Metabolic Disorders

Metabolic Disorders

Figure 1.21. The same infant showing the Pierre Robin sequence: micrognathia, macroglossia, a protruding tongue, and a cleft palate which is often posterior. Cyanosis is often due to the tongue falling back and obstructing the posterior oropharynx. The infant should be managed in a prone position to avoid breathing problems. Micrognathia improves significantly over time.

Figure 1.20. Mandibular hypoplasia, a small underdeveloped mandible, can be an isolated finding or part of a sequence such as Pierre Robin, Treacher-Collins, Hallermann Streiff, or trisomy 18. This may cause severe respiratory distress.

Figure 1.21. The same infant showing the Pierre Robin sequence: micrognathia, macroglossia, a protruding tongue, and a cleft palate which is often posterior. Cyanosis is often due to the tongue falling back and obstructing the posterior oropharynx. The infant should be managed in a prone position to avoid breathing problems. Micrognathia improves significantly over time.

Figure 1.22. A lateral radiograph of the head and neck showing mandibular hypoplasia in an infant with trisomy 18. This caused severe respiratory distress because the hypoplastic mandible is associated with narrowing of the nasopharyngeal air passages. (Singleton, E., Wagner, M.)

Figure 1.23. Congenital goiter is most commonly seen in an infant when there is a history of maternal ingestion of goitrogens such as antithyroid medications or iodides. Goiters can occur endemically in areas with insufficient maternal dietary iodine intake. This neck mass is symmetric about the midline of the neck.

Figure 1.24. A radiograph of the neck in another infant who developed a very large congenital goiter as a result of maternal use of an iodide-containing medication. It compromised the airway, causing severe respiratory distress.

Goiter Extending Heart

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Cystic Hygroma Face

Figure 1.25. Cystic hygromas are typically benign unilateral masses in the lateral neck and are lymphatic in origin. They characteristically involve the face and upper trunk. They can occur as an isolated finding or may be associated with Turner's syndrome. The cervical variety can extend into the anterior and middle mediastinum, thus producing respiratory distress.

Figure 1.25. Cystic hygromas are typically benign unilateral masses in the lateral neck and are lymphatic in origin. They characteristically involve the face and upper trunk. They can occur as an isolated finding or may be associated with Turner's syndrome. The cervical variety can extend into the anterior and middle mediastinum, thus producing respiratory distress.

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Cystic Hygroma Transillumination

Figure 1.26. Transillumination of the cystic hygroma in the same infant as in Figure 1.25 reveals that die hygroma is filled with clear lymphatic fluid.

Figure 1.26. Transillumination of the cystic hygroma in the same infant as in Figure 1.25 reveals that die hygroma is filled with clear lymphatic fluid.

Figure 1.27. A branchial cleft cyst is a condition which arises from a persistent cervical sinus or second branchial groove. It is always located along the sternocleidomastoid muscle and is generally painless unless infected. If large, it can cause respiratory distress.

Figure 1.28. Radiograph of a branchiogenic cyst which is a product of ectopic branchial epithelium. It can be air- or fluid-filled. Enlarging cysts with mucinous secretions can lead to airway compromise. Bronchogenic cysts are most commonly located near the carina.

Figure 1.29. This anteroposterior radiograph of a normal chest shows the heart on the left, and complete aeration of the lungs with the diaphragm at the level of the 8th intercostal space. The diaphragm of the normal infant is usually rounded smoothly on both frontal and lateral radiographs, and the anterior costophrenic angle is usually quite shallow. The trachea is displaced slightly to the right of the midline by the left aortic arch.

Degree Angle Lung

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Figure 1.30. A lateral radiograph of a normal chest in an infant showing that the anteroposterior diameter is approximately the same as the thoracic diameter. Note the rounded diaphragm.

Large Thymic ShadowLarge Thymic Shadow

Figure 1.31. Normal chest radiographs of the same infant, showing an inspiratory view on the left and an expiratory view on the right. There can be dramatic differences in chest shape, cardiac and thymic shadow location, and degree of lung expansion, depending on the timing of the exposure. Incomplete aeration of the lungs may simulate pneumonia, and the thymus may appear large. The caliber of the trachea varies with respiration.

Figure 1.31. Normal chest radiographs of the same infant, showing an inspiratory view on the left and an expiratory view on the right. There can be dramatic differences in chest shape, cardiac and thymic shadow location, and degree of lung expansion, depending on the timing of the exposure. Incomplete aeration of the lungs may simulate pneumonia, and the thymus may appear large. The caliber of the trachea varies with respiration.

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Thymus Autopsy

Figure 1.32. Chest radiographs of the same infant as in Figure 1.31 taken in sequence show a skin fold. Note the linear nature of this shadow across the right lung and diaphragm in the view on the left and the normal appearance of the chest in the view on the right. Skin folds should be recognized as normal artifacts and should not be mistaken for pathologic conditions.

Figure 1.32. Chest radiographs of the same infant as in Figure 1.31 taken in sequence show a skin fold. Note the linear nature of this shadow across the right lung and diaphragm in the view on the left and the normal appearance of the chest in the view on the right. Skin folds should be recognized as normal artifacts and should not be mistaken for pathologic conditions.

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Skinfold Artifact

Figure 1.33. This is another example of a skin fold seen on a radiograph of the right chest. Despite the abnormal appearance of the radiograph, the infant was asymptomatic.

Figure 1.34. The perfect roundness and symmetry of the radiolucent defect in the right hemithorax and diaphragm in this radiograph suggest that this is an artifact created by a hole in the top of the incubator. This is not an uncommon finding when the radiograph is taken through the top of the incubator. (Singleton, E., Wagner, M.)

Holes Top Neonatal Incubators

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Figure 1.35. This is another example of a chest radiograph showing die "hole" in the incubator. This artifact lies directly over the mediastinum and should not be considered padiologic.

Figure 1.36. In this chest radiograph there is a symmetric round shadow overlying the mediastinum. This is an artifact caused by a pacifier lying directly on the infant's chest.

Figure 1.35. This is another example of a chest radiograph showing die "hole" in the incubator. This artifact lies directly over the mediastinum and should not be considered padiologic.

Ectodermal Dysplasia Baby

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Normal Chest Radiographs Infant
Figure 1.37. Chest radiograph of this infant shows a normal chest, but the scapulae are abnormal and diere is amelia of the upper extremities bilaterally. This illustrates the importance of evaluating all osseous structures in any radiograph.

Figure 1.38. The lack of enamel and tooth buds is noted in this lateral radiograph. This infant had a chest radiograph taken for mild respiratory distress, and when the lack of tooth buds was noted, the diagnosis of ectodermal dysplasia was suspected and later confirmed.

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Ectodermal Dysplasia

Figure 1.39. A chest radiograph was taken of this infant with Poland's anomaly to check for any abnormalities of the ribs. Note die increased lu-cency of the right upper part of the chest and loss of soft tissue shadow caused by the absence of the sternocostal head of die pectoralis muscle. Also note how clearly the scapula can be seen on the right side, also because of the absence of the pectoralis muscle.

Figure 1.40. A small, bell-shaped thorax is seen in this infant widi severe lung hypoplasia. After attempted resuscitation, air leaked into die thoracic and mediastinal spaces. Elevation of the lobes of the thymus created a "butterfly wing" appearance caused by mediastinal air. The abdomen is gasless and the opaque area to the right of the midline above the brim of the pelvis is the umbilical cord. The lung hypoplasia, confirmed at autopsy, occurred as a result of renal agenesis and oligohydramnios.

Difficulty in initiating respiration with minimal chest wall excursion, decreased lung expansion, decreased or absent air entry on auscultation, and persistent cyanosis should alert one to the possibility of lung hypoplasia.

Excursion Lungs
Figure 1.41. The lungs usually comprise 2% of total body weight in infants. This abdominal pregnancy resulted in severe oligohydramnios and severe hypoplasia of the lungs. The lungs weighed only 1% of total body weight in this infant.
Ectopic Lungs Infant

Figure 1.42. Radiograph of the head and neck in an infant with tracheal agenesis. An endotracheal tube could not be passed below the proximal tracheal obstruction. This lethal condition occurs when there is unequal division of the foregut between the esophagus and trachea. Affected infants are usually live-born; they may gasp but cannot introduce air into the lungs. Polyhydramnios is common. The lung architecture below the obstruction is suprisingly normal, and the lungs are typically larger than predicted for age. There may be an associated broncho- or tracheoesophageal fistula. If this is present, the infant may survive for a few hours by exchanging air through the fistula that communicates with the esophagus. Note the tube in the esophagus.

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Responses

  • lucas
    How long is goiters medication?
    8 years ago
  • Antje
    What is hydrops assocted with cystic hygroma?
    8 years ago

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