Midface Anomalies in Children1
Lisa H. Lowe, MD,
Timothy N. Booth, MD,
Jeanne M. Joglar, MD and
Nancy K. Rollins, MD
1 From the Department of Radiology, Vanderbilt Children's Hospital, 1161 21st Ave S, D-1120 Medical Center North, Nashville, TN 37232-2675 (L.H.L.); and the Department of Radiology, Children's Medical Center of Dallas, Dallas, Tex (T.N.B., J.M.J., N.K.R.). Received April 6, 1999; revision requested June 11 and received July 21; accepted July 23. Address correspondence to L.H.L. (e-mail:lisa.lowe@mcmail.vanderbilt.edu).
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Figure 1. Schematic illustrates midface embryogenesis at 4 , 5, 6, 7, and 10 weeks of development. (Reprinted, with permission, from reference 2.)
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Figure 2. Normal embryologic development of the nasofrontal region. (a) Schematic illustrates the temporary fonticulus nasofrontalis and prenasal space. (b) Schematic illustrates how the fonticulus frontalis closes, the foramen cecum is formed, and a projection of dural diverticulum contacts the tip of the nose. (c) Schematic illustrates how the dural diverticulum retracts into the cranium and the prenasal space is obliterated. (Reprinted, with permission, from reference 5.)
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Figure 3. Normal nasal cavity anatomy in a 28-month-old girl. Axial CT scan demonstrates the pyriform aperture (pa), posterior choanae (pc), and vomer (V). The maxillary spines (ms) mark the inferior margin of the pyriform aperture.
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Figure 4. Normal nasal cavity anatomy in a 10-year-old boy. Coronal CT scan depicts the septal tumescence (*) and frontonasal suture (fn).
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Figure 5a. Normal anatomy of the nasofrontal region in a 3-month-old boy. (a) Axial CT scan shows the nasolacrimal duct (nl), pituitary canal (pit), and a normal cleft between the paired nasal bones (arrow). (b) Coronal CT scan demonstrates the nonossified crista galli (cg), the cribriform plate (cp), and the perpendicular plate of the ethmoid bone (pe). A focal bulge in the nasal septum, the septal tumescence (*), should not be mistaken for a mass.
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Figure 5b. Normal anatomy of the nasofrontal region in a 3-month-old boy. (a) Axial CT scan shows the nasolacrimal duct (nl), pituitary canal (pit), and a normal cleft between the paired nasal bones (arrow). (b) Coronal CT scan demonstrates the nonossified crista galli (cg), the cribriform plate (cp), and the perpendicular plate of the ethmoid bone (pe). A focal bulge in the nasal septum, the septal tumescence (*), should not be mistaken for a mass.
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Figure 6. Normal anatomy of the nasofrontal region in a 10-year-old boy. Coronal CT scan demonstrates the ossified crista galli (cg), the cribriform plate (cp), and the perpendicular plate of the ethmoid bone (pe).
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Figure 7. Normal anatomy of the nasofrontal region in a 15-year-old boy. Coronal T1-weighted MR image shows bright signal intensity in the crista galli (cg) due to fatty replacement, a finding that should not be mistaken for a dermoid cyst.
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Figure 8. Normal nasolacrimal drainage system. Schematic illustrates the drainage of nasolacrimal secretions from the orbit into the nasal cavity. (Reprinted, with permission, from reference 10.)
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Figure 9. Osseomembranous choanal atresia in a 7-month-old girl with chronic rhinorrhea. Axial CT scan shows inward bowing of the maxilla, bone stenosis of the right posterior choanae (arrowheads), and a membrane occluding the choanal lumen (arrow). The vomer is normal in thickness.
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Figure 10. Pyriform aperture stenosis in a 2-day-old boy with respiratory distress. Axial CT scan reveals inward bowing of the maxillary spines (arrow) and narrowing of the pyriform aperture (pa).
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Figure 11a. Faulty development of the nasofrontal region leading to formation of various midface masses. (a) Schematic illustrates how dermal sinus tracts form when there is no involution or only partial involution of the dural diverticulum that extends through the foramen cecum to the columella. Dermoid and epidermoid cysts may form anywhere along the course of the dermal sinus tract owing to desquamation of tissue lining the tract. (b) Schematic illustrates how nasal gliomas form when the dural diverticulum that extends through the foramen cecum does not retract and involute normally, leaving sequestered neurogenic tissue that may be connected to the intracranial contents by a fibrous stalk. (c) Schematic illustrates how frontonasal encephaloceles form when the fonticulus nasofrontalis remains patent. (d) Schematic illustrates how nasoethmoidal encephaloceles form when the foramen cecum fails to close and the prenasal space remains patent. (Reprinted, with permission, from reference 5.)
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Figure 11b. Faulty development of the nasofrontal region leading to formation of various midface masses. (a) Schematic illustrates how dermal sinus tracts form when there is no involution or only partial involution of the dural diverticulum that extends through the foramen cecum to the columella. Dermoid and epidermoid cysts may form anywhere along the course of the dermal sinus tract owing to desquamation of tissue lining the tract. (b) Schematic illustrates how nasal gliomas form when the dural diverticulum that extends through the foramen cecum does not retract and involute normally, leaving sequestered neurogenic tissue that may be connected to the intracranial contents by a fibrous stalk. (c) Schematic illustrates how frontonasal encephaloceles form when the fonticulus nasofrontalis remains patent. (d) Schematic illustrates how nasoethmoidal encephaloceles form when the foramen cecum fails to close and the prenasal space remains patent. (Reprinted, with permission, from reference 5.)
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Figure 11c. Faulty development of the nasofrontal region leading to formation of various midface masses. (a) Schematic illustrates how dermal sinus tracts form when there is no involution or only partial involution of the dural diverticulum that extends through the foramen cecum to the columella. Dermoid and epidermoid cysts may form anywhere along the course of the dermal sinus tract owing to desquamation of tissue lining the tract. (b) Schematic illustrates how nasal gliomas form when the dural diverticulum that extends through the foramen cecum does not retract and involute normally, leaving sequestered neurogenic tissue that may be connected to the intracranial contents by a fibrous stalk. (c) Schematic illustrates how frontonasal encephaloceles form when the fonticulus nasofrontalis remains patent. (d) Schematic illustrates how nasoethmoidal encephaloceles form when the foramen cecum fails to close and the prenasal space remains patent. (Reprinted, with permission, from reference 5.)
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Figure 11d. Faulty development of the nasofrontal region leading to formation of various midface masses. (a) Schematic illustrates how dermal sinus tracts form when there is no involution or only partial involution of the dural diverticulum that extends through the foramen cecum to the columella. Dermoid and epidermoid cysts may form anywhere along the course of the dermal sinus tract owing to desquamation of tissue lining the tract. (b) Schematic illustrates how nasal gliomas form when the dural diverticulum that extends through the foramen cecum does not retract and involute normally, leaving sequestered neurogenic tissue that may be connected to the intracranial contents by a fibrous stalk. (c) Schematic illustrates how frontonasal encephaloceles form when the fonticulus nasofrontalis remains patent. (d) Schematic illustrates how nasoethmoidal encephaloceles form when the foramen cecum fails to close and the prenasal space remains patent. (Reprinted, with permission, from reference 5.)
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Figure 12. Dermoid cyst in a 2-year-old girl with a midline nasal mass. Axial CT scan reveals a midline fatty mass (arrows) with nasal bone erosion. The mass, characteristically located at the glabella, is not connected to the foramen cecum (arrowheads).
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Figure 13a. Dermoid cyst with a dermal sinus tract in a 16-month-old girl with a midline nasal dimple. (a) Sagittal T1-weighted MR image obtained with a vitamin E tablet placed over the midline dimple (E) shows soft-tissue signal intensity within the fat of the glabella (black arrows) and high signal intensity within the crista galli (white arrow), findings that are worrisome for intracranial extension. (b, c) Axial (b) and coronal (c) fat-suppressed T2-weighted MR images show bright signal intensity within the soft tissue of the glabella (solid arrow in b) and the foramen cecum (open arrow). These findings are consistent with a glabellar dermoid cyst accompanied by a dermal sinus tract extending through the foramen cecum.
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Figure 13b. Dermoid cyst with a dermal sinus tract in a 16-month-old girl with a midline nasal dimple. (a) Sagittal T1-weighted MR image obtained with a vitamin E tablet placed over the midline dimple (E) shows soft-tissue signal intensity within the fat of the glabella (black arrows) and high signal intensity within the crista galli (white arrow), findings that are worrisome for intracranial extension. (b, c) Axial (b) and coronal (c) fat-suppressed T2-weighted MR images show bright signal intensity within the soft tissue of the glabella (solid arrow in b) and the foramen cecum (open arrow). These findings are consistent with a glabellar dermoid cyst accompanied by a dermal sinus tract extending through the foramen cecum.
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Figure 13c. Dermoid cyst with a dermal sinus tract in a 16-month-old girl with a midline nasal dimple. (a) Sagittal T1-weighted MR image obtained with a vitamin E tablet placed over the midline dimple (E) shows soft-tissue signal intensity within the fat of the glabella (black arrows) and high signal intensity within the crista galli (white arrow), findings that are worrisome for intracranial extension. (b, c) Axial (b) and coronal (c) fat-suppressed T2-weighted MR images show bright signal intensity within the soft tissue of the glabella (solid arrow in b) and the foramen cecum (open arrow). These findings are consistent with a glabellar dermoid cyst accompanied by a dermal sinus tract extending through the foramen cecum.
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Figure 14a. Nasal glioma in a 6-month-old boy with a glabellar skin tag. Axial CT scans show a nonenhancing soft-tissue mass involving the right nasal cavity (arrows in a) and glabella (arrow in b).
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Figure 14b. Nasal glioma in a 6-month-old boy with a glabellar skin tag. Axial CT scans show a nonenhancing soft-tissue mass involving the right nasal cavity (arrows in a) and glabella (arrow in b).
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Figure 15. Frontonasal encephalocele in a 5-day-old girl with a midline mass. Sagittal T1-weighted MR image shows brain parenchyma within a glabellar mass (*). Outward bowing of the inferior frontal bone (arrow) and associated absence of the corpus callosum (arrowheads) are also noted.
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Figure 16. Nasoethmoidal encephalocele in a 10-year-old boy with an intranasal mass. Coronal T1-weighted MR image demonstrates a nasal mass containing cerebrospinal fluid (open arrows) and brain parenchyma (solid arrow).
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Figure 17. Nasolacrimal duct stenosis in a 4-year-old boy with right epiphora. On a coronal CT scan, the right nasolacrimal duct (arrowhead) is slightly larger than the left nasolacrimal duct (arrow) due to accumulation of secretions proximal to the stenotic Hasner valve. The left nasolacrimal duct contains air, a finding that could be interpreted as normal.
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Figure 18a. Dacryocystoceles in a 14-day-old boy with bilateral medial canthal swelling. Axial fat-suppressed T2-weighted MR images obtained at the level of the medial canthus (a) and inferior meatus (b) demonstrate bilateral, well-defined, thin-walled masses with fluid signal intensity that follow the expected course of the nasolacrimal ducts (arrows).
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Figure 18b. Dacryocystoceles in a 14-day-old boy with bilateral medial canthal swelling. Axial fat-suppressed T2-weighted MR images obtained at the level of the medial canthus (a) and inferior meatus (b) demonstrate bilateral, well-defined, thin-walled masses with fluid signal intensity that follow the expected course of the nasolacrimal ducts (arrows).
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Figure 19a. Dacryocystitis in a 5-week-old boy with bilateral dacryocystoceles. (a) Contrast-enhanced axial CT scan shows left-sided preseptal inflammation (solid arrows) and a left medial canthal mass (*) with a thick, enhancing wall (open arrows). (b) Contrast-enhanced axial CT scan demonstrates bilateral low-attenuation and well-defined, thin-walled nasal masses (arrows).
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Figure 19b. Dacryocystitis in a 5-week-old boy with bilateral dacryocystoceles. (a) Contrast-enhanced axial CT scan shows left-sided preseptal inflammation (solid arrows) and a left medial canthal mass (*) with a thick, enhancing wall (open arrows). (b) Contrast-enhanced axial CT scan demonstrates bilateral low-attenuation and well-defined, thin-walled nasal masses (arrows).
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Figure 20a. Apert syndrome in a 5-day-old boy with proptosis and syndactyly. (a) Axial CT scan demonstrates shallow proptotic orbits (arrowheads) and gradual narrowing of the nasal cavity from front to back (arrows). (b) Axial CT scan demonstrates midface hypoplasia (arrows), narrowing of the pyriform aperture (pa), and posterior choanal stenosis (pc).
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Figure 20b. Apert syndrome in a 5-day-old boy with proptosis and syndactyly. (a) Axial CT scan demonstrates shallow proptotic orbits (arrowheads) and gradual narrowing of the nasal cavity from front to back (arrows). (b) Axial CT scan demonstrates midface hypoplasia (arrows), narrowing of the pyriform aperture (pa), and posterior choanal stenosis (pc).
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Figure 21a. Crouzon syndrome in a 14-year-old girl. (a) Sagittal T1-weighted MR image reveals a steep clivus (arrowheads), a vertically oriented brainstem (*), and a Chiari I malformation (arrow). The signal voids in the calvaria are caused by sutures from previous cranioplasty. (b) Maximum-intensity-projection image from an MR venogram shows markedly abnormal venous drainage including absence of flow in the region of the jugular bulbs (*), multiple collateral veins (arrowheads), and hypoplastic transverse sinuses (arrows).
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Figure 21b. Crouzon syndrome in a 14-year-old girl. (a) Sagittal T1-weighted MR image reveals a steep clivus (arrowheads), a vertically oriented brainstem (*), and a Chiari I malformation (arrow). The signal voids in the calvaria are caused by sutures from previous cranioplasty. (b) Maximum-intensity-projection image from an MR venogram shows markedly abnormal venous drainage including absence of flow in the region of the jugular bulbs (*), multiple collateral veins (arrowheads), and hypoplastic transverse sinuses (arrows).
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Figure 22. Treacher Collins syndrome in a 2-year-old boy with micrognathia. Axial CT scan demonstrates midface hypoplasia (solid arrows), shallow orbits, proptosis (open arrows), and hypoplastic orbital walls.
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Copyright © 2000 by the Radiological Society of North America.
