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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_5770_Библиотеки_им_академика_М_И_Перельмана.pdf
188
J. Weidemann and G. H. A. Engelcke
Table 9.2 Differential diagnosis of solid lesions
Suprahyoid
Parotid space
Lymph node
Lymphofollicular proliferation (HIV)
Malignoma
Submandibular space
Lymph node
Infrahyoid
Midline
Thyroid nodule (adenoma, carcinoma)
Dermoid/epidermoid
Lateral
Lymph adenopathy
Teratoma
Lacking denition by the hyoid
Ectopic thymic tissue (occasionally found cranially to the
mediastinal thymus)
Reactive or malignant lymph node enlargement
Malignant tumors
In infants: neuroblastomas, lymphomas, rhabdomyosarcomas
In older children: mainly lymphomas and thyroid carcinomas
Fibromatosis colli
Table 9.3 Differential diagnosis of vascular anomalies
Vascular tumors
Benign (hemangioma)
Locally aggressive or borderline vascular tumors
(hemangioendothelioma, kaposi sarcoma, others)
Malignant vascular tumors (angiosarcoma, epithelioid
hemangioendothelioma, others)
Vascular malformations
Simple vascular malformations
Capillary malformations
Lymphatic malformations
Venous malformations
Arteriovenous malformations (high-ow lesion)
Arteriovenous stula (high-ow lesion)
Combined vascular malformations (dened as two or more
vascular malformations found in one lesion)
Of major named vessels (anomalies of origin, course, number,
length, diameter, communication, persistence of embryonal
vessel)
Associated with other anomalies (e.g., Sturge-Weber syndrome)
a
Classication according to the International Society for the Study of
Vascular Anomalies (ISSVA) [7]
Table 9.4 Differential diagnosis of lymph node lesions
Cystic
Viral and bacterial lymph node enlargement with presence of
purulent foci and stulas
Solid
Viral and bacterial lymph node enlargement without presence of
purulent foci and stulas
Lymphomas
Calcication
Atypical mycobacterial infections (Mycobacterium avium-
intracellulare- scrofulaceum [MAIS] complex)
Lymph node metastasis of thyroid carcinoma
a
Key Points
• Child-friendly examination conditions
• Standardized approach and documentation
• Assignment of pathology based on morphological components, anatomical region, and possible embryonic
genesis
• Possible syndromic connection
9.2 Suprahyoid Space
9.2.1 Parotid andSubmandibular Spaces
Inammatory Lesions oftheSalivary Glands
The most common diseases of the salivary glands are acute
inammations, which are often viral or, more rarely, bacte-
rial. Typical viral pathogens are mumps, mononucleosis, and
cytomegalovirus. The salivary glands are painfully swollen
and show an increased hypoechoic tissue pattern with
increased vascularization in color duplex.
Bacterial infections may occur in children, especially
involving the parotid gland. Most patients are children who
are preterm, less than 1year old, or immunosuppressed. The
infections are most likely the result of diverted inammation of the oral cavity or, rarely, of stone obstruction, especially for the submandibular gland. The most common
pathogen is Staphylococcus aureus. In addition to edematous changes, sonographic ndings can range from enlargements of intraglandular and extraglandular lymph nodes to
abscesses. In the case of stones, extensions of the excretory
ducts may also be found.
Chronic recurrent parotitis is the most common form of
chronic inammation in children, typically aged 3–6years.
The disease affects only the parotid gland. The cause is
unknown. The disease can occur on one or both sides. The
gland is intermittently swollen and painful, with concomitant
fever. Multiple hypoechoic (lymphofollicular) foci with a
diameter of 2–4mm are typical (Fig.9.1). The condition is
treated supportively and heals spontaneously in adolescence
or adulthood in most cases.
Other forms of chronic salivary gland inammation are
caused by granulomatous diseases (Mycobacterium avium
infection, actinomycosis, histoplasmosis) or occur in the
context of autoimmune diseases (such as Sjögren’s syndrome or Heerfordt’s disease). Lymphofollicular proliferation may develop as part of HIV disease. Sonographically,
there are multiple hypoechoic, partly echo-free (cystic)
nodules.
Neoplasms
Most masses of the salivary glands are caused by vascular
tumors (infantile hemangiomas and lymphatic vascular
9 Pediatric Sonography oftheNeck: Characteristic Findings
189
Fig. 9.1 Chronic recurrent parotitis. A 2-year-old girl. The gland was
intermittently swollen and painful, with concomitant fever. Multiple
hypoechoic (lymphofollicular) foci
malformations); the gland is typically only partially involved.
Infantile hemangiomas are true neoplasms, usually occurring early after birth and showing rapid proliferative activity
in the rst year of life, with a tendency to spontaneous regression in subsequent years. Sonographically, predominantly
lobulated, hypoechoic areas with vigorous vascularization
are found in the proliferative phase (Fig. 9.2; Video 9.1).
Single, focal calcications are possible. In the regression
phase, vascularization and hypoechoic lobules are regressed.
A proportion of hyperechoic adipose tissue often remains.
Lymphatic vascular malformations appear as cystic masses
with individual septa. Hemorrhage into individual cysts is
possible and leads to a more inhomogeneous sonographic
picture.
Solid epithelial tumors (benign and malignant) are rare in
childhood but match the image of the adult. Differential
diagnoses include malformations of the rst pharyngeal
arch, as they do not always show a clear cystic picture
because of intermittent inammation (Fig. 9.3; Videos 9.2
and 9.3).
Other tumors that may affect this region are neurobromas, lipomas, more rarely rhabdomyosarcomas, lymphomas
(in the salivary gland as MALT lymphoma due to Sjögren’s
syndrome or HIV), tongue-bottom thyroid glands, leukemias, and metastases.
Fig. 9.2 Infantile hemangioma of parotid gland. An 11-week-old girl.
Note the hypoechoic islands (asterisk) of vascular tissue in the gland.
(See Video 9.1)
Fig. 9.3 Cystic malformation of the rst pharyngeal arch. A 15-month-
old girl. Detritus/pus in the parotid gland. (See Videos 9.2 and 9.3)
190
J. Weidemann and G. H. A. Engelcke
Fig. 9.5 Median cervical cyst. A 4-year-old girl. Cyst slightly below
the hyoid bone in front of the larynx (asterisk) without recognizable
signs of inammation. (See Video 9.4)
Fig. 9.4 Cyst of the second branchial arch. A 9-month-old boy. Cyst
dorsolateral to the submandibular gland with mild inammatory
changes. A fat-saturated contrast-enhanced T1-weighted MRI (bottom)
shows inammatory enhancement surrounding the cyst
Suprahyoid Cystic Lesions
Cysts of the rst branchial arch occur in the parotid gland (as
in Fig.9.3). Parts of a cystic lymphatic malformation may
affect the parotid glands (see Table9.1). Tumors and inammation can lead to cystic changes in the intraglandular lymph
nodes of the parotid gland; the submandibular gland has no
intraglandular lymph nodes. Cysts of the second branchial
arch are typically dorsolateral to the submandibular gland
and may appear with or without inammatory changes
(Fig.9.4).
In the midline, the median cervical cyst should be mentioned as the remnant of the thyroglossal duct. This is usually
in the middle or left paramedian at the level of the hyoid
bone or a little further caudal (Fig.9.5; Video 9.4). Sublingual
cysts around the oor of the mouth may correspond to a dermoid/epidermoid cyst or mucoid retention cyst of the sublingual gland, called a ranula (Fig.9.6; Video 9.5). Dorsal to
the mylohyoid muscle, descending ranulas are also called
diving ranulas. Tumors and inammation can lead to cystic
changes of the lymph nodes. Cysts located on the dorsal root
of the tongue may correspond to retention cysts of the valleculae and may lead to airway obstruction.
Fig. 9.6 Mucoid retention cyst (ranula). A 6-year-old boy. Cyst adja-
cent to the right sublingual gland (view from below, oor of mouth).
(See Video 9.5)
9.2.2 Masticator Space
Muscular tumors (sarcomas), as well as local odontogenic
inammation and cysts, can affect the masticator space. The
odontogenic origin is not always sonographically palpable
and therefore should be veried, if necessary, with further
imaging techniques. For example, bony alterations should be
veried with CT scans (Fig. 9.7). Vascular malformations
(lymphatic or lymphatic-venous) may affect this space
proportionally.
Key Points
• Lateral cervical cyst originates from the second pharyngeal arch.
• Median neck cyst appears along the course of the thyroglossal duct, mostly around the level of the hyoid bone.
• Inammatory and malignant lymph node changes, including intraglandular lymph nodes of the parotid gland.
9 Pediatric Sonography oftheNeck: Characteristic Findings
Fig. 9.7 Odontogenic cyst. A
12-year-old boy. Cyst
(asterisk) with bony
expansion on the left side
(semitransparent thin bony
border). A CT scan (bottom)
veries the nature of the bony
alteration
191
• Hemangiomas and vascular malformations (lymphatic
and lymphatic-venous) may involve the parotid gland.
• The masticatory muscles and the lower jaw can be affected
by tumors or odontogenic cysts.
9.3 Infrahyoid Space
The thyroid gland may be completely absent (Fig.9.8; Video
9.6), or only a single thyroid lobe may be missing (Fig.9.9),
more often on the left. In any case, the course of the thyroglossal duct from the oor of the mouth to the thymus should
be examined in order to avoid overlooking ectopic thyroid
tissue. Congenital hypothyroidism may be caused by hypoplasia of the thyroid gland or by goiter, in cases of congenital
metabolic disturbances of the child or mother. A normalsized thyroid gland, however, may be present in the context
of a pseudohypoparathyroidism or a trisomy 21, despite a
demonstrated hypothyroidism.
An inammatory cyst may be due to cysts of the 3rd and
4th branchial cleft, usually found in the left upper thyroid
lobe. Acute inammations of the thyroid are otherwise rare
and usually are due to these cystic malformations. The detec-
Fig. 9.8 Complete thyroid aplasia. A 10-year-old girl. Absent thyroid
gland tissue in normal position around the trachea (asterisk). (See
Video 9.6)
tion of a typical cyst, however, often fails because of the
inammatory changes.
More common are autoimmune-mediated inammations
of the thyroid gland. Hashimoto’s thyroiditis is the most
common thyroid disease of the child and adolescent
(Fig. 9.10). It occurs frequently in association with type 1
192
J. Weidemann and G. H. A. Engelcke
diabetes. There may be over-function in the acute phase or
under-function in the chronic phase. Sonography shows a
swollen gland with increased vascularization in the acute
phase, with multiple hypoechoic nodules ranging in size
from 1 to 6mm. The parenchyma appears increasingly septate and lobulated. A spontaneous regression is possible, or
nodular remodeling with shrinkage of the gland may occur.
Grave’s disease (Fig. 9.11) is caused by autoantibodies
that bind directly to thyroid-stimulating hormone (TSH)
Fig. 9.9 Hemiaplasia of the thyroid gland. A 5-year-old girl. Almost
complete absence of thyroid tissue on the left side (asterisk)
receptors. The disease causes thyroid enlargement, exophthalmos, and hyperthyroidism. In addition to an inhomogeneously hypoechoic, swollen parenchyma, ultrasound shows
increased vascularization in color duplex.
De Quervain’s thyroiditis is a rare focal inammation of
the thyroid gland most likely due to a viral infection.
Thyroid nodules are rare in childhood. The distinction
between benign versus malignant is based on morphological
criteria (with limited safety!) and the time course [4–6]. Unclear
lesions require a biopsy. The criteria for malignancy are primarily solid lesions with inhomogeneous margins, internal calcication (especially microcalcications) (Fig.9.12), a shape
taller than it is wide, the absence of a hypoechoic halo, internal
vascularization in color duplex (Fig.9.13), and pathological
lymph nodes in the neighborhood. Benign lesions include follicular adenoma (round, well-dened, often with hypoechoic
halo, no internal vascularization in color duplex) (Fig.9.14) or
degenerative (colloid) cysts (complex internal structure, echorich comet-tail artifact typical) (Fig. 9.15; Video 9.7).
Multinodular goiter is rare in childhood but may require regular control to exclude focal malignant development.
Papillary thyroid carcinoma is the most common thyroid
carcinoma in childhood and adolescence (80%). It metastasizes
lymphogenously. Follicular thyroid carcinoma comprises 17%
of thyroid carcinomas in these patients; it may have hematog-
Fig. 9.10 Hashimoto’s thyroiditis. A 14-year-old boy. Multiple
hypoechoic nodules in a swollen, septated, and lobulated thyroid gland.
Hypervascularization in color duplex
Fig. 9.11 Grave’s disease. An 11-year-old girl. Thyroid gland with
hypoechoic, swollen parenchyma. Hypervascularization in color duplex
9 Pediatric Sonography oftheNeck: Characteristic Findings
193
Fig. 9.12 Papillary thyroid carcinoma with microcalcications.
(Courtesy of Tilman Rohrer, Department of Pediatrics, Saarland
University Medical Center, Homburg/Saar, Germany)
Fig. 9.13 Papillary thyroid carcinoma (asterisk) in the lower pole of
left lobe (longitudinal section) with internal vascularization in color
duplex. (Courtesy of Tilman Rohrer, Department of Pediatrics, Saarland
University Medical Center, Homburg/Saar, Germany)
enous spread. Medullary thyroid carcinoma is less common, is
associated with multiple endocrine neoplasia type II (MEN II),
and is often metastatic to the lymph nodes and lungs upon discovery. Other tumors of the thyroid are lymphomas (e.g., in the
context of Hashimoto’s thyroiditis) and teratoma.
The four parathyroids are usually indistinguishable from
the isoechogenic thyroid parenchyma. In children with secondary hyperparathyroidism due to renal insufciency and in
rare cases of primary hyperparathyroidism due to an adenoma (Fig. 9.16), the glands may become visible as
hypoechoic nodules. Characteristic is an enlargement of the
Fig. 9.14 Follicular adenoma (asterisk) without microcalcications.
No abnormal internal vascularization in color duplex (not shown).
(Courtesy of Tilman Rohrer, Department of Pediatrics, Saarland
University Medical Center, Homburg/Saar)
Fig. 9.15 Colloid cyst. A 17-year-old girl. Cyst (arrow) with typical
echo-rich comet-tail artifact inside. (See Video 9.7)
pole-supplying vessel of the inferior thyroid artery.
Sometimes the corresponding gland is ectopic (e.g., mediastinal) and can be searched for using MRI.
Rare, cervically located (bronchogenic/esophageal) foregut cysts can be detected sonographically. Laryngoceles can
appear as air-lled or uid-lled paralaryngeal pouches of
the laryngeal ventricle.
Key Points
• An ectopic thyroid gland can be found along the thyro-
glossal duct.
• Hashimoto’s thyroiditis is the most common form of
autoimmune thyroiditis and occurs more commonly in
type 1 diabetes mellitus.
• Third and fourth branchial cleft cysts may occur as focal
thyroid-inltrating inammation.
194
J. Weidemann and G. H. A. Engelcke
Fig. 9.16 Parathyroid adenoma. An 11-year-old girl. Primary hyperpara-
thyroidism associated with multiple endocrine neoplasia type I (MEN I).
Hypoechoic adenoma (asterisk). Note the pole-supplying vessel
• Thyroid nodules are rare in childhood. Possible signs of
malignancy are primarily solid lesions, inhomogeneous
margins, internal calcication (especially microcalcications), taller-than-wide shape, absence of a hypoechoic
halo, internal vascularization in color duplex, and pathological lymph nodes in the neighborhood.
• Papillary, follicular, and medullary thyroid carcinomas
are found in childhood; medullary carcinomas occur
mainly in the context of multiple endocrine neoplasia
type II (MEN II).
• Parathyroid enlargement occurs as part of primary hyperparathyroidism (adenoma) or secondary hyperparathyroidism (renal insufciency).
9.4 Lacking Denition by theHyoid
Branchial cleft cysts are noticeable as cystic, often infected
lesions. In most cases, the lesions originate from the second
arch, in rarer cases from the rst arch [3]. Cysts of the third
and fourth arch are much less common.
The thymus develops from outpourings of the third and
fourth pharyngeal pouches and migrates during development
Fig. 9.17 Normal thymus. A 6-month-old boy. Cross and longitudinal
sections of typical thymus tissue (asterisks)
Fig. 9.18 Ectopic thymus tissue. A 3-year-old boy. Thymus tissue
nestles between the muscles and the vessels of the neck without a bigger mass effect. (See Video 9.8)
along the carotid sheath into the upper mediastinum
(Fig.9.17). Ectopic thymic tissue, although clinically unremarkable, can occasionally be found during cervical sonography and must be differentiated from other lesions (Fig.9.18;
Video 9.8). Sonographically, the ectopic thymus tissue corresponds to the typical echo pattern of the orthotopic thymus,
9 Pediatric Sonography oftheNeck: Characteristic Findings
with its sharply dened shape and soft structure without mass
effect on neighboring structures. Thymic cysts may occur
along the developmental route and (similar to the branchial
cleft cysts) can be transformed by inammation.
Dermoid or epidermoid cysts appear primarily as painless
midline cysts. They occur most often in the area of the oor
of the mouth or the suprasternal area (Fig.9.19; Video 9.9).
Cervical teratomas show up as partly solid, partly cystic
masses in the anterior and lateral cervical region, with a
space-occupying effect on the respiratory tract.
Vascular anomalies can be classied into vascular tumors
and vascular malformations according to the International
Society for the Study of Vascular Anomalies (ISSVA) [7]
(see Table9.3). Infantile hemangiomas, by far the most common vascular tumors, have a typical echo pattern and time
course (Figs.9.20 and 9.21). Larger hemangiomas in the tri-
195
Fig. 9.19 Dermoid cyst. A 7-month-old girl. Cyst of the jugulum near
the sternum (asterisk). No signs of inammation. (See Video 9.9)
Fig. 9.20 Infantile hemangioma of the right cheek. A 2-month-old
girl. Note the hypoechoic vascular part and the more hyperechoic fatty
part of the hemangioma. A fat-saturated T2-weighted MRI (upper left)
and a T1 without fat suppression (lower left) show the vascular and fatty
parts of the lesion
196
Fig. 9.21 Same infantile hemangioma as in Fig.9.20. Note the fatty
transformation 18months later (after propranolol therapy)
geminal area may occur as part of a PHACES syndrome
(posterior fossa malformations, hemangioma, arterial anomalies, coarctation of the aorta and cardiac defects, eye anomalies, and sternal anomalies). These lesions should be
distinguished from capillary vascular malformations in this
area occurring in connection with a Sturge-Weber syndrome
(associated with intracranial leptomeningeal capillary malformation and frequently choroidal or scleral angiomatosis
anomalies leading to seizures and glaucoma.
Vascular malformations are further classied as high-ow
and low-ow lesions (Figs. 9.22 and 9.23) and by the
involved vascular components (arterial, venous, lymphatic).
Malformations can extend over several fascia spaces.
Assessment of the spread to deeper regions of the midface
and neck may require the use of MRI (Fig.9.24). Phleboliths
and bleeding into cystic or cavernous portions of a vascular
malformation can modify the sonographic image.
Abnormalities of the lymph nodes (see Table 9.4) are
among the most common ndings in sonography of the
J. Weidemann and G. H. A. Engelcke
child’s neck [8]. Reactive lymph node enlargement is often
found as a result of inammation of the pharynx or tonsils
(Fig.9.25; Video 9.10). Most unilateral pyogenic inammations are caused by Staphylococcus aureus or group A
beta- hemolytic streptococci (Video 9.11) [9]. These should
be distinguished from peritonsillar abscesses (Video 9.12,
compared with the inconspicuous tonsil shown in Video
9.13). Bilateral lymph node inammation is often caused
by viruses, such as Epstein-Barr virus (EBV) (Fig.9.26).
In these cases, sonography shows regionally enlarged
lymph nodes with preserved oval shape and hilus differentiation, with more or less edematous surrounding reaction
(cellulitis). Purulent intranodal foci may occur in primary
bacterial inltration and mycobacteriosis (Fig.9.27; Video
9.14). Calcications and chimney-like stulas can occur
during mycobacterial infections (Fig.9.28). Pathological
lymph nodes in the context of lymphoma often show a
roundish form, a hypoechoic parenchyma with loss of
hyperechoic hilus, and a marked activity in FDG PET
(Figs.9.29 and 9.30). Color duplex may show increased
ow centrally and peripherally. Supraclavicular lymph
node enlargement is highly suspicious for malignancy
(Fig.9.30). Additional bony lesions associated with atypical lymph nodes may indicate Langerhans cell histiocytosis (Fig.9.31).
Fibromatosis colli typically occurs as lateral neck swelling with accompanying torticollis in infants between weeks
2 and 8. Focal swelling is typically found in the caudal third
of the sternocleidomastoid muscle (Fig. 9.32; Video 9.15
with normal sternocleidomastoid muscle and Video 9.16
with bromatosis). The echotexture is irregularly bromatous here. In the acute phase, an increased vascularization
can be seen in color duplex. The cause of the disease is not
fully understood; birth traumas may play a role but are not a
necessary prerequisite. The lesion typically heals spontaneously. Fibromatous shortening of the muscle can be avoided
by concomitant physiotherapy.
Carcinomas in the head and neck region account for 5%
of all childhood cancers. The most common malignant
tumors in children under the age of 6 are neuroblastomas
(Fig.9.33), lymphomas, and rhabdomyosarcomas. In the age
group of 7–13years, lymphomas, thyroid carcinomas, and
rhabdomyosarcomas are the most common. After puberty,
lymphomas are the most common malignant tumors. The
morphology of lymph node metastases often corresponds to
that of the original tumors or the lymphoma picture.
Ultimately, any tissue of the neck can be the site of origin of
a mass (Figs.9.34, 9.35, 9.36, and 9.37; Videos 9.17, 9.18,
and 9.19).
Iatrogenic lesions are most commonly hematomas after
punctures or thrombosis along central vascular access routes.
9 Pediatric Sonography oftheNeck: Characteristic Findings
Fig. 9.22 Venous
hemangioma (low-ow
lesion). A 23-month-old girl.
MRI shows uid-uid levels
and sparse contrast
enhancement of the lesion.
Color duplex shows sparse
ow signal in some of the
vessels
197
Fig. 9.23 Lymphatic malformation. A 6-year-old boy. Note the thrombus (asterisk) as a possible consequence of a hemorrhage. A fat-saturated
T2-weighted MRT (right) shows the cystic nature of the lesion and the thrombus