulties, growth impairment,
and respiratory complications.
Classification
Classification based on Location
UPPER ESOPHAGEAL MOTILITY LOWER ESOPHAGEAL MOTILITY
DISORDERS (STRIATED MUSCLE) DISORDERS (SMOOTH MUSCLE)
Cricopharyngeal Achalasia Achalasia
Diffuse Esophageal spasm
Cricopharyngeal incoordination(self Nutcracker Esophagus
limited) GERD
Zenker’s Diverticulum Hypertensive Lower Esophageal
sphincter(LES)
Systemic diseases (SECONDARY CAUSES) SECONDARY CAUSES
Upper Esophageal
0 Motility Disorders
1
01 Upper Esophageal & Upper Esophageal Sphincter(UES)
Disorders
Cricopharynge Systemic
Cricopharyngeal Zenker’s
al diorders that
Achalasia Diverticulum
Incoordination can affect UES
• Primary crycopharyngeal A self-limited Cricopharyngeal spasm • cerebral palsy,
achalasia consists of a failure • Arnold-Chiari
form of may be severe enough
of the crycopharyngeus to produce posterior malformation
muscle to relax at the
cricopharyngeal • syringomyelia,
incoordination pharyngeal (Zenker )
appropriate time during the diverticulum above the • bulbar palsy or cranial
swallowing process in the occurs in infancy obstructive sphincter; nerve defects (transient
absence of other motor and remits this entity occurs rarely infantile paralysis of the
abnormalities. spontaneously in in children superior laryngeal nerve),
• A rare condition, in which a • transient pharyngeal
the 1st yr of life
newborn presents with May present with • muscle dysfunction,
dysphagia, choking,
if • spinal muscular atrophy
nutrition is Dysphagia, Bad breath,
salivation and nasal reflux on Chronic cough, • muscular dystrophy,
feeding . maintained Sensation of “a lump in • multiple sclerosis,
• Detected on despite the the throat” • infections (botulism,
videofluoroscopic dysphagia. Regurgitation of food tetanus,poliomyelitis,
• evaluation of swallowing or (often hours after a diphtheria),
by Manometry. meal) • inflammatory and
• Can be treated with dilation, autoimmune diseases
Botox injection, and • familial dysautonomia
Cricopharyngeal Achalasia Zenker’s Diverticulum
Lower
0 Esophageal
2 Motility
Disorders
ACHALASIA
Achalasia is a primary esophageal motor
disorder of unknown etiology characterized by
loss of LES relaxation and loss of esophageal
peristalsis, both contributing to a functional
obstruction of the distal esophagus.
Degenerative, autoimmune (antibodies to
Auerbach plexus), and infectious (Chagas
disease caused by Trypanosoma cruzi ) factors
are possible causes.
ALLGROVE SYNDROME
In rare cases, achalasia is familial or part of the achalasia, alacrima, and adrenal
insufficiency, known as triple A syndrome or Allgrove syndrome .
PSEUDOACHALASIA
Pseudoachalasia refers to achalasia caused by various forms of cancer via
obstruction of the gastroesophageal junction, infiltration of the submucosa and
muscularis of the LES, or as part of the paraneoplastic syndrome with formation
of anti-Hu antibodies.
In achalasia, inflammation surrounds ganglion cells,
which are decreased in number. There is selective loss of postganglionic
inhibitory neurons that normally lead to sphincter relaxation, leaving
postganglionic cholinergic neurons unopposed. This imbalance produces high
basal LES pressures and insufficient LES relaxation. The loss of esophageal
peristalsis can be a secondary phenomenon.
There’s no opposition by Loss of inhibitory
inhibitory neurons which neurons results in loss
results in overactivity of of sphincter relaxation
excitatory neurons
resulting in increased tone
Clinical Features
• The mean age inchildren is 8.8 yr, with a mean duration of symptoms
before diagnosis of 23 mo; it is uncommon before school age
• Achalasia manifests with regurgitation and dysphagia for solids and
liquids and may be accompanied by undernutrition or chronic cough;
• Retained esophageal food can produce esophagitis.
• The presentations of chronic regurgitation/vomiting with weight loss,
and chronic cough have led to misdiagnoses of anorexia nervosa and
asthma, respectively.
Investigations
• Chest radiograph shows an air–fluid level in a dilated esophagus.
• Barium fluoroscopy reveals a smooth tapering of the lower
esophagus leading to the closed LES, resembling a bird's beak.
• Manometry is the most sensitive diagnostic test and helps
differentiate the three types of achalasia; it reveals the defining
features of aperistalsis in the distal esophageal body and
incomplete or absent LES relaxation, often accompanied by high-
pressure LES and low-amplitude esophageal body contractions
Management
The goals of achalasia therapy are
• relief of symptoms,
• improvement of esophageal emptying, and
• prevention of megaesophagus
Treatment Options
1. Medical
2. Botulinum Toxin Injection
3. Pneumatic Dilatation
4. Myotomy (Surgical/ Laparoscopic/ Endoscopic)
1) Medical:
Calcium channel blockers (nifedipine) and phosphodiesterase inhibitors offer
temporary relief of dysphagia
2)Pneumatic dilation:
Pneumatic dilation is the initial treatment of choice and does not
preclude a future myotomy
3)Myotomy: Three types of Myotomy:
Surgical: Surgeons often supplement a myotomy with an antireflux procedure to prevent
the gastroesophageal reflux disease.
Laparoscopic: Laparascopic myotomy is a particularly effective procedure in adolescent
and young adult males.
Endoscopic: Peroral endoscopic
myotomy (POEM) may be a
feasible, safe, and an
effective alternative to the
laparoscopic method
4)Botulinum Toxin Injection
• Endoscopic injection of the LES with botulinum toxin
inhibits the release of acetylcholine from nerve terminals.
• Botulinum toxin is effective in 50–65% of patients and
is expensive;
• half the patients might require a repeat injection
within 1 yr. Most eventually require dilation or
surgery.
Diffuse
Esophageal
Spasm
Diffuse esophageal spasm causes chest
pain and dysphagia and affects
adolescents and adults.
It is diagnosed manometrically and
can be treated with nitrates or calcium-
channel-blocking agents
Nutcracker
Esophagus
Nutcracker esophagus, jackhammer
esophagus, or hypercontractile peristalsis,
is a disorder of the movement of the
esophagus characterized by contractions in
the smooth muscle of the esophagus in a
normal sequence but at an excessive
amplitude or duration.
It is extremely rare in childrean and can
present with dysphagia and chest pain.
Diagnosed via Manometry (pressure
>180mmHg)
Approach to a Child
with Esophageal
Dysphagia
THANK YOU!
ANY QUESTIONS?
