Metastatic Lung Tumors

7 Resection of metastases in the pediatric population has been proven to be beneficial for pediatric

cancers. Eradication of the primary tumor and absence of other metastases are necessary for

improvement in survival.58 Resection is considered most frequently for osteogenic sarcoma, soft tissue

sarcoma, and Wilms tumor.58,59

Osteogenic Sarcoma

There is evidence that resection of metastases in osteogenic sarcoma improves survival if a complete

resection can be performed. The factors which improve survival are ipsilateral involvement, smaller

tumor size, and longer disease-free survival between diagnosis and occurrence of metastasis.59

Metastases which are more peripheral have a better outcome compared with centrally located

metastases.60 In addition to metastectomy chemotherapy is required for elimination of micrometastatic

disease. Metastases become calcified and therefore are palpable aiding with resection. There are some

data that the outcomes are comparable between initial resection of metastases and resection of a second

recurrence.59 Improved survival is seen with tumor necrosis greater than 98% and disease-free interval

of greater than 1 year. Mean survival after complete resection with aggressive resection is 33.6 months

while mean survival without resection is 10.1 months.61 Thoracoscopy is felt to be inappropriate for this

disease process since CT scans underestimate the number of lesions which can be palpated at

thoracotomy.62 Bilateral lesions can be identified even when CT scans demonstrate only unilateral

lesions leading some authors to recommend prophylactic contralateral thoracotomy. With bilateral

involvement, in an appropriate population, simultaneous bilateral thoracotomy may lessen morbidity

compared to the traditional staged thoracotomies.63

Figure 102-7. Enterogenous cyst. This represents another abnormality in the spectrum of bronchopulmonary foregut

malformations. A: Computerized tomography demonstrating a large mass occupying the right hemithorax. B: Surgical specimen.

Soft Tissue Sarcomas

Nonrhabdomyosarcomas such as synovial sarcoma respond poorly to chemotherapy and radiation

therapy. In these tumors, complete resection of pulmonary metastases, but not debulking improves

survival.64 Lung metastasis in rhabdomyosarcoma responded to chemotherapeutic regimens but local

treatment of the metastases did not improve survival.65 Ewing sarcoma has a high rate of pulmonary

metastases which have traditionally been treated with radiation therapy. More recent data suggest that

metastectomy after irradiation leads to improved survival.66 Delayed surgery after neoadjuvant therapy

with resection of the chest wall lesion leads to decreased extent of excision and a higher rate of negative

margins in tumors less than 8 cm.67

Wilms Tumor

Pulmonary metastectomy in Wilms tumor is only indicated for persistent lesions after a 27-week

regimen of chemotherapy and radiation therapy.68 Thoracoscopy with resection of lesions is indicated

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for diagnostic purposes. The outcomes demonstrate that there is improved 5-year survival for patients

with CT-only metastases and that lung radiation in this group does not improve survival.69

Primary Lung Tumors

Pediatric primary tumors are rare with comprehensive series documenting approximately 383 patients

with primary malignant pulmonary neoplasm as of 1993 (Table 102-1). The majority of primary

neoplasms are malignant but benign lesions also have significant morbidity due to location of the

lesions. Operative exploration with total resection of all involved tissues is recommended.56

Bronchial Adenomas

Bronchial adenomas are the most common of the malignant lung lesions (40%) in the pediatric

population and include several different histologies including carcinoid tumor, mucoepidermoid

carcinoma, and adenoid cystic carcinoma. Carcinoid is the most common malignant pulmonary lesion in

the pediatric population and accounts for 85% of bronchial adenomas. Mucoepidermoid carcinomas

account for 10% of the adenomas and originate from mucous glands in the submucosal and respiratory

mucosa. They are centrally located. Adenoid cystic carcinoma comprises 5% of the bronchial adenomas

and is described as a salivary gland-type tumor. Cough, pneumonitis, fever, respiratory distress, and

hemoptysis are the most common presenting symptoms. Delay in diagnosis is common due to the

nonspecific symptomatology. The disease is diagnosed with chest radiograph followed by CT scan.

Bronchoscopy is required for diagnosis and biopsy can be performed but there is a high risk of

hemorrhage. Treatment is by surgical resection which can include lobectomy, bilobectomy,

pneumonectomy, or sleeve resection with regional lymphadenectomy. Intraoperative frozen section is

useful to assure complete resection and to limit lung resection. Bronchial carcinoids do not usually

present with carcinoid syndrome and presentation with the syndrome is a sign of extrapulmonary

disease.56

Table 102-1 Primary Pulmonary Neoplasms in Children

Bronchogenic Carcinoma

Bronchogenic carcinoma is the second most common malignant pulmonary cancer. Bronchogenic

carcinoma consists of adenocarcinoma and undifferentiated carcinomas in the pediatric population.

These tumors have been associated with CPAMs but have been reported in normal lung as well.56,70

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Human papilloma virus has been associated with the squamous cell variant. Resection with ipsilateral

lymphadenectomy is the treatment of choice. Prognosis for bronchogenic carcinoma is poor as the

presentation tends to be with advanced stages. Symptoms are related to metastases with bone pain or

tumor burden with weight loss.56

Pleuropulmonary Blastoma

Pleuropulmonary blastoma is the third most frequent lung tumor in children and is most common in

children under 4 years of age.56,71,72 The lesion consists of mesenchymal elements and embryonal

stroma without epithelial neoplasia. The tumor is known to be part of the spectrum of tumors related to

the mutation of the DICER1 gene.71 The tumor usually presents peripherally. These tumors are

associated with CPAMs. There are three types; type I consisting of multilocular cysts, type II consisting

of a combination of cystic and solid components, and type III having proliferating mesenchyme without

cysts.72 Treatment is with resection and adjuvant chemotherapy. Survival in type I has been reported at

91% while type II/III have survivals of 43%.71

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Figure 102-8. Lung neoplasm in an 8-year-old child: inflammatory pseudotumor. A: Presenting chest radiograph with right lung

collapse. B: Biopsy of an obstructing endobronchial mass demonstrates inflammatory pseudotumor (inflammatory myofibroblastic

tumor). C: Computerized tomographic “virtual bronchoscopy” image after initial biopsy demonstrates deformation of the right

main stem bronchus and carina due to tumor infiltration. D: Computerized tomography reconstruction of the external appearance

of airways demonstrating the deformation of the carina and right main stem bronchus. The child underwent excision of the right

main stem bronchus and carina and tracheobronchial reconstruction. E: Postoperative transverse thoracic computerized

tomography at the level of the reconstruction of the carina.

Other Malignant Tumors

Sarcomas, including fibrosarcoma, rhabdomyosarcoma, and leiomyosarcoma are the next most common

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primary malignant lung neoplasm in children. They may arise endobronchially or peripherally. These

have been reported to arise in documented cystic disease.73 Inflammatory pseudotumor is considered to

be the most common “benign” tumor in children. The tumor is a myofibroblastic spindle cell soft tissue

tumor with infiltrative plasma cells, lymphocytes, and eosinophils. Recently these have been classified

as intermediary lesions with a molecular rearrangement involving the tyrosine kinase receptor

anaplastic lymphoma kinase. Symptoms are nonspecific such as fever, chest pain, cough, and recurrent

pneumonias.74 These may present as an endobronchial lesion but are more often peripherally located.

The lesion may invade local structures and be aggressive locally and may recur locally but spontaneous

regression has been reported. Complete resection is recommended as no adjuvant regimen has gained

widespread acceptance. Nonsteroidal anti-inflammatory agents have been utilized with some success

(Fig. 102-8).

Hamartoma

Harmartomas are lung nodules composed of cartilage and gland-like formations in an irregular and

haphazard orientation. They grow slowly and are the most common benign lesion in adults but the

second most common in children.56 Radiographically, they have a “popcorn” calcification in 10% to 25%

of cases. The majority are located in the periphery of the lung. Conservative surgical resection is

recommended. These may be associated with gastric smooth muscle tumors and extra-adrenal

paragangliomas (Carney triad) in adolescent females; and in this age group, a workup should be

performed to exclude these diagnoses.

Mediastinal Tumors

Mediastinal tumors in the pediatric population can be benign or malignant with 37% of lesions being

malignant in children.53 Tumors have a predilection for arising from the different components of the

mediastinum. Neurogenic tumors are the most common mediastinal tumors in the pediatric

population.54 These tumors arise in the posterior mediastinum from the sympathetic ganglia, the

intercostal nerves, and paraganglia cells.53 These may grow very large before detection and may

manifest with Horner syndrome or opsoclonus–myoclonus syndrome. The neurogenic tumors may be

malignant (neuroblastoma, ganglioneuroblastoma) or benign (schwannoma, ganglioneuroma, or

neurofibroma).54,75,76 Neuroblastoma originating in the mediastinum has a better prognosis than in

other sites. These tumors have more favorable biology and lower rates of N-myc amplification as well

as improved rates of event-free and overall survival.77 Diagnosis is by CT and MRI with MRI allowing

improved assessment of extension into the spinal foramina.78,79 Tumors may be resected by a

thoracoscopic approach or via thoracotomy Figure 102-9.80,81

Neurofibromas and schwannomas arise from intercostal nerves of sympathetic nerves. The

schwannomas are encapsulated without nerve fibers within the tissue. Neurofibromas are

unencapsulated, have nerve fibers running through the mass and are common in patients with

neurofibromatosis.82 Isolated tumors may be removed but tumors associated with neurofibromatosis

tend to extend over nerve sheaths precluding complete removal. These are associated with malignant

degeneration.

Lymphomas arise in the anterior and middle mediastinum and are the most common anterior

mediastinal mass in children.79 These are usually inhomogeneous and resection is not required. Both

Hodgkin and non–Hodgkin lymphoma arise in the mediastinum. Diagnostic workup involves biopsy for

tissue diagnosis as the diagnosis often requires tissue subtyping.54 Due to risk of airway compromise,

the most accessible tissue, such as supraclavicular lymph nodes, should be biopsied and special care is

required for biopsy of the mediastinum including partial upright positioning, spontaneous ventilation,

availability of rigid bronchoscope, and avoidance of muscle relaxants. Risk of airway compromise is

increased in patients with greater than 50% reduction in tracheal cross-sectional area, or those with

dyspnea and orthopnea. Treatment is with chemotherapy.

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Figure 102-9. A: Large neural tumor in coronal view CT scan with calcifications (neuroblastoma). B: Same mass on transverse

view.

Teratomas are the most common mediastinal germ cell tumor and are composed of all three germinal

cell layers.53,79 These lesions are benign, usually cystic and encapsulated. They frequently contain

calcification on radiography (Fig. 102-10). Most are asymptomatic but may present with respiratory

distress. Presenting symptoms can include stridor, tachypnea, and severe respiratory distress especially

when lying supine.82 The cysts frequently contain mature ectodermal elements such as hair, skin, and

neuroglial elements and may contain bone, teeth, and fat. The tumors may be large and tend to displace

rather than invade surrounding tissue.79 Workup includes preoperative measurement of α-fetoprotein

and β-HCG. Resection is the treatment of choice and confirms the diagnosis. Treatment of mature

teratomas is resection. Immature teratomas which contain malignant elements are treated with

chemotherapy and radiation in addition to surgery.

Figure 102-10. Mediastinal teratoma. A: Transverse thoracic computerized tomography of a large anterior mediastinal teratoma

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presenting with airway compression symptoms in a 9-month-old female. B: Tumor removal via a median sternotomy.

Other mediastinal tumors are very rare but often require surgical biopsy or extirpation. These include

neurolemmoma, pheochromocytoma germ cell tumors, rhabdomyosarcomas, and thymic tumors.

Chest Wall Tumors

Ewing sarcoma, chondrosarcoma, and liposarcoma are malignant tumors which may arise from the chest

wall. Local resection may require extensive chest wall excision and prosthetic reconstruction.83

Primitive neurectodermal tumors are malignant small cell tumors carrying a poor prognosis.

Improvement in survival is accomplished with multimodal therapy which includes chemotherapy,

radiation, and surgery with aggressive management of pulmonary metastases.66,67,83

Respiratory Papillomatosis

Recurrent respiratory papillomatosis is caused by neoplastic proliferation associated with acquisition of

papillomavirus during delivery. It is usually confined to the larynx and upper tracheal airway with

spread in less than 1% of cases. Treatment is with laser ablation and may require multiple treatments.

Prognosis is poor due to the recurrence of the disease.78

ESOPHAGEAL ATRESIA/TRACHEOESOPHAGEAL FISTULA

8 The first description of the classic proximal esophageal atresia with distal fistula was by Thomas

Gibson in 1697. The first description of esophageal atresia was by Durston in 1670 who found a blind

esophageal pouch in one pair of thoracopagus conjoined twins.84 Initial attempts at repair or palliation

were unsuccessful. Leven and Ladd reported survivors but only after staged reconstruction. The first

successful primary repair was reported by Dr. Cameron Haight in 1941.85,86 Outcomes have improved

related to refinements in surgical technique and postoperative care.84,87–91 In the modern era, survival is

related to the degree of prematurity and the presence of cardiac disease.

Embryology

The development of the human respiratory system begins as a folding of the flat endodermal layer into

the primitive gut tube. The respiratory system is derived from the foregut endoderm with the

appearance of the laryngo–tracheal groove in the ventral floor just caudal to the level of the pharynx.

Despite intense study, the precise events in morphogenesis are not well understood. There are two

theories, one of which suggests that the respiratory system develops as a rapid outgrowth from the

original foregut tube. This theory is consistent with the accepted pattern of rapid longitudinal growth at

the distal ends of the bronchopulmonary structures. Other theories support the fusion of the lateral

foregut walls resulting in the separation of the trachea from the esophagus.92,93 The third theory based

on studies of chick embryos supports the development of three folds, anterocranial (laryngeal), dorsal

(pharyngoesophageal), and inferior (tracheoesophageal). The paired laryngeal and single

tracheoesophageal folds appear first defining the tracheoesophageal space. Subsequently continued

movement of the folds results in the definition of the separate trachea and esophagus. At the same time

the pharyngoesophageal fold defines the boundary of the pharynx and esophagus.

One theory of the development of tracheoesophageal fistula is caused by failure of tracheal growth. A

second theory is that there is failure of the active separation process. Another theory is the abnormal

movement of the pharyngoesophageal fold leading to failure of division. Sonic hedgehog has been

implicated in the development of esophageal atresia/tracheoesophageal fistula based on adriamycininduced malfor-mations in rats.

Classification

Descriptive terminology is used to indicate the anatomic configuration of a patient with esophageal

atresia. The most common type of lesion is a proximal atresia with a distal tracheoesophageal fistula

which accounts for 76% to 85% of all cases in recent series.87,88,90,91,94 Esophageal atresia without

fistula occurs in approximately 3% to 17% and TEF without fistula in 4% to 6% of cases. Proximal

fistula and both proximal and distal fistula occur in approximately 1% of cases each. Initially alphabetic

classification schemes were devised and the most common variety of esophageal

atresia/tracheoesophageal fistula is still commonly referred to as “Gross type C” (Fig. 102-11).

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Incidence and Epidemiology

Esophageal atresia is relatively common occurring in 1:2,500 to 1:4,500 live births. There is a slight

preponderance of males.84,91,94 Chromosomal anomalies, congenital heart disease, and other anomalies

are more common than expected.

Figure 102-11. 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).

Clinical Findings, Diagnosis, and Pathophysiology

Diagnosis of esophageal atresia is suspected with a history of choking with feeding and is confirmed by

the inability to pass an NG tube into the stomach. Excessive drooling may be noted. Maternal

polyhydramnios should raise suspicion for the diagnosis. If not diagnosed early, respiratory distress and

aspiration may occur. Confirmation of the diagnosis is with radiograph identifying a tube in a proximal

pouch in most cases. Occasionally 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.96 Reflux of gastric contents through the

fistula can enter the lungs which becomes more problematic in the face of respiratory compromise and

is less of an issue with spontaneous ventilation. In cases of severe respiratory distress, gastrostomy or

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gastrostomy plus division of fistula become an emergency procedure.

Associated Anomalies

Associated anomalies are present in approximately one-half of the neonates with esophageal atresia. The

most common associated anomalies are cardiovascular defects which occur in 17% to 50% of infants.

Genitourinary, other gastrointestinal, skeletal neurologic, and craniofacial defects are also found. The

occurrence of vertebral anomalies, anorectal malformations, cardiac, tracheoesophageal renal, and limb

(usually radial) anomalies is known as the VACTERL association. Two or more of the defects must occur

to be denoted as VACTERL and this occurs in 10% to 21% of cases.

Preoperative Management

Attempts at feeding are discontinued and sump tube is placed in the upper esophageal pouch.

Precautions such as head-up position are advocated to decrease reflux via the fistula into the lung.

Intubation is avoided as it increases the risk of reflux into the lungs via the fistula. Ventilatory support

is more likely to be needed for low birth weight or prematurity. Preoperative antibiotics are given in

the majority of patients.97 Emergent bedside gastric decompression may be necessary before definitive

surgery. If gastrostomy decompression does not improve ventilation, emergency operative ligation or

placing an occluding distal esophageal balloon may allow for ventilation.98

In addition to chest radiograph, echocardiograpy is performed to determine the side of the aortic arch

as well as to evaluate for major cardiac anomalies. In patients with a right-sided arch, the operative

approach is modified.99 Radiographs of the spine are also commonly performed.

In an attempt to stratify infants into groups to determine outcomes, Waterston developed criteria

based on birthweight, pneumonia, and associated anomalies. With improvements in neonatal critical

care, the criteria have been revised to with the current Spitz classification which uses a 1,500 g weight

as the cutoff, with or without major cardiac disease. Currently survival for greater than 1,500 g infants

without cardiac disease is approximately 97%, survival for either cardiac disease or less than 1,500 g

approaches 82% and for both birth weight less than 1,500 g and cardiac anomaly survival is

approximately 50%.90

Surgical Technique

In most patients, gastrostomy is unnecessary and the technique is a posterolateral right extrapleural

thoracotomy (Fig. 102-12). There is no need to divide the serratus or the latissimus muscles. A more

posterior approach through the auscultatory triangle affords excellent exposure. 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.100

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Figure 102-12. A: Schematic illustration of esophageal atresia with distal tracheoesophageal fistula. B: The initial management of

this anomaly includes upright or prone posture, sump suction of the blind esophageal pouch. C: The right extrapleural operative

approach. After division and closure of the fistula, primary esophagoesophagostomy is performed.

High-risk infants who cannot safely undergo surgical correction of their esophageal abnormality may

be treated with delayed repair. A decompressive gastrostomy, proximal pouch suctioning, and

parenteral nutrition are used until the infant is ready for operation. Severe respiratory failure (usually

from prematurity) and structural heart disease are the most common reasons to delay esophageal

surgery. 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.

In the case of pure esophageal atresia without fistula, a prolonged period before definitive repair may

be used to improve the likelihood of completing a primary repair. A feeding gastrostomy is the initial

operative management and may be technically challenging due to microgastria. Using bouginage the

ends of the esophagus are elongated and once the ends are close, a thoracotomy is performed to repair

the esophagus (Fig. 102-13). Other methods of elongation have been described with staged repair using

traction sutures in the upper and lower esophagus (Fig. 102-14). Daily tensioning results in lengthening

of the esophagus over a few weeks.95 Alternatively, extrathoracic elongation may be utilized.101,102

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Figure 102-13. Patient with pure esophageal atresia treated initially with feeding gastrostomy, and proximal pouch stretching. This

“gap-o-gram” demonstrates close approximation of a dilator in the proximal pouch and a probe placed in the distal esophagus via

the gastrostomy. The child subsequently underwent primary repair of the esophagus.

Figure 102-14. In patients with ultra-long-gap esophageal atresia, traction sutures are placed on the upper pouch and lower

segment. Tension is increased daily until the segments lengthen, allowing primary repair.

Esophageal replacement is reserved for the worst cases of long-gap atresia, pure atresia or failed

initial operations. The esophagus may be replaced later using stomach, gastric tube, or colon. These

cases may be temporized with spit fistula and sham feeds.

Tracheoesophageal fistula with esophageal atresia is referred to as H-type fistula. The orientation is

such that the opening into the trachea is always proximal to the esophageal entry site. 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.

Thoracoscopic approaches to the repair of esophageal atresia have been described with results

demonstrating equivalent outcomes to open repair.103,104 Thoracoscopic techniques for esophageal

lengthening in long-gap atresia have also been described.105

Results, Complications, and Outcomes

Technical mishaps may occur during the procedure when anatomy is misidentified or dissection is

carried out improperly. Injury to the vagus nerves, recurrent laryngeal nerves, posterior trachea, and

adjacent vessels must be avoided.

Anastomotic leak occurs in approximately 10% to 20% of cases and most may be treated

nonoperatively. The presence of a leak prolongs hospitalization.106 When detected, the retropleural tube

is left in place, the infant is not fed, and antibiotics are administered. Repeat contrast radiographs are

employed to document healing prior to initiation of oral feeds.

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Esophageal stricture is seen in up to 40% of infants after repair. These usually respond to a few

sessions of dilation performed in the operating room with endoscopic and fluoroscopic assistance as

necessary to assure the safe traverse of dilators or placement of balloon dilators.107 More recalcitrant

strictures require more frequent dilation sessions, medical and/or surgical control of gastroesophageal

reflux, and occasionally reoperation with stricture resection and reanastomosis.

Recurrent tracheoesophageal stricture is reported in up to 10% of patients. These may respond to

endoscopic ablation, a construct placed into the fistula or may require operative division placing tissue

between the esophagus and trachea to separate the suture lines.108

Gastroesophageal reflux is present in nearly all patients with esophageal atresia but is asymptomatic

in many. When symptomatic, it may lead to respiratory symptoms and stricture formation. Medical

management with thickened feeds, acid reduction, and prokinetics is the first line of therapy. Refractory

reflux may require surgical control of reflux.

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. On bronchoscopy, the trachea appears oval in shape rather than round and the

anterior and posterior walls coapt during the respiratory cycle. The trachea appears to fish-mouth in the

anteroposterior dimensions during spontaneous expiration. Patients may develop tracheomalacia “fits”

when agitated and the condition may deteriorate with complete inspiratory block and unconsciousness.

Intubation and resuscitation may be required. The symptoms are completely relieved by intubation.

Patients with less severe symptoms may improve over time as the trachea grows. Severe symptoms

require either tracheostomy or a tracheal suspension with an aortopexy under bronchoscopic guidance.

It may be difficult to determine whether the symptoms are from gastroesophageal reflux or

tracheomalacia and sometimes both an antireflux procedure and a tracheomalacia procedure may be

required.109

Long-term results have improved and most children can look forward to normal existence. Although

growth may be slow in the first 5 years of life, by 20 years of age less than one-third of the children

have heights or weights below the 25th percentile.110 Mortality remains 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.111 The very long-term effects are unknown but

Barrett esophagus and esophagitis have been reported112 but the incidence is low and further studies are

required to determine the need for long-term surveillance.

OTHER CONGENITAL ANOMALIES OF THE TRACHEA AND

ESOPHAGUS

Laryngotracheoesophageal Cleft

Laryngotracheoesophageal clefts are rare anomalies resulting from the failure of normal separation of

the esophagus and trachea. These are the most severe anomalies and the presence of the common

aerodigestive channel plus the association of gastroesophageal reflux leads to aspiration with

respiratory damage and a high mortality rate. The lesions are classified by the length of the common

channel. Type I clefts involve the supraglottic larynx. Type II defects extend beyond the carina to the

cervical trachea. Type III clefts involve the entire trachea and type IV defects extend onto one or both

bronchi. Type III and IV clefts are frequently associated with other anomalies such as esophageal

atresia, microgastria, and congenital heart disease.

Type I clefts may be repaired via an endoscopic or translaryngeal approach and do not require

tracheostomy. Type II clefts require cervical exposure either laterally or via the anterior wall of the

larynx and trachea. The anterior approach provides good exposure and decreases the risk of damage to

neurovascular structures. The approach to type III and IV defects is via a right thoracotomy and a right

neck incision. However reports of midline exposure have been described. The common channel is

divided and repaired with interrupted sutures with tracheostomy.113 Oromotor function and chronic

aspiration are problems and most children require gastrostomy and many require operative control of

gastroesophageal reflux.

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