Congenital anomalies of esophagus

Congenital anomalies of esophagus-Tracheoesophageal fistula, Esophageal atresia, esophageal stenosis, esophageal duplication cyst, esophageal webs or rings,, diverticulum of esophagus and congenital short esophagus

1. CONGENITAL ANOMALIES
OF ESOPHAGUS
Ms Alisha Talwar

2. Overview
•Embroyologyand related A/P of Esophagus
•Esophagealatresia and tracheoesophagealfistula
•Laryngotracheoesophagealcleft
•Esophagealstenosis and webs
•Esophagealduplications
•Diverticulum of esophagus
•Congenital short esophagus

3. Development of esophagus
•During the 4
th
wk., a small diverticulum appears in the
ventral wall of Pharynx.
•A ‘TracheoesophagealSeptum’ gradually separates the
ventral Respiratory diverticulum from the dorsal part of
foregut.
•As a result, the Pharynx is divided into;
•a ventral portion the “respiratory primordium”,
•a dorsal portion, the “esophagus”.

5. Growth of Esophagus
•Up to the 4
th
week it is very short.
•Then, it elongates rapidly due to
the descent of developing heart
and lungs.
•By the 7
th
week it reaches its final
position.
•Its lumen is completely or partially
obliterated due to proliferation of
its epithelial lining.

6. Contd…
•Recanalization occurs by the end of embryonic period (after
8
th
wk).
•Its muscles developed from the surrounding mesoderm.
•It is striated in the upper 1/3,
•mixed in the middle 1/3 and
•smooth in the lower 1/3 (vagus)

7. Associated anomalies incidence
•Cardiovascular 24%
•Genitourinary 21%
•Gastrointestinal 21%
•Musculoskeletal 14%
•Central nervous system 7%
•VACTERL association 20%
•Overall incidence 50%-70%
•Incidence of the VACTERL association in the EA population is -
20%.

8. VACTERL
•Requires a minimum of three of the components to qualify
as a “VACTERL patient.”
•Vertebral -17%
•Anal -12%
•Cardiac -20%
•TEF
•Renal -16%
•Limb-10%
•VACTERL –H-HYDROCEPHALUS

9. Anatomy and Physiology of
Esophagus

13. Classification
•Gross classification
•Waterston risk group
•Okamoto modification of spitzclassification

14. Anatomical classification
•EsophgealAtresia with a distal tracheoesophagealfistula -
84% GROSS TYPE C
•EA without TEF-6% GROSS TYPE A
•TEF without EA-4% GROSS TYPE E
•EA with a fistula to both pouches-1% -GROSS TYPE D
•EA with a proximal TEF -5%-GROSS TYPE B

15. Waterston Risk Group

16. Okamoto modification of spitz
classification

17. TracheoesophagealFistula
•Tracheoesophagealfistula is an abnormal connection in one
or more places between the esophagusand the trachea.
•TE fistula is a birth defect, which is an abnormality that
occurs as a fetusis forming in its mother's uterus.

18. TracheoesophagealFistula
•When a baby with a TE fistula swallows, the liquid can pass
through the abnormal connection between the esophagus
and the trachea.
•When this happens, liquid gets into the baby's lungs. This
can cause pneumonia and other problems.

20. Esophagealatresia
•EsophagalAtresia is the failure of oesophagus to form a
continuous passage from the pharynx to the stomach.
•Liquid that a baby swallows cannot pass normally through
the esophagusand reach the stomach. Milk and other fluids
cannot be digested if the esophagusdoes not connect to
the stomach.
•If a TE fistula is also present, liquid that a baby swallows
can pass through the connection between the esophagus
and the trachea and go into the lungs. This can cause
pneumonia and other problems.

21. •https://www.youtube.com/watch?v=gn0p-rnWeIg

22. Epidemiology
•TEF occurs in 1 in 3500 births, with slight male dominance.
•EA with or without TEF is common in prematurity, with 34%
of cases weighing less than 2500 grams.
•Approximately 50% of neonates with EA or TEF have other
anomalies also.
•Usually cardiac anomalies are seen in 14.7 –28 % cases of
TEF

23. Types of Trache-oesophageal
Fistula

24. Classification
•Type A:isolatedesophagealatresia(8%)
•Type B:proximal fistula with distal atresia (1%)
•Type C:proximal atresia with distal fistula (85%)
•Type D:double fistula with intervening atresia (1%)
•Type E:isolated fistula (H-type)(4%)

25. Etiology
•Maternal alcohol and smoking
•Exogenous sex hormones
•Exposure to methimazole
•Prolonged mechanical ventilation via Endotracheal or
tracheotomy tube
•Maternal polyhydamnious
•In first trimester exposure to Diabetes mellitus

26. Contd..
•Trisomy 13, 18, or 21
•Other digestive tract problems (such as diaphragmatic
hernia, duodenal atresia, or imperforate anus)
•Heart problems (such as ventricular septal defect, tetralogy
of Fallot, or patent ductusarteriosus)
•Kidney and urinary tract problems (such as horseshoe or
polycystic kidney, absent kidney, or hypospadias)

27. Contd..
•Muscular or skeletal problems
•VACTERL syndrome (which involves Vertebral, Anal,
Cardiac, TE fistula, Renal, and Limb abnormalities)
•Up to one-half of all babies with TE fistula or esophageal
atresia have another birth defect.

28. Pathophysiology
•At about 4 weeks of gestation, a laryngo-tracheal groove is
formed which divides the foregut into two longitudinal
tubes, which further develop into the respiratory tract and
the digestive tract.
•Defective separation due to deviated or incomplete septum
or incomplete fusion of tracheal fold results in malformation
of trachea and oesophagus

29. •https://www.youtube.com/watch?v=cWNQEoEPFTM

30. Clinical Manifestations
•Violent response occurs on feeding
•Infant coughs and chokes
•Fluid returns through nose and mouth.
•Cyanosis occur
•The infant struggles
•Excessive secretions coming out of nose and constant
drooling of saliva.

31. Clinical Manifestation
•Saliva is frothy.
•Abdominal distension occurs in presence of type III, IV and
V fistula.
•Intermittent unexplained cyanosis and laryngospasm,
caused by aspiration of accumulated saliva in blind
oesophageal pouch.
•Pneumonia may occur due to overflow of milk and saliva
from oesophagus through fistula into the lungs.

33. Diagnosis
•History and Physical examination
•Endoscopy
•Contrast X-ray
•Ultrasonography
•CT scan

34. Prenatal suspicion
•Ultrasound examination reveals polyhydramnios, absence of
a fluid-filled stomach, a small abdomen, lower-than-
expected fetalweight, and a distended esophagealpouch.
•FetalMRI may be used to confirm the presence of EA/TEF
•Earliest clinical sign of EA -usually excessive salivation that
results from pooling of secretions in the pharynx.
•Regurgitation, choking, and coughing

35. Detected Post-natally
•X-ray taken with radiopaque catheter placed in esophagus
to check for obstruction, chest X-ray shows a dilated air-
filled upper esophagealpouch and can demonstrate
pneumonia.
•Inability to pass a NG tube into stomach because it meets
resistance
•Bronchoscopy visualizes fistula between trachea and
esophagus

36. Contd..
•Abdominal ultrasound and echocardiogram to check for
cardiac abnormalities.
•Abdomen distends as inspired air passes into stomach.
•Chemical pneumonitis due to gastric acid reflux
•Flexible catheter such as a feeding tube will often curl up in
the upper pouch
•ECHO, Karotyping, USG KUB

38. TEF without EA
•Repeated coughing or choking during feedings, often with
pulmonary infiltrates on chest radiograph suggesting
aspiration.
•The diagnosis can be made by barium esophagographyin
the prone position.
•CONFIRMATION –BRONCHOSCOPY WITH OR W/O
ESOPHAGOSCOPY

40. Management
•Minimize further aspiration, prevent and treat pulmonary
infections, provide supportive therapy until definitive
surgery can be performed.
•Gastrostomy tube to reduce reflux
•Acid suppression therapy
•The bed head should be elevated to prevent further passive
regurgitation
•Antibacterial therapy

41. Contd..
•Pneumonitis from aspiration of upper pouch secretions and
reflux of gastric acid through the TEF causing worsening
respiratory distress.
•Continuous aspirating saliva under low-pressure suction.
(DoubLumen REPLOGLE TUBE)
•Endotracheal intubation to be avoided

43. Surgical Management
Prompt Primary Repair
•Fistula found by bronchoscopy is divided, followed by
anastomosis of proximal and distal segments if infant birth
weight permit and is out of pneumonia.
Staging
•Initially fistula division gastrostomy are performed with later
secondary esophagealanastomosis or colonic transposition
performed approximately 1 year later to effect total repair

45. Surgical Management
•Circular esophagotomy: It may be performed on
proximal pouch to gain length and allow foe primary
anastomosis at initial surgery
•Cervical esophagotomy: When ends of esophagusare
too widely separated ,esophagusreplacement with segment
of intestine (colonic transposition ) is done at ages 18 to 24
months.
•FiberopticTracheoscopy: Repair of trachesophageal
fistula can expedite and facilitate surgery on ventilated
patient

46. Complications
EARLY
•Anastomotic leak
•Anastomotic stricture
•Recurrent
tracheoesophagealfistula
LATE
•GERD
•Tracheomalacia
•Respiratory Disease
•Disordered esophagealperistalsis

47. Anastomotic Leak
•Most leaks are clinically insignificant and can be managed
with adequate drainage and nutritional support.
•Retropleuralapproach + patent mediastinaldrainage-95%
heals
•Breakdown of anastomosis-identified in 24-48 hours.
•Infant frequently deteriorates from tension pneumothorax
or mediastinitis.
•Redoprocedureor esophagostomy+ gastrostomy to be
done.

48. Stricture
•Stricture -presence of symptoms (dysphagia and recurrent
respiratory problems from aspiration or foreign body
obstruction) and narrowing noted on endoscopy or contrast
esophagography.
•Traditionally treated by dilation.
•Balloncatheter is recently used.
•First dilatation at 1month postop. 2-3 sessions of dilatation
usually suffice. GERD should be prevented or treated.
•Redo anastomosis or esophagealreplacement

49. Recurrent TEF
•Attributed to anastomotic leak with local inflammation and
erosion through the previous site of TEF repair.
•Symptoms can be typical of those seen with a congenital H-
type TEF including coughing and choking or cyanosis with
feedings.
•Diagnosis may be suggested by an air-filled esophaguson
plain radiographs of the chest.
•Thoracotomy with fistula ligation and division WITH pleura,
intercostal muscle, or pericardium interposed between the
esophagusand trachea.

50. Gastroesophagealreflux disease
•GERD occurs in 30% to 70% of patients after repair of EA.
REASONS-
•Shortening of the intra-abdominal portion of the esophagus
due to traction.
•Manipulation by instruments
•Esophagealmotor dysfunction
•Congenital

51. Contd
TREATMENT-
•Thickening of Feedings.
•Positioning of the infant in a prone or upright posture.
•Administration of acid reduction agents such as histamine-
2 blockers, proton pump inhibitors, and prokineticagents.
•Nissenfundoplication with floppy wrap.

52. Tracheomalacia
•Tracheomalaciahas been noted to affect up to 75%.
•Tracheomalacia-generalized or localized weakness of the
trachea that allows the anterior and posterior tracheal walls to
come together during expiration or coughing.
•Embryologically, shorter cartilage fails to provide the support
necessary to maintain a patent airway.
•“Brassy” or “barking” cough in mild cases to recurrent
pneumonia or acute, life-threatening apneicspells.
•In acute life-threatening events, the operative treatment of
choice is aortopexy.

54. Congenital EsophagealStenosis
•Congenital esophagealstenosis (CES) is a rare anomaly,
resulting from incomplete separation of the respiratory tract
from the primitive foregut at the 25th day of life (Serrao,
Santos, Gaivao, Tavares & Ferreria, 2010)
•It is characterized by intrinsic narrowing of
theesophagussecondary tocongenital malformationof the
esophagealwall architecture (Lees & Dicken, 2016)

55. Classification
•Group I -Tracheobronchial rests (cartilage, respiratory
mucus glands, ciliated epithelium)
•Group II -Membranous diaphragm
•Group III -Fibromuscularstenosis

56. Etiology

57. Pathophysiology
•CES is a narrowing of a region of the esophagus.
•A web, or diaphragm, consists of a thin squamous epithelial
membrane in the esophageallumen.
•It typically causes a partial obstruction in the middle to
lower esophagus.
•Congenital muscular hypertrophy is characterized by
submucosalproliferation of smooth muscle and fibrous
connective tissue beneath a normal squamous epithelium.
Individuals with congenital muscular hypertrophy may be
asymptomatic.

58. Clinical Manifestations
•First clinical signs are abnormalities of the swallowing
mechanism caused by the intrinsic narrowing of the
esophagus(Serrao, Santos, Gaivao, Tavares & Ferreria,
2010)
•History of postprandial vomiting of undigested food
particles beginning in the weaning period, with no report of
weight loss (Serrao, Santos, Gaivao, Tavares & Ferreria,
2010)
•High lesions present with respiratory symptoms, while low
lesions present with vomiting.

59. Types
Three anatomic types of CES are distinguished:
•Esophagealmembranes (EM) or web,
•Fibromuscularstenosis (FMS)
•TBR

60. Diagnosis
•Diagnosis is usually delayed, requiring an accurate history
and high level of suspicion, alongside with an
esophagogram.
•Definite diagnosis is only confirmed by histological
examination (Serrao, Santos, Gaivao, Tavares & Ferreria,
2010)

61. Asymmetric, regular and tapered narrowing of a short segment of the distal esophagus. Tertiary
contractions were observed along the esophagus, except in the narrowed segment, which
showed no peristalsis. The proximal portion of the lower esophaguswas greatly dilated

62. Esophagoscopyshowed a circumferential, slightly non-central
narrowing at the distal esophagus, 2cm proximal to the
esophagogastricjunction.

63. Diagnostics Tests
Esophagogram
•Normal findings
•concentric, aperistaltic, sometimes asymmetric narrowing of
the upper, mid, or distal esophagus, with variable length
(about 1–2cm), smooth contours and tapered borders
•Proximal dilatation and GER can occur

64. Contd….
Endoscopy
•Narrowing of the upper, mid-or distal esophagus
Endoscopic ultrasonography
•Asymmetric thickness of the muscular layer at the narrow
portion of the upper, mid-or distal esophagus

65. Contd…
CT
•Diffuse, circumferential, asymmetric and non enhanced wall
thickening of the upper, mid-or distal esophagus
MRI
•Diffuse, circumferential and asymmetric wall thickening of
the upper, mid-or distal esophaguswith intermediate signal

66. Differential Diagnosis
•Gastroesophagealreflux (GER)
•Achalasia
•Stenosis secondary to GER
•Foreign body or Neoplasiccauses (Serrao, Santos, Gaivao,
Tavares & Ferreria, 2010)

67. Management
•Treatment usually involves surgery, depending on the
severity, location and type of stenosis.
•Endoscopic dilation orexcision
•Excision of the affected segment by laparotomyor
thoracotomy with an end-to-end Esophageal
anastomosis is commonly performed, though circular
myectomyand laparoscopic esophageal
stricturoplastyhas also been reported. In some cases
Fundoplicationhas also been performed.

68. EsophagealDuplication Cyst
•Esophagealduplication cysts are congenital anomalies of
the foregut resulting from aberration of the posterior
division of the embryonic foregut at 3-4 wkgestation
•They represent either simple epithelial-lined cysts, or true
esophagealduplication, which is a duplication of the
muscularismucosa and externawithout epithelial
duplication.

70. Contd..
•Up to 80% of cases are diagnosed during childhood
•Most of them are found in the mediastinum and manifest
themselves as separate masses along or in continuity with
the native esophagus.
•Intramural esophagealcysts are considered very
uncommon in adults
•Esophagealduplication cyst accounts for 10-15% of all
congenital duplication cysts involving the GI tract

71. Pathophysiology
•An esophagealduplication may be open at both ends
(double esophagus), open at one end (diverticulum), or
closed (elongated cyst).
•Rests are areas where embryonic tracheal or esophageal
cells reside in mesodermal tissues.
•These areas may form cysts in the muscular tissues.
•A choristomais a distinct cartilaginous cyst that partially or
completely encircles a region typically in the lower third of
the esophagus.

72. Clinical Manifestation
•Symptoms are caused by compression or displacement of
surrounding mediastinalstructures
•It comprise of
•Dysphagia
•Respiratory distress
•Failure to thrive
•Retrosternal pain

73. Diagnosis
•Esophagogastroduodenoscopy
•CT scan
•MRI
•EUS

74. Management
•Surgical enucleationis the treatment of choice in
symptomatic cases.
•In asymptomatic cases, surgical resection has been
suggested due to the rare development of complications or
malignancy, but many of these lesions can be safely
observed.
•Some experts also recommend close surveillance alone
when the diagnosis has been confirmed by needle
aspiration, but no standard regimen for surveillance exists

75. Laryngo-tracheo-esophagealcleft
•A laryngo-tracheo-esophagealcleft (LC) is a congenital
malformation characterized by an abnormal, posterior,
sagittal communication between the larynx and the
pharynx, possibly extending downward between the trachea
and the esophagus.

76. Epidemiology
•The estimated annual incidence of LC is 1/10,000 to
1/20,000 live births, accounting for 0.2% to 1.5% of
congenital malformations of the larynx.
•These incidence rates may however be underestimated due
to difficulty in diagnosing minor forms and a high mortality
rate in severe forms.
•A slightly higher incidence has been reported in boys than
in girls. No specific geographic distribution has been found.

77. Classification

78. Contd…
•Type 0: submucosalcleft
•Type I: supraglottic, interarytenoidcleft, above the vocal fold
level
•Type II: cleft extending below the vocal folds into the cricoid
cartilage
•Type III a: cleft extending through the cricoid cartilage but not
into the trachea
•Type III b: cleft extending through the cricoid cartilage and into
the cervical trachea
•Type IV: cleft extending into the thoracic trachea, potentially
down to the carina

80. Laryngotracheoesophagealcleft, type I
(Benjamin and Inglisclassification).

81. Etiology
•Laryngeal clefts result from failure of fusion of the posterior
cricoid lamina and abnormal development of the tracheo-
esophagealseptum.

82. Etiopathology
•Several models have been proposed to explain tracheo-
oesophageal anomalies, including LC:
•Intra-embryonic pressure: an intense curvature of the
cervical region, during the heart's development, could cause
a strain and a displacement of the oesophagus, resulting in
growth anomalies.
•Epithelial occlusion: the oesophagus has a solid stage of
development, and eventually re-canalizes. A failure of this
latter process could result in malformations.

83. Contd….
•Vascular occlusion: the persistence of an abnormal
vessel could lead to vascular insufficiency in the foregut,
resulting in growth anomalies.
•Differential cell growth: abnormal cell development in
either the ventral or dorsal part of the developing tracheo-
oesophagus could result in oesophageal or tracheal defects.

84. Contd…
•It has been suggested that a premature arrest in
development of the tracheo-oesophageal septum, and the
lack of fusion of the two lateral parts of the developing
cricoid cartilage, could be responsible for LC.
•This model does not explain the mechanism of associated
malformation, such as laryngeal atresia and tracheo-
oesophageal fistula.

85. Clinical Description
•Depending on the severity of the malformation, patients
may present with
•Stridor
•Hoarse cry
•Swallowing difficulties
•Aspirations
•Cough
•Dyspnea
•Cyanosis through to early respiratory distress.

86. Most frequent clinical symptoms
•Swallowing disorders (50%)
•Aspiration and cyanosis during feeding (53 to 80%)
Chronic cough (27 to 35%)
•Pharynx and larynx (43%)
•Stridor (10 to 60%)
•Toneless or weak cry (16%)
•Pharyngeal hypersecretions(10 to 23%)
•Respiratory (37%)
•Recurrent pneumonia (16 to 54%)
Respiratory distress at birth

87. Associated Syndromes
•The OpitzG/BBB syndrome,
characterized by laryngeal
malformations (including LCs)
and craniofacial defects

88. •The 22q11 monosomy
(CATCH 22, 22q11
microdeletion, DiGeorge
syndrome...). This syndrome
may include numerous
malformations especially
hypoplasicthymus and
parathyroid glands, cardiopathy,
velopharyngealinsufficiency with
or without cleft palate, and
sometimes LC.

89. •The PallisterHall syndrome is
characterized by the
association of laryngeal (LC),
gastrointestinal,
cardiopulmonary, limb
(polydactyl and syndactyl)
and neurological
malformations (congenital
hypothalamic
hamartoblastomawith
hypopituitarism). Its
inheritance is autosomal
dominant.

90. Contd..
•The VACTERL association. Its aetiology is still unknown in
man. The acronym stands for Vertebral, Anal, Cardiac,
Tracheo-oesophageal (including LC), Ear (middle and
inner), Renal, and Limb malformations.

91. 2 day Infant with sacrococcygealteratomatype 1,absent anal
opening, small perineum, vestibular fistula, TEF, EA, increased
cardiothoracic ratio, left multicystickidney
Rahul Gupta and Vinita Chaturvedi
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India

92. •The CHARGE syndrome results
in the main, from a CHD7 gene
mutation. The acronym stands
for Coloboma, Heart
malformations, choanalAtresia
(uni-or bilateral), growth and
mental Retardation,
Genitourinary malformations,
and Ear malformations
(external, middle, and inner
ear).

93. Diagnostic methods
•The age of diagnosis depends mainly on the severity of the
clinical symptoms and therefore on the extent of the LC.
•Diagnosis is made either based on clinical manifestations or
on investigations, such as endoscopy, X-ray, CT scan,
performed for other conditions.

94. Contd..
Antenatal diagnosis
•Prenatal diagnosis of LC has never been reported, although
associated anomalies may be detected on fetal
ultrasonography.
Genetic counseling
•LC appears to be mostly sporadic although some familial
cases with suspected autosomal dominant transmission
have been reported.

95. Management
•Management involves maintenance of satisfactory
ventilation, prevention of secondary pulmonary
complications as a result of repeated aspirations, and
adequate feeding.
•Endotracheal intubation may be required for respiratory
distress in severe cases.

96. Contd..
•Treatment requires endoscopic or external surgery to close
the cleft.
•Surgery should be performed as early as possible to avoid
complications related to aspiration and gastric reflux, except
in type 0 and type 1 cases in which conservative measures
must first be attempted.

97. Columnar Epithelium Lined Lower
Esophagus(Barrett’s Esophagus)
•Columnar-lined esophagus(CLE) is commonly called
“Barrett’s esophagus”.
•Barrett’s epithelium, Barrett’s mucosa, Barrett’s ulcer,
Barrett’s stricture and Barrett’s carcinoma are used
frequently in the literature.

98. Barrett’s Esophagus
•It is a pathological premalignant condition in which the
lower segment of the esophagusbecomes lined by a
metaplasticcolumnar epithelium in the form of either
circumferential extension of the gastric columnar epithelium
or islands of columnar mucosa or both.
•The presence of specialized columnar epithelium anywhere
in the esophagusrepresents a true metaplasia of the
normal squamous epithelium and is highly suggestive for
the diagnosis of CLE.

100. Diagnosis
•Barrett’s esophaguscan only be seen through an
endoscope but always requires biopsies for diagnosis.

101. Treatment of Barrett’s esophagus
The management of newly discovered Barrett’s esophagus
has three objectives:
•The treatment of esophagitis
•Elimination of Barrett's tissue in patients with dysplasia
•The early detection or prevention of cancer through a
surveillance program

102. Medication Treatment
•Esophagitis is commonly treated with medications to control
acid production or secretion (primarily proton pump
inhibitors).
•Proton pump inhibitors (PPIs) are recognized as the most
powerful and effective drugs used to inhibit acid secretion
and allow healing of tissue damage in the esophagus.

103. •Guidelines from the American College of Gastroenterology
recommend periodic check-ups (surveillance) or action as
follows:
In the presence of Barrett's esophaguswith:Recommendation:
No dysplasia Endoscopic examination every 2-3 years
Low-grade dysplasia Endoscopic examination every 6 months for
one year, and if negative results, once per year
thereafter.
High-grade displaysia Expert confirmation (second opinion).
Depending on individual factors doctor may
recommend selective resection (surgical
removal of the esophagus) or ablation
therapy (endoscopic therapies that remove
or destroy the Batterr'stissue). Or endoscopic
examination every 3 months.

104. Elimination of Barrett's tissue
•They include freezing (cryo) or burning (radiofrequency)
the Barrett's tissue.
•PPIs improve the esophagitis and heartburn. Ablation
(elimination) of Barrett's tissue is presently indicated only in
those with dysplasia.
Early detection or prevention

105. Esophagealwebs or Rings
•Esophagealwebs or rings are thin, membranous folds of
tissue that form in the esophagus.
•Health professionals may use both “webs” and “rings” to
refer to the same structure. These structures make the
esophagusnarrower, blocking it fully or partially.
•Rings and webs are the most common structural
abnormalities of the esophagus.

107. Esophagealring
•An esophagealring is defined as a concentric, smooth, thin
(3-5 mm) extension of normal esophagealtissue consisting
of 3 anatomic layers of mucosa, submucosa, and muscle.
•Usually is found in the distal esophagus.
•Three types of esophagealrings exist, and they are
classified alphabetically as A, B, and C.

108. Diagram of A, B (Schatzki), and C rings in the distal
esophagus. The B ring marks the proximal border of a hiatal
hernia. V=vestibule and HH=hiatal hernia.

109. Multiple rings found in the entire esophagus

110. Esophagealrings-A ring
•The A ring is uncommon and represents a muscular ring
proximal to the squamocolumnarjunction (SQJ).
•This muscular ring is not a fixed structure and may only be
demonstrated in some barium swallows. It is located
approximately 1.5 cm proximal to the SQJ, and it is rarely
symptomatic.
•Hirano et al described a unique manometricpattern of
peristaltic, high-amplitude, long-duration, and multiple
peaked esophagealcontractions in 3 men with A rings.

111. B ring
•The most famous and common ring in the esophagusis the
B ring orSchatzkiring
•Schatzkiring is a web because it is composed of only
mucosa and submucosa.
•It is located at the SQJ, and it marks the proximal margin of
a hiatal hernia.

112. C ring
•The C ring is a rare anatomic finding on radiographic
studies referring to theindentation caused by the
diaphragmatic crura. It is rarely symptomatic.

113. Pathophysiology
•Several theories have been proposed for the formation of
esophagealwebs and rings. These include etiologiesrelated
to congenital origin, iron deficiency, developmental
abnormality, inflammation, and autoimmunity.

114. Clinical Manifestations
•Asymptomatic
•The hallmark symptom of esophagealrings and webs is
dysphagia.
•Achalasia, nutcracker esophagus, or diffuse esophageal
spasm.
•If acquired supporting history for Schatzkirings includes
heartburn and a hiatal hernia.

115. Clinical manifestations
•Alarming symptoms that are not consistent with esophageal
rings are progressive dysphagia, weight loss, and anemia.
•These symptoms suggest a mechanical, fixed obstruction,
such as a stricture or malignancy
•In Paterson-Brown-Kelly syndrome (PVS), physical findings
include koilonychia(spoon nails), cheilosis, and glossitis.

116. Clinical Manifestation
•Rare skin diseases, such as epidermolysisdystrophicaand
benign mucous membrane pemphigoid, present with skin
bullous lesions.
•Acute caustic ingestion of lye or alkaline solution can lead
to oropharyngealinjury manifested as ulcerations,
exudates, edema, and erythema.

117. Diagnosis
Laboratory Studies
•It is not necessary in most patients.
•To establish a diagnosis of celiac sprue, iron deficiency anemia,
and Paterson-Brown-Kelly syndrome (PVS), the following tests
are necessary:
•CBC count
•Iron panel
•Ferritin
•Antigliadinantibodies
•Antiendomysialantibodies

118. Imaging Studies
Barium swallow
•Initial test in patients with dysphagia.
•Barium swallow allows detection of intraluminal obstruction.
•It is useful in detecting peptic strictures, pill-induced
esophagitis and/or strictures, mucosal tumors, intramural
tumors(primarily leiomyomas), and extraesophageal
compression. Infections related to candidiasis,
cytomegalovirus, herpes simplex virus, and idiopathic ulcers
sometimes may be suspected following barium studies

119. Esophagogastroduodenoscopy
•Showing the presence of hypermotilityof the esophagus,
the presence of excessive fluid and secretion in the
esophagus, presbyesophagus(tortuosity), or very small
rings and webs.
•Endoscopy allows biopsy of lesions, such as strictures,
polyps, or masses, if any query of malignancy exists.

120. Histologic Findings
•Histologic findings of esophagealwebs are usually normal
squamous epithelia with occasional chronic inflammatory
cells in the subepithelialtissue.

121. Management
Pharmacological Management
•Histamine type 2 (H2)-receptor antagonists, including
cimetidine, famotidine, and ranitidine, may be used for
mild-to-moderate GERD symptoms.
•For severe GERD symptoms, proton pump inhibitors (eg,
omeprazole, lansoprazole, rabeprazole, pantoprazole) are
recommended.

122. Surgery
•Endoscopic sphincterotomy-Endoscopic electrocautery
incision using a papillotomecatheter was reported to be
successful in alleviating symptoms associated with
refractory lower esophagealrings

123. Other Rarer Anomalies
Congenital diverticulum of esophagus
•Symptoms include emesis, regurgitation, and recurrent lung
infections.
•Treatment is surgical excision of the diverticulum,
fundoplication, and pyloroplasty

124. Congenital short esophagus
•This condition is usually associated with a hiatus hernia and
an intrathoracicstomach.
•Infants usually present during the first month of life with
failure to thrive.
•Surgical intervention involves an antirefluxprocedure, often
combined with an esophageal-lengthening procedure (eg,
Collis-Nissenfundoplication).

125. Nursing Management
Pre-Operative
•Risk for aspiration related to structural abnormality.
•Risk for deficient fluid volume related to inability to take
oral feeds.
•Impaired breathing related to frequent laryngospasm and
excessive secretions in the trachea.

126. Post-Operative
•Ineffective airway clearance related to disease process.
•Impaired nutrition related to surgery.
•Altered comfort related to surgical process.
•Impaired gaseous exchangerelated to abnormal opening
between esophagusand trachea as evidenced by cyanosis.
•Impaired swallowingrelated to mechanical obstruction.

127. Contd…
•Risk for injuryrelated to surgical procedure.
•Anxietyrelated to difficulty swallowing, discomfort due to
surgery.
•Altered family processesrelated to children with physical
defects.
•Risk for aspirationrelated to difficulty in swallowing.

128. Nursing Interventions
•A nasogastric tube is put in the upper oesophageal segment
and is aspirated frequently, to prevent collection and
aspiration of secretion into the trachea.
•Intravenous fluids are administered to maintain the
hydration status of infant.
•Place the infant in semi-upright position and administer
oxygen if cyanosis is present.

129. Contd..
•A cervical oesophagostomyis made to drain out secretions
from blind oesophageal pouch and administer oxygen to
infant.
•Feed the infant orally or by gastrostomy.
•Position the baby comfortably in semi-fowler’s position and
administer analgesics.
•Do suction as required.
•Ensure proper nutrition by consulting with physician for
enteral feedings, preferably a PEG tube in most cases.

130. Contd…
•Check placement before feeding, using tube markings, x-ray
study (most accurate), pH of gastric fluid, and colorof
aspirate as guides; if ordered by physician, put several
drops of blue or green food coloringin tube feeding to help
indicate aspiration.
•Test the glucose in tracheobronchial secretions to detect
aspiration of enteral feeding
•Elevate the head of bed to 30 to 45 degrees while feeding
aspiration.

131. Contd..
•Assist the patient in developing new anxiety-reducing skills
(e.g.,relaxation, deep breathing, positive visualization, and
reassuring self-statements)

132. Conclusion
•Evaluate the patient of EA-TEF with complete care to rule out
any association & stratify the risk factors without come.
•Proper & specific pre-operative management and complete plan
of management including expected post-operative complication.
•Presentation of the patient, operative & post-operative morbidity
to be discussed with parents after complete assessment.
•Early diagnosis allows time and place of delivery to be planned
so that early pediatricssurgical correction and neonatal care is
available.

133. References
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