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Pediatric Neck Masses, TEF, Diaphragmatic Hernia
Pediatric Neck Masses, TEF, Diaphragmatic Hernia
TEF , Diaphragmatic
Hernia
Dr. Mahmoud AbuAwad
Supervised By : Dr.Mohammad Al-Omari
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Disclosures.
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Contents
Pediatric Neck Masses
Esophageal Atresia/Tracheoesophageal Fistula.
Congenital Diaphragmatic Hernia.
Conclusion.
References.
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Pediatric Neck Masses
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Pediatric Neck Masses.
Imaging.
Congenital
Branchial Anomalies.
Thyroglossal Duct Cysts.
Dermoid Cysts.
Thymic Cysts.
Infectious.
Lymphatic Malformation ( Cystic Hygroma ).
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Imaging.
• Advantages
• CT available in emergent situations ,
• Completed quickly for patients who are critically ill.
• No need for sedation and minimal artifact from movement.
• Offering more osseous detail such as remodeling or erosion, as well as
calcifications.
• Disadvantage
• The exposure to ionizing radiation, which could potentially have carcinogenic
effects in the long term.
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• MRI
• Advantages
• Excellent soft tissue detail and avoidance of ionizing radiation.
• Details of the margins and possible neural spread or intracranial extension of a mass can be noted with
the use of a contrast-enhanced MRI.
• Disadvantages
• Requires sedation due to concerns of movement artifact in the pediatric population
• Modalities of Mx
• Infection antibiotics prior to excision (dissection and identification of
important structures easier ).
• Abscesses needle aspiration or if needed , incision and drainage. ( be
careful for the nerves ).
• Recurrence for branchial cleft cysts 3%, if redo up to 20% .
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• Anatomy and Embryology
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First branchial cleft anomalies :
• 8% of branchial cleft anomalies,
• Two types
• Type 1 branchial cysts a fistula around the conchal cartilage , The tract can
follow the course of the external auditory canal and is lined by squamous
epithelium.
• Type 2 branchial lesions near the angle of the mandible and are composed of
both epithelium and mesoderm.
• Both can be closely associated with the facial nerve Type 2
branchial lesions can loop under the facial nerve, pushing it laterally and
inferiorly.
• Course The tract for a first branchial cleft usually extends from
the opening near the mandible, near the posterior aspect of the
parotid, and toward the external auditory canal.
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Second branchial cleft anomaly
• M.C , 90% of branchial cleft anomalies.
• Isolated cyst or as a sinus tract/fistula that extends from the cervical
skin to the tonsillar fossa.
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Third branchial cleft Anomaly
• Rare.
• Presentation
• cysts low and anterior in the neck.
• Fistulas can course from the piriform fossa and drain to the anterior cervical
skin ( pierces the thyrohyoid membrane and tracks under the
glossopharyngeal nerve and internal carotid artery, but stays above the vagus
nerve).
• These lesions can be intimately involved with the thyroid, and
sometimes a hemithyroidectomy is required for repeated
recurrences.
• An infected TGDC or third branchial cleft cyst may be the cause of
suppurative thyroiditis in children.
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Fourth branchial cleft Anomaly
• Extremely rare.
• The external location for a fourth branchial cleft is the same as the
second and third, but the internal opening is near the apex of the
piriform sinus.
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Treatment
• The standard treatment for all types of branchial anomalies is
complete surgical excision.
• Under GA
• Position supine with neck slightly extended with a shoulder roll.
• sinus tract or fistula present a small ellipse is made around this
opening , cannulation with a small lacrimal probe with methylene
blue .
• The tract is then dissected cephalad until the end of the sinus tract or
until the internal opening of the fistula is reached.
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• For Second Branchial cleft Anomalies :
• The tract is then ligated with absorbable suture. Often a direct laryngoscopy
or simply a finger placed in the oropharynx can help to visualize the course of
the tract and assist with dissection.
• Some patients might require a “stepladder” incision to trace the tract
superiorly
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• For Cartilaginous remnants : ( rarely get infected , removal for
cosmetic indications) are normally small and present in the
subcutaneous tissue along the anterior border of the
sternocleidomastoid, are usually palpable and easily resected.
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Preauricular cysts, pits, and sinuses
• Common ,
• From the first two branchial arches.
• bilateral in comparison to first branchial cleft cysts.
• Rarely become infected.
• The tracts extend from the skin down to the helical cartilage of the
auricle and are lined with squamous epithelium.
• Treatment is surgical excision
• Recurrence 19% to 40%.
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Thyroglossal Duct Cysts
• Congenital
• Thyroglossal duct tract that occurs during the descent of the thyroid.
• The thyroid descends from the foramen cecum at the sixth week of gestation
• Tracts along the anterior neck in close association with the developing hyoid bone.
( involutes , but if it persists TGDC at any point from the foramen cecum to the thyroid
• Presentation A midline neck mass that may move with protrusion of the
tongue.
• Imaging
• US ( characteristics of the cyst ),
• determine if there is a normal thyroid gland present.
Rarely, TGDC could be the sole functioning thyroid tissue in the patient.
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• Treatment Surgical excision and removing the entire tract with a
core of tissue and the central
• Portion of the hyoid bone ( minimize recurrence).
• Complete excision ( curative )
• Recurrence less than 10%
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• Under GA .
• Postion Supine , the neck extended and placement of a shoulder
roll.
• A horizontal skin incision is made over the cyst, with no need to
excise skin unless a fistulous tract or infection has involved the
dermis.
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• Careful dissection around the cyst then allows continued dissection
superiorly toward the hyoid bone.
• Muscular attachments to the central hyoid bone are removed with
cautery and the hyoid is removed en bloc with the cyst and tract.
• A core of tissue is then removed above the hyoid bone toward the
base of tongue.
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• The tract is then ligated with absorbable suture.
• Placing a gloved finger at the base of tongue can sometimes facilitate
this maneuver
• The wound should then be irrigated copiously and closed in a layered
fashion.
• A drain is often used.
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Dermoid Cysts
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Thymic Cysts
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Infectious
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• Lymphadenitis
• M.C in the submandibular region and cervical nodes,
• Sources
• Viral, Bacterial upper respiratory infections are the most common cause
of cervical lymphadenopathy,
• Fungal ( Immunocompromised )
• Neoplastic.
• Cat scratch disease,
• Mononucleosis,
• Atypical mycobacterium,
• Tuberculosis
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• Acute suppurative lymphadenitis M.C in the 6-month to 3-year
range, with lymph node enlargement associated with an upper
respiratory tract infection.
• Etiologies Staphylococcus aureus and group A beta-hemolytic
streptococci.
• Presentation Overlying skin changes with erythema and cellulitis,
with an associated leukocytosis and fever.
• The lymph node enlarges and eventually outgrows the blood supply,
resulting in central necrosis.
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• Management
• Needle aspiration of purulence, but often requires an incision and drainage
with placement of a drain or packing under anesthesia. ( care of near vital
structures).
• Antibiotics are geared toward Staphylococcus and Streptococcus but cultures
should be taken with a change in antibiotic therapy if necessary.
• Excisional biopsies If L.N > 2 cm , > 8 weeks, immobile or rapidly growth
( cultures, histopathology, and flow cytometry).
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• Types
• Microcystic
• Above the mylohyoid and can involve the floor of mouth, tongue, parotid,
and lip .
• Poorly defined borders and infiltrate nearby muscle and fatty planes
• Macrocystic
• below the mylohyoid and have multiple large,
• loculated cysts in varying size and shape ( infection or trauma, which can
lead to airway obstruction, functional issues (swallowing, speech), or
deformity
• Mixed lesions.
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• Treatment
• Observation, sclerotherapy, or surgery.
• Macrocystic Amenable to both sclerotherapy and surgical excision,
• Microcystic Exision would often result in unacceptable morbidity
(infiltrative nature).
• Subtotal resection high recurrence rate , Difficult surgery
( previous inflammation and scarring from sclerotherapy) .
• Radiofrequency ablation relief of ulceration tongue lesions or oral
mucosa.
• The CO2 laser symptomatic lesions of the airway or oral cavity,
( reduce bleeding and ulceration).
• Rapamycin or “sirolimus” possible oral medication
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Esophageal Atresia/Tracheoesophageal
Fistula.
Embryology.
Classification.
Incidence and epidemiology.
Clinical Findings, Diagnosis, and Pathophysiology.
Associated Anomalies.
Preoperative Management.
Surgical Technique.
Results, Complications, and Outcomes.
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Esophageal Atresia/Tracheoesophageal
Fistula.
Embryology
• Respiratory system
• Develops from the laryngotracheal groove on the ventral foregut
( gestational weeks 3 and 4 ).
• The groove develops into a diverticulum (outpouching) and
elongates to form the laryngotracheal tube.
• The developing respiratory system is partitioned off from the
esophagus by the tracheoesophageal septum.
• The proximal end of this tube becomes the larynx, the middle
becomes the trachea, and the distal end forms the lung buds.
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• Classification
The anatomy of the variants of esophageal atresia and tracheoesophageal fistula (TEF).
A: Proximal pouch with distal TEF (most common type occurring in 85% of patients; Gross type “C”).
B: Esophageal atresia without TEF (5%).
C: TEF without esophageal atresia (<5%).
D: Esophageal atresia with proximal and distal TEF (rare).
E: Proximal TEF and distal pouch(rare).
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Incidences and Epidemiology
• 1:2, 500 to 1:4, 500 live births.
•M>F
• Chromosomal anomalies, congenital heart disease, and other
anomalies are more common than expected.
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Clinical Findings, Diagnosis, and Pathophysiology
• Diagnosis
• History of choking with feeding , confirmed by the inability to pass an
NG tube into the stomach, Excessive drooling
• Past History of Maternal polyhydramnios should raise suspicion for
the diagnosis.
• Early (Risk of respiratory distress and aspiration).
• Confirmation
• Radiograph identifying a tube in a proximal pouch in most cases,
• Injection of air to outline the pouch will be necessary for confirmation .
• Presence of a scaphoid abdomen with no intragastric on radiograph taken
several hours after birth confirms the diagnosis of pure esophageal atresia.
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• Reflux of gastric contents through the fistula can enter the lungs
respiratory compromise
• In case of severe respiratory distress, gastrostomy or gastrostomy
plus division of fistula become an emergency procedure.
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Associated Anomalies
• Present in approximately one-half of the neonates with esophageal
atresia.
• Cardiovascular defects which occur in 17% to 50% of infants. ( M.C )
• Genitourinary,
• Other gastrointestinal,
• Skeletal neurologic,
• Craniofacial defects.
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Surgical Technique.
• The extrapleural plane is established at the fourth intercostal space and
developed sufficiently to allow control of the fistula and mobilization of
the proximal pouch.
• Initially, the fistula is divided and ligated followed by mobilization of the
proximal pouch.
• Anastomosis is performed in a single layer with an absorbable suture,
more recently using a monofilament.
• If needed there are various techniques for elongation and the esophagus
may be mobilized both proximally and distally.
• Myotomies on the upper pouch, either circular or spiral and flap
tubularization have been described.
• A transesophageal tube is often passed during the case and can be utilized
for feeding prior to performance of a postoperative esophagogram.
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• High-risk infants ( Severe respiratory failure (usually from
prematurity) and structural heart disease ) delayed repair.
• A decompressive gastrostomy, proximal pouch suctioning, and
parenteral nutrition are used until the infant is ready for operation.
• A staged repair that employs gastrostomy, surgical division of the
tracheoesophageal fistula, followed by esophageal anastomosis as a
second thoracic operation is rarely used except as a lifesaving
approach.
• Tracheoesophageal fistula with esophageal atresia H-type fistula.
• Repair is performed via a cervical approach after bronchoscopically stenting
the fistula with a small catheter.
• The catheter aids in dissection and care must be taken to stay in the plane of
the esophagus.
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Complications,
• Injury to the vagus nerves, recurrent laryngeal nerves, posterior trachea, and
adjacent vessels.
• Anastomotic leak occurs in approximately 10% to 20% of cases and most may
be treated nonoperatively.
• The presence of a leak prolongs hospitalization the retropleural tube is left in
place, the infant is not fed, and antibiotics are administered.
• Esophageal stricture is seen in up to 40% of infants after repair.
• Recurrent tracheoesophageal stricture is reported in up to 10% of patients.
• Gastroesophageal reflux is present in nearly all patients with esophageal
atresia.
• Esophageal dysmotility is present in the majority or patients and may be
responsible for dysphagia and recurrent respiratory problems.
• Tracheomalacia occurs in approximately 10% of patients and may be diagnosed
after the repair of esophageal atresia.
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Outcomes
• Mortality high for infants with severe congenital heart defects and
chromosomal anomalies.
• Late deaths may occur related to respiratory disease (tracheomalacia,
reactive airway disease, gastroesophageal reflux, and aspiration).
• Adult survivors of esophageal atresia repair have a normal quality of
life with the exception of morbidity from esophageal, functional, and
respiratory disorders.
• The very long-term effects are unknown but Barrett esophagus and
esophagitis have been reported
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Congenital Diaphragmatic Hernia.
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Embryology of the Diaphragm
• The diaphragm develops more superiorly than its postnatal
location but maintains its innervation from cervical roots C3,
C4, and C5.
• It is formed from four embryologic structures that fuse by
week 7 :
■ The septum transversum is formed by mesodermal tissue that
projects from the ventral body wall to partially separate the thoracic
cavity and abdominal cavity. In the adult, the septum transversum
forms the central tendon of the diaphragm.
■ The pleuroperitoneal folds extend from the dorsolateral sides of the
body wall to form the pleuroperitoneal membranes, which then fuse
with the septum transversum.
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The body wall also extends from the dorsal and lateral sides (after
the pleuroperitoneal folds have closed the thoracic cavity) to form
the peripheral, muscular portion of the adult diaphragm.
■ The dorsal mesentery of the esophagus forms the portion that is
dorsal to the esophagus and ventral to the aorta.
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• Pathology and Pathophysiology
• 1:3,000 to 2:4,000 live births.
• Most of the deaths other fatal anomalies.
• Defects are more common on the left side
• The cause of CDH is unknown ( combination of intrinsic
predisposition (genetic factors) and environmental factors (teratogen
or deficiency) ) abnormal diaphragm and lung development.
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• During the early development of the diaphragm, the midgut is largely
extracoelomic.
• Failure of closure of pleuroperitoneal canal the abdominal viscera
herniate through the lumbocostal trigone into the ipsilateral thoracic cavity.
prevents its normal counterclockwise rotation and fixation.
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• Left-sided CDH is characterized by a 2- to 4-cm posterolateral defect
in the diaphragm through which the abdominal viscera have
translocated into the ipsilateral thoracic cavity.
• On a right-sided defect, the large right lobe of the liver can occupy
most of the hemithorax, The hepatic veins may drain ectopically into
the right atrium, and the liver and lung may be fused.
• Pulmonary parenchymal compression by the herniated organs
growth and maturation of the lung.
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Diagnosis
• Prenatal ultrasound examination (66%) .
• Polyhydramnios is common.
• Prenatal MRI is utilized for more accurate delineation of the defect.
• Presentation cyanosis and respiratory distress shortly after birth in
severely affected neonates.
• On physical examination scaphoid abdomen.
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• Echocardiography ( Evaluate for degree of pulmonary
hypertension and to determine presence of cardiac defects).
• Differential diagnosis
• Eventration of the diaphragm,
• Anterior diaphragmatic hernia (Morgagni),
• Congenital pulmonary malformations,
• Unilateral pulmonary effusion, and primary agenesis of
the lung.
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Prognostic Factors
• The lung to head ratio (LHR) by multiplying the simultaneous
sonographic measurements of the size if the contralateral lung in an
anteroposterior and lateromedial direction, and dividing by the head
circumference.
• Ratios less than 1 have been associated with poor outcome.
• Liver above the diaphragm has also been used to determine outcome with
decreased survival with liver-up.
• The combination of a low LHR with liver-up has a poor outcome.
• The size of the defect was a significant factor in survival,
• A major cardiac anomaly and 1-minute Apgar sores of ≤4 were associated
with increased mortality.
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Treatment
• Referral to an appropriate tertiary care perinatal center with the
availability of the full array of respiratory care strategies.
• Resuscitation with standard neonatal resuscitation guidelines
• Endotracheal intubation and nasogastric tube insertion.
• Careful management of temperature regulation, glucose
homeostasis, and volume status should be performed.
• Fluid resuscitation should be performed with crystalloid, blood
products, and colloid.
• Inotropes such as dopamine or dobutamine are utilized if needed.
• ventilatory methods,
• ECMO (when ventilator therapy has failed) .
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Surgery
• Semielective
• Repair
• subcostal incision or thoracotomy incision. Both
thoracoscopic and laparoscopic.
• The abdominal organs are returned to the abdomen.
• The defect in the diaphragm is repaired.
• Primary repair with nonabsorbale suture is preferred.
• If the defect cannot be repaired primarily due to size, a
prosthetic patch such as Goretex has been utilized.
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Outcome
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Foramen of Morgagni Hernia
• The anterior CDH rare ( 3% to 5% of all CDHs).
• Diagnosed during childhood.
• By chest or abdominal radiographs being performed for presumed
pneumonia or gastrointestinal symptoms
• This is an anteromedial hernia at the junction of the septum transversum
and the thoracic wall with contents being seen in a retrosternal position.
• There is a hernia sac present and the sac may contain colon, small intestine,
or liver.
• The hernias are repaired due to the risk of segmental intestinal volvulus or
obstruction.
• Repair may be performed through a laparoscopic approach. The sac is
excised and the diaphragm is sutured to the undersurface of the posterior
rectus sheath at the costal margin.
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Eventration of the Diaphragm
• Diagnosed The abnormal elevation of one or both
hemidiaphragms .
• Two Types congenital ( Infection ).
• Acquired injury to the phrenic nerve.
• Surgery symptomatic cases after several months of observation
• The surgical management is with plication of the diaphragm with
nonabsorbale sutures.
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Conclusion.
• Infants are obligate nasal breathers for the first 3 to 6 months of life;
therefore, any nasal obstruction can cause respiratory distress.
• The site of the neck mass is important (midline vs. lateral) for
narrowing the differential diagnoses.
• Benign cervical lymphadenopathy is the most common neck mass in
the pediatric population.
• Although rare, always consider malignancy to avoid delay in
diagnosis.
• History is important in the work up and treatment of an airway
foreign body
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Conclusion.
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References.
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• THANK YOU
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