Pituitary Dwarfism

Patients with pituitary dwarfism can either have isolated growth hormone deficiency or multiple pituitary hormonal deficiencies.

Patients with isolated growth hormone deficiency have a thin or truncated pituitary stalk (the stalk is rarely absent or normal), normal or small anterior pituitary, and an ectopic posterior pituitary.

Patients with multiple pituitary hormonal deficiencies, on the other hand, may have a small or absent pituitary stalk, a small or absent anterior pituitary, and an ectopic or absent posterior pituitary.

To sum up the above: A thin stalk is strongly suggestive of isolated growth hormone deficiency, while an absent stalk is strongly suggestive of multiple pituitary hormonal deficiencies.

A contrast-enhanced MRI is needed to fully define the infundibulum.

The above case is a non-contrast MRI with an absent posterior pituitary bright spot (yellow arrow), an absent or thin stalk (green arrow), and an ectopic bright spot in the region of the median eminence (pink arrow). Without contrast, we can't tell if the stalk is absent or very thin.

An ectopic posterior pituitary lobe can also be seen in cases of pituitary stalk transection and septo-optic dysplasia and as an asymptomatic variant.

References

• Fujisawa I, Kikuchi K, Nishimura K, Togashi K, Itoh K, Noma S, Minami S, Sagoh T, Hiraoka T, Momoi T, et al. Transection of the pituitary stalk: development of an ectopic posterior lobe assessed with MR imaging. Radiology. 1987 Nov;165(2):487-9.
• Grossman RI and Yousem DM. Chapter 11. in Neuroradiology: The Requisites (second edition). Mosby, 2003. pp 539-541.

Solitary Rib Lesions

• Healing fracture
• Fibrous dysplasia
• Enchondroma
• Multiple myeloma
• Metastatic disease
• Osteomyelitis
• Osteochondroma
• Langerhans cell histiocytosis
• Ewing sarcoma

No pathology for the above case is available; however, it has remained stable for 2 years and is suspected to be fibrous dysplasia.

Ligamentum Flavum "Buckling"

From the terminology desk: Thickening of the ligamentum flavum is often attributed to the buckling of a normal ligamentum flavum when the disk at that level narrows. Chokshi et al found that there was correlation between facet joint arthropathy and ligamentum flavum thickening and between facet joint arthropathy and ligamentum flavum enhancement. This new data suggests that the perceived thickening represents actual thickening due to the spread of inflammation from the adjacent facet joints.

References

Chokshi FH, Quencer RM, Smoker WR. The "thickened" ligamentum flavum: is it buckling or enlargement? AJNR Am J Neuroradiol. 2010 Nov;31(10):1813-6.

Radiographic Appearance of Glass

The common notion that the opacity of glass on radiographs depends on its lead or heavy metal content is not correct. All glass has some degree of radiopacity on radiographs. Detection can be made easier by projections that free the fragment from overlying structures and expose its greatest diameter. Ultrasound is the imaging modality of choice for superficial foreign objects.

References

• Felman AH, Fisher MS. The radiographic detection of glass in soft tissue. Radiology. 1969 Jun;92(7):1529-31.
• Horton LK, Jacobson JA, Powell A, Fessell DP, Hayes CW. Sonography and radiography of soft-tissue foreign bodies. AJR Am J Roentgenol. 2001 May;176(5):1155-9.

Pear-Shaped Urinary Bladder

A pear-shaped urinary bladder on urography, also appreciated as a gourd or inverted tear drop, refers to symmetric bladder compression and elevation and can be seen with the following conditions:

• Pelvic hematoma: Most common cause.
• Extraperitoeal urinoma:
• Pelvic lipomatosis: Also causes increased radiolucency of the pelvis.
• Inferior vena cava obstruction: Compression is by engorged pelvic collaterals.

Radiolucent Filling Defects in the Urinary Tract

Differential considerations for radiolucent filling defects in the urinary tract on a contrast study:

• Radiolucent calculi: For example, uric acid.
• Neoplasm: Transitional cell carcinoma most common.
• Blood clot:
• Infectious debris:
• Sloughed papilla:
• Fungal material:

Incomplete Partition

Congenital malformations of the inner ear can be divided into two types of incomplete partition:

Incomplete partition type I, also known as cystic cochleovestibular malformation, is a condition where the cochlea lacks the entire modiolus and cribriform area, resulting in a cystic appearance. This is accompanied by a large cystic vestibule. On CT, there is an empty, cystic cochlea, a dilated vestibule, and absence of any partition within the cochlea. The vestibular aqueduct is not enlarged.

Incomplete partition type II is the classic Mondini deformity. The cochlea consists of 1.5 turns, in which the middle and apical turns coalesce to form a cystic apex, accompanied by a dilated vestibule and enlarged vestibular aqueduct. CT shows a dilated vestibule, an enlarged vestibular aqueduct, and confluence of middle and apical turns of the cochlea.

References

Sennaroglu L, Saatci I. A new classification for cochleovestibular malformations. Laryngoscope. 2002 Dec;112(12):2230-41.

Large Vestibular Aqueduct Syndrome

Large vestibular aqueduct syndrome (also known as enlarged endolymphatic duct and sac syndrome), is the most frequent cause of bilateral progressive sensorineural hearing loss in children. While the deformity of the endolymphatic duct and sac is congenital, the hearing loss is acquired and thought to be related to minor head trauma (the exact mechanism is unknown).

The vestibular aqueduct is considered enlarged on CT when it measures ≥ 1 mm at its midpoint and ≥ 2 mm at the operculum. T2-weighted images show a high signal intensity enlarged endolymphatic sac.

10%-15% of patients have Pendred syndrome, which is the most common syndromic cause of hearing loss. It is an inherited disorder consisting of a dyshormonogenic goiter (cannot organify iodide efficiently) and sensorineural hearing loss and may be accompanied by vestibular symptoms. Imaging shows a large vestibular aqueduct and inner ear malformations. The perchlorate discharge test is used for diagnosis.

References

• Goldfeld M, Glaser B, Nassir E, Gomori JM, Hazani E, Bishara N. CT of the ear in Pendred syndrome. Radiology. 2005 May;235(2):537-40.
• Naganawa S, Koshikawa T, Fukatsu H, Ishigaki T, Nakashima T. Serial MR imaging studies in enlarged endolymphatic duct and sac syndrome. Eur Radiol. 2002 Dec;12 Suppl 3:S114-7.
• Vijayasekaran S, Halsted MJ, Boston M, Meinzen-Derr J, Bardo DM, Greinwald J, Benton C. When is the vestibular aqueduct enlarged? A statistical analysis of the normative distribution of vestibular aqueduct size. AJNR Am J Neuroradiol. 2007 Jun-Jul;28(6):1133-8.

The Swirl Sign and Internal Hernias

Gastric bypass, a one-shot surgical solution for complex, life-long psychosocial issues, has become routine therapy for morbid obesity, with laparoscopic Roux-en-Y being the most commonly performed bariatric procedure in many institutions. Internal hernias, which are more common after laparoscopic gastric bypass than after open gastric bypass, are a dreaded complication. The presentation is often nonspecific, making CT an important part of diagnosis.

A number of CT findings have been associated with internal hernias. The best single predictor of internal hernia is the mesenteric swirl (or whirl) sign: The swirled appearance of vessels and fat at the mesenteric root (see video, which may be blocked by Websense). The problem with the swirl sign is that mesenteric swirling can be seen in healthy persons and post-laparoscopic Roux-en-Y gastric bypass patients without an internal hernia, so the finding must be considered in conjunction with the clinical picture.

The degree of swirl may be used to increase confidence. In one series, all cases of mesenetric swirl of at least 270° were associated with a hernia at surgery. In patients with a mesenteric swirl but no hernia at surgery, the median amount of swirl was less than 90°.

References

• Iannuccilli JD, Grand D, Murphy BL, Evangelista P, Roye GD, Mayo-Smith W. Sensitivity and specificity of eight CT signs in the preoperative diagnosis of internal mesenteric hernia following Roux-en-Y gastric bypass surgery. Clin Radiol. 2009 Apr;64(4):373-80.
• Lockhart ME, Tessler FN, Canon CL, Smith JK, Larrison MC, Fineberg NS, Roy BP, Clements RH. Internal hernia after gastric bypass: sensitivity and specificity of seven CT signs with surgical correlation and controls. AJR Am J Roentgenol. 2007 Mar;188(3):745-50.

Grynfeltt-Lesshaft Hernia

A Grynfeltt-Lesshaft hernia is a herniation through the superior lumbar (Grynfeltt-Lesshaft) triangle. The superior lumbar triangle is bordered medially by the quadratus lumborum muscle, superiorly by the 12th rib, and laterally by the internal oblique muscle. The floor of the lumbar triangle is the transversalis fascia and the aponeurosis of the transversalis muscle of the abdomen. The roof of the triangle is the external oblique and latissimus dorsi muscles.

References

Orcutt TW. Hernia of the superior lumbar triangle. Ann Surg. 1971 Feb;173(2):294-7.

De Quervain Tenosynovitis: Radiographic Findings

De Quervain tenosynovitis is a stenosing tenosynovitis of the first dorsal compartment of the wrist at the radial styloid. While diagnosis is primarily clinical (e.g., the Finkelstein test), MRI can be used for confirmation. Radiographs may be normal or reveal soft tissue swelling along the radial styloid process (seen in above image).

In addition, focal bony radial styloid abnormalities have been found to correlate with the presence of de Quervain tenosynovitis. These include radial styloid periosteal bone formation and cortical erosion without and with sclerosis (image above) distal to the level of the physeal plate remnant.

A normal variant that should not be confused with cortical irregularity of the distal radius is the normal cortical contour bulge or peak at the level of the physeal plate remnant.

References

Chien AJ, Jacobson JA, Martel W, Kabeto MU, Marcantonio DR. Focal radial styloid abnormality as a manifestation of de Quervain tenosynovitis. AJR Am J Roentgenol. 2001 Dec;177(6):1383-6.

Congenital Cochlear Eponyms

• Mondoni defect: Most common congenital cochlear deformity. Incomplete development of the 2.5 turns of the cochlea, with a relatively well-formed basal turn and ballooning of the middle and apical turns into a cyst. 20% of cases have associated enlarged endolymphatic sacs and vestibular aqueducts.
• Cock deformity: Also known as Michel dysplasia. Second most common congenital cochlear deformity. Common cavity of the vestibule and cochlea. The modiolus is absent. Semicircular canals may be affected.
• Michel aplasia: Complete absence of the inner ear structures. Associated with other skull base abnormalities, abnormal course of the facial nerve, jugular vein abnormalities, absent stapes, absent oval window.

References

Neuroradiology: The Requisites (2nd ed). pp 592-594.

Peliosis Hepatis

Peliosis hepatis is an uncommon vascular disorder of the liver characterized by multiple blood-filled cysts of various sizes. The cysts may be lined by endothelium (phlebectatic variant)or represent hemorrhagic liver cell necrosis (parenchymal variant).

Peliosis hepatis is associated with hormonal stimulation (steroids, oral contraceptives, tamoxifen, estrogens), immunoglobulin therapy, azathioprine, malignancies, hematologic disorders (Hodgkin disease, multiple myeloma), transplantation, and infections (pulmonary tuberculosis, HIV, Bartonella henselae and Rochalimaea henselae).

The lesions appear homogeneously hypoechoic in patients with a fatty livers and hyperechoic in patients with healthy livers. The lesions will appear heterogeneously hypoechoic when hemorrhagic. Doppler can show hypervascularity within and surrounding the lesions.

The lesions are low-attenuation on noncontrast CT, but can be missed when smaller than 1 cm. Lesions may have increased attenuation in cases of hemorrhage or internal calcification. During the arterial phase there is early globular enhancement with small pools of contrast in the center of the lesions (target sign). During the portal venous phase, the lesions may demonstrate a centrifugal (more common) or centripetal (mimicking hemangioma) progression of enhancement. The phlebectatic variant may have late diffuse homogeneous hyperattenuation, but not the parenchymal variant, since the latter is composed of hemorrhagic parenchymal necrosis. Larger cavities that communicate with sinusoids can have the same attenuation as blood vessels.

MRI findings will depend on the stage of the blood within the lesions. The lesions are usually T1-hypointense and usually T2-hyperintense with foci of high signal from hemorrhagic necrosis. Like CT, the enhancement pattern is typically centrifugal. Delayed phase images may show strong enhancement with a branching pattern representing the vascular component of the lesion.

In contrast to the nonspecific appearance seen on ultrasound, CT, and MR, angiographic findings are unique: Multiple pools of contrast on late arterial phase images that persist on parenchymal and early venous phases.

Differential considerations for multiple hemorrhagic hepatic lesions include:

• Hemorrhagic metastases:
• Hepatic adenomas: Increased risk with glycogen storage diseases, diabetes mellitus, hemochromatosis, acromegaly, oral contraception and anabolic steroids.
• Hepatocellular carcinoma:
• Peliosis hepatis:

References

• Iannaccone R, Federle MP, Brancatelli G, Matsui O, Fishman EK, Narra VR, Grazioli L, McCarthy SM, Piacentini F, Maruzzelli L, Passariello R, Vilgrain V. Peliosis hepatis: spectrum of imaging findings. AJR Am J Roentgenol. 2006 Jul;187(1):W43-52.
• Savastano S, San Bortolo O, Velo E, Rettore C, Altavilla G. Pseudotumoral appearance of peliosis hepatis. AJR Am J Roentgenol. 2005 Aug;185(2):558-9.

Stercoral Colitis

Stercoral colitis is a rare inflammatory colitis caused by increased intraluminal and colonic wall pressure from a fecaloma (large mass of dry, hard stool), which leads to decreased blood supply. If left untreated, it can cause ulceration and perforation. In about 75% of cases, the ulcers are found in the sigmoid colon or rectum, with the most common locations being the anterior rectum just proximal to the peritoneal reflection; the antimesenteric border of the rectosigmoid junction; and the apex of the sigmoid colon.

On CT, in a region of fecal impaction, there is thickening of the colonic wall and stranding of the pericolonic fat. Extraluminal bubbles of gas and an abscess suggest perforation.

References

Heffernan C, Pachter HL, Megibow AJ, Macari M. Stercoral colitis leading to fatal peritonitis: CT findings. AJR Am J Roentgenol. 2005 Apr;184(4):1189-93.

Pyogenic Granuloma and Silver Nitrate

Pyogenic granuloma is a common, benign, vascular lesion of the skin that presents as a rapidly growing papule or polyp that bleeds easily after minor trauma. It is an acquired reactive hyperproliferative vascular response to various injuries (penetrating trauma, infection) and chemical stimuli (hormones, retinoid therapy). The most commonly affected sites are the hands, feet, lips, face, and upper trunk.

On inspection, the typical exophytic pyogenic granuloma pops out of the skin with inward growth of the surrounding rim of epidermis, which can cause a pedunculated appearance of the lesion. You can see this on the lateral oblique view of the thumb (left panel) in the above image.

Many treatment strategies exist, ranging from expectant management to surgical excision. A simple treatment is silver nitrate cauterization, which can be done in the office or the ED. The patient above had silver nitrate cauterization, and the silver nitrate can be seen as small radioopaque fragments near the wound.

The bone destruction seen above (right panel) is osteomyelitis, related to the infectious etiology of the pyogenic granuloma in this patient.

It should go without saying that this is not a radiologic diagnosis.

References

• Gilmore A, Kelsberg G, Safranek S. Clinical inquiries. What's the best treatment for pyogenic granuloma? J Fam Pract. 2010 Jan;59(1):40-2.
• Quitkin HM, Rosenwasser MP, Strauch RJ. The efficacy of silver nitrate cauterization for pyogenic granuloma of the hand. J Hand Surg Am. 2003 May;28(3):435-8.
• Zaballos P, Llambrich A, Cuéllar F, Puig S, Malvehy J. Dermoscopic findings in pyogenic granuloma. Br J Dermatol. 2006 Jun;154(6):1108-11.

Freiberg Infraction

Freiberg infraction refers to the collapse of subchondral bone with osteonecrosis involving the metatarsal head (usually the second or third). The joint may secondarily be affected by osteoarthritis.

It is felt to be related to acute or repetitive injury or to vascular compromise. High-heeled shoes are thought to be one causative factor and the condition is more common in women.

The image above shows flattening of the second metatarsal head with associated osteoarthritis.

MRI early in the course of the disease will show low T1 and high T2 signal in the metatarsal head. The main differential consideration at this point includes a stress reaction. Later in the course of the disease, there will be flattening of the metatarsal head with low T2 signal corresponding to sclerosis.

References

Ashman CJ, Klecker RJ, Yu JS. Forefoot pain involving the metatarsal region: differential diagnosis with MR imaging. Radiographics. 2001 Nov-Dec;21(6):1425-40.

Prestyloid Parapharyngeal Space Masses

The parapharyngeal space is an inverted pyramid extending from the base of the skull to the hyoid bone. It is bordered laterally by the parotid and masticator spaces, medially by the pharyngeal mucosal space, and posteromedially by the retropharyngeal space.

The tensor-vascular-styloid fascia extends through the parapharyngeal space between the tensor veli palatini muscle and the styloid process. It can be used to divide the parapharyngeal space into pre- and post-styloid compartments. The post-styloid compartment contains the carotid sheath and is also known as the carotid space. The pre-styloid compartment is what most people in the field are actually referring to when they talk about the parapharyngeal space.

The post-styloid compartment (also known as the carotid space) contains the internal carotid artery, internal jugular vein, cranial nerves IX-XII, the cervical sympathetic chain, and the glomus bodies. The pre-styloid compartment (also known simply as the parapharyngeal space) contains fat, lymph nodes, minor salivary glands, nerves (branches of V₃), arteries (internal maxillary and ascending pharyngeal), and veins (pharyngeal venous plexus).

Most commonly, a mass found in the parapharyngeal space has invaded from adjacent spaces:

• Salivary gland masses: Pleomorphic adenoma of the parotid gland most common; diving ranula rare.
• Neurogenic tumors:
• Glomus tumors: Glomus vagale (post-styloid/carotid space),
• Pharyngeal carcinoma: Invasion of the parapharyngeal space seen in 65% of cases at time of diagnosis.
• Tonsillar tumors
• Synovial sarcoma: Fluid levels, hemorrhage, and calcification.
• Lymphadenopathy
• Infection: Tonsillitis, pharyngitis, odontogenic abscess, parotid abscess.
• Chordoma
• Meningioma
• Other: Second branchial cleft cysts (uncommon).

Primary lesions of the prestyloid parapharyngeal space are much less common and can arise from the contents mentioned above:

• Lymphadenopathy: Most common mass in the prestyloid compartment.
• Salivary gland tumors: Pleomorphic adenoma and other minor salivary gland tumors (e.g., mucoepidermoid carcinoma, adenoid cystic carcinoma).
• Neural tumors: Schwannoma.

References

• Shin JH, Lee HK, Kim SY, Choi CG, Suh DC. Imaging of parapharyngeal space lesions: focus on the prestyloid compartment. AJR Am J Roentgenol. 2001 Dec;177(6):1465-70.
• Neuroradiology requisites (2nd ed), pp 721-723.

Single-Suture Craniosynostoses

Classic appearances associated with premature closure of various sutures:

• Sagittal suture: Most common form. Scaphocephaly (skaphe=a light boat or skiff) or dolichocephaly (dolicho=long). Long and narrow head.
• Metopic suture: Trigonocephaly (trigonon=triangle). The head looks triangle-shaped when viewed from above with a ridge along the middle of the forehead. Hypotelorism and parietooccipital bossing.
• Coronal suture: Unilateral: Harlequin or winking eye, due to ipsilateral frontal bone flattening that leads to elevation of the superior orbital rim and contralateral frontal bossing that leads to depression of the superior orbital rim. Bilateral: Brachycephaly (brachys=short). Decreased anteroposterior dimension and increased bitemporal distance and height.
  
  The harlequin eye can also be seen with neurofibromatosis (due to dysplasia of the greater wing of the sphenoid) or focal calvarial expansion from an intracranial mass.
• Lambdoid suture: Very rare. Posterior plagiocephaly (plagio=oblique). Most commonly the appearance represents deformational (positional) plagiocephaly. The ears will be pulled back, in contrast to the much more common positional plagiocephaly, where the ears are pushed forward.

Vascular Loop Syndrome

Vascular loop syndrome refers to the compression of a nerve root by a vascular loop and can most commonly affects the trigeminal and facial nerves.

In the case of the trigeminal nerve, patients may present with trigeminal neuralgia as the result of the contact. High-resolution, heavily T2-weighted images are used to reveal a serpiginous asymmetric vessel in the high cerebellopontine angle along the proximal preganglionic segment of the trigeminal nerve or at its root entry zone.

In the case of the facial nerve (shown above), patients present with involuntary hemifacial spasm. High-resolution, heavily T2-weighted images are used to reveal a serpiginous asymmetric vessel in the mid cerebellopontine angle along the proximal facial nerve or at its root entry zone.

Masses of the Meckel Cave

Meckel cave tumors are usually asymptomatic and can be followed if characteristically benign findings are seen on MRI or CT. Differential considerations for masses in the Meckel cave include:

• Trigeminal schwannoma: The case shown above. Relatively rare. Can be seen with neurofibromatosis type 2 or as an isolated entity. Smooth margins, usually low on T1 and high on T2. Avid enhancement with or without cystic components.
• Epidermoid tumor: Arise from incomplete cleavage of neural ectoderm from cutaneous ectoderm and grow by desquamation of epithelial cells. Lobulated margins, Low on T1 and high on T2, minimal mass effect.
• Meningioma: Isointense to surrounding normal brain parenchyma on T1- and T2-weighted images. May calcify.
• Trigeminal lipoma: Rare. Looks like fat. Infiltrates the trigeminal nerve fascicles.
• Primary lymphoma: Can mimic trigeminal schwannoma. Consider this diagnosis in immunocompromised patients. The presence of an apparent dural tail can suggest lymphoma in the absence of hyperostosis (typically seen with meningiomas).
• Arachnoid cyst: Fluid signal on all sequences.
• Chordoma: Arises from adjacent clivus.

References

• Abdel Aziz KM, van Loveren HR. Primary lymphoma of Meckel's cave mimicking trigeminal schwannoma: case report. Neurosurgery. 1999 Apr;44(4):859-62; discussion 862-3.
• Beck DW, Menezes AH. Lesions in Meckel's cave: variable presentation and pathology. J Neurosurg. 1987 Nov;67(5):684-9.
• Yuh WT, Wright DC, Barloon TJ, Schultz DH, Sato Y, Cervantes CA. MR imaging of primary tumors of trigeminal nerve and Meckel's cave. AJR Am J Roentgenol. 1988 Sep;151(3):577-82.

Eosinophilic Fasciitis

Eosinophilic fasciitis (Shulman disease) is clinically characterized by stiffness, swelling, and pain in the extremities that typically presents suddenly after strenuous activity and responds to steroid therapy. Other causes of eosinophilic fasciitis include exposure to simvastatin, trichloroethylene, and L-tryptophan (contaminated and otherwise).

Patients may have peripheral eosinophilia, hypergammaglobulinemia, elevated sedimentation rate, and sclerodermalike skin findings primarily of the extremities. Involvement of the trunk has also been reported.

MRI in patients with active eosinophilic fasciitis reveals fascial thickening, increased fluid signal within the fascia, and fascial enhancement.

References

• Baumann F, Brühlmann P, Andreisek G, Michel BA, Marincek B, Weishaupt D. MRI for diagnosis and monitoring of patients with eosinophilic fasciitis. AJR Am J Roentgenol. 2005 Jan;184(1):169-74.
• Blauvelt A, Falanga V. Idiopathic and L-tryptophan-associated eosinophilic fasciitis before and after L-tryptophan contamination. Arch Dermatol. 1991 Aug;127(8):1159-66.
• Hamilton ME. Eosinophilic fasciitis associated with L-tryptophan ingestion. Ann Rheum Dis. 1991 Jan;50(1):55-6.
• Moulton SJ, Kransdorf MJ, Ginsburg WW, Abril A, Persellin S. Eosinophilic fasciitis: spectrum of MRI findings. AJR Am J Roentgenol. 2005 Mar;184(3):975-8.

Arthritis and Primary Biliary Cirrhosis

Patients with primary biliary cirrhosis may present with an erosive arthropathy distributed in the distal and proximal interphalangeal joints with relative sparing of the metacarpophalangeal joints. These marginal erosions vary from small, punched-out lesions to large defects with sparing of the articular surface. Cartilage loss and joint space narrowing can also be seen.

Chondrocalcinosis, which is seen with liver disorders such as hemochromatosis and Wilson disease can also be seen. Hypertrophic osteoarthropathy is a rare complication.

The picture may be complicated by coexistent rheumatoid arthritis.

References

O'Connell DJ, Marx WJ. Hand changes in primary biliary cirrhosis. Radiology. 1978 Oct;129(1):31-5.

Carpal Coalitions

Carpal coalition refers to failure of segmentation of the carpal bones. Isolated coalitions tend to occur between two bones in the same carpal row (e.g., luontriquetral and scapholunate), while syndromic coalitions tend to cross carpal rows and involve more than two bones.

Various syndromes and congenital abnormalities have been associated with carpal coalitions.

• Arthrogryposis: The clinical finding of multiple congenital contractures that affect two or more different areas of the body. Seen as part of more than 300 different disorders. Carpal coalitions more common than tarsal coalitions.
• Ellis-van Creveld syndrome: Also called chondroectodermal dysplasia. Usually coalition is between the capitate and hamate.
• Hand-foot-genital syndrome: Limb malformations and urogenital defects. The most common malformation is mild bilateral shortening of the thumbs and great toes, predominantly of the distal phalanges and/or the first metacarpal or metatarsal. Urogenital abnormalities include abnormalities of the ureters and urethra, incomplete Müllerian fusion (females), and hypospadias (males). The most common coalition is between the scaphoid and trapezium.
• Symphalangism: Partial or total absence of interphalangeal joints sometimes with tarsal and carpal coalition. The most common coalition is between the triquetrum and the hamate.

The case shown here is lunotriquetral coalition, which is the most common of the carpal coalitions and is twice as common in females. Because patients with lunotriquetral coalition tend have widened scapholunate intervals without injury to the scapholunate ligament, this finding should be considered a normal variant in patients with lunotriquetral coalition.

References

• Bamshad M, Van Heest AE, Pleasure D. Arthrogryposis: a review and update. J Bone Joint Surg Am. 2009 Jul;91 Suppl 4:40-6.
• Innis JW. Hand-Foot-Genital Syndrome . In: Pagon RA, Bird TC, Dolan CR, Stephens K, editors. GeneReviews [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2006.
• Metz VM, Schimmerl SM, Gilula LA, Viegas SF, Saffar P. Wide scapholunate joint space in lunotriquetral coalition: a normal variant? Radiology. 1993 Aug;188(2):557-9.
• Poznanski AK, Holt JF. The carpals in congenital malformation syndromes. Am J Roentgenol Radium Ther Nucl Med. 1971 Jul;112(3):443-59.

Retroverted or Curled Epiglottis

We came across a curled epiglottis recently as part of a cervical spine evaluation. Keats does not list this as a variant. The otoaryngology literature describes a retroverted epiglottis touching the base of the tongue that can cause symptoms of globus pharyngeus. The symptoms resolved in this case series of 4 patients following laser excision of the retroverted part of their epiglottis. Our patient didn't have any symptoms of globus pharyngeus according to the clinic notes, so the significance of the curled epiglottis in this case is unknown.

References

Agada FO, Coatesworth AP, Grace AR. Retroverted epiglottis presenting as a variant of globus pharyngeus. J Laryngol Otol. 2007 Apr;121(4):390-2.

Madelung Deformity

Madelung deformity is a growth disturbance of the distal radius caused by focal dysplasia of the physis that results in triangular deformity and medial and volar tilt of the distal radius. Females are more commonly affected than males.

Radiographs will show increased volar bowing of the distal radius, a widened distal radioulnar joint, increased volar tilt of the distal radial articular surface, and an exaggerated ulnar tilt of the distal radius. The carpal bones wedge into the triangular deformity created by the distal radius. In addition, osteophytes may form along the inferior ulnar border of the radius.

Some measurements can be used for the diagnosis of Madelung deformity. When any one of these thresholds is met, a diagnosis of a Madelung deformity can be made.

• Ulnar tilt ≥ 33°: On frontal radiographs, the acute angle between the longitudinal axis of the ulna and a line tangential to the proximal surfaces of the scaphoid and lunate is subtracted from 90.
• Lunate subsidence ≥ 4 mm: On frontal radiographs, the distance between the most proximal point of the lunate and a line perpendicular to the longitudinal axis of the ulna and through its distal articular surface.
• Lunate fossa angle ≥ 40°: On frontal radiographs, the acute angle between the longitudinal axis of the ulna and a line across the lunate fossa of the radius subtracted from 90.
• Palmar carpal displacement ≥ 20 mm: Distance between the longitudinal axis of the ulna and the most palmar point on the surface of the lunate or capitate.

The images show a case where a diagnosis of Madelung deformity was suspected, but only the lunate subsidence was positive.

On MRI, in addition to the radiographic findings, there will be a thickened ligament arising from the ulnar aspect of the distal radius to the lunate, the so-called Vickers ligament.

Differential considerations include:

• Idiopathic Madelung deformity:
• Turner syndrome:
• Dyschondrosteosis: Rare form of mesomelic dysplasia. Characterized by short stature and a Madelung deformity.

References

• Castriota-Scanderbeg A, Dallapiccola B. Chapter 5: Long bones. In Abnormal skeletal phenotypes. Castriota-Scanderbeg A and Dallapiccola B. Springer-Verlag Berlin Heidelberg, 2005. page 313.
• Dawe C, Wynne-Davies R, Fulford GE. Clinical variation in dyschondrosteosis. A report on 13 individuals in 8 families. J Bone Joint Surg Br. 1982;64(3):377-81.
• Dubey A, Fajardo M, Green S, Lee SK. Madelung's deformity: a review. J Hand Surg Eur Vol. 2010 Mar;35(3):174-81.
• Kim HK. Madelung deformity with Vickers ligament. Pediatr Radiol. 2009 Nov;39(11):1251.
• McCarroll HR Jr, James MA, Newmeyer WL 3rd, Manske PR. Madelung's deformity: diagnostic thresholds of radiographic measurements. J Hand Surg Am. 2010 May;35(5):807-12.

Tarsal Tunnel Syndrome

Tarsal tunnel syndrome refers to pain and paresthesia in the plantar aspect of the foot and toes due to nerve entrapment or compression at the level of the posterior tibial nerve or its branches as they pass through the tarsal tunnel.

The flexor retinaculum forms the roof of the tarsal tunnel. Anteriorly, the flexor retinaculum inserts on the anteromedial surface of the medial malleolus and posteriorly, it inserts into the medial tuberosity of the calcaneus. Inferiorly the flexor retinaculum splits to envelop the abductor hallucis muscle.

The floor of the tarsal tunnel is formed by the medial surface of the talus, the sustentaculum tali, and the medial wall of the calcaneus.

The structures of the tarsal tunnel, from anterior to posterior, include the well-known Tom, Dick, and Harry: the tibialis posterior tendon, the flexor digitorum longus tendon, the posterior tibial neurovascular bundle, and the flexor hallucis longus tendon.

Nerve compression can be intrinsic or extrinsic. Intrinsic causes include accessory muscles, ganglion cysts, neurogenic tumors, varicose veins, lipomas, synovial hypertrophy, and scar tissue. Extrinsic causes include foot deformities, hypertrophic and accessory muscles, os trigonum, and excessive pronation in some sports.

References

• Erickson SJ, Quinn SF, Kneeland JB, Smith JW, Johnson JE, Carrera GF, Shereff MJ, Hyde JS, Jesmanowicz A. MR imaging of the tarsal tunnel and related spaces: normal and abnormal findings with anatomic correlation. AJR Am J Roentgenol. 1990 Aug;155(2):323-8.
• Rosenberg ZS, Beltran J, Bencardino JT. From the RSNA Refresher Courses. Radiological Society of North America. MR imaging of the ankle and foot. Radiographics. 2000 Oct;20 Spec No:S153-79.

Pulmonary Langerhans Cell Histiocytosis

Pulmonary Langerhans Cell Histiocytosis is a chronic progressive interstitial lung disease strongly associated with cigarette smoking.

The images here show an upper lobe-predominant process characterized by irregular cysts and nodules in a peribronchiolar distribution. Differential considerations include pulmonary Langerhans cell histiocytosis and lymphangioleiomyomatosis. Emphysema, which shouldn't be a consideration once you note that the cysts have perceptible walls, is included in some differentials.

The classic teaching is that cysts associated with pulmonary Langerhans cell histiocytosis are less uniform in appearance than those associated with lymphangioleiomyomatosis and that Langerhans cell histiocytosis is associated with nodules (especially early in the course of the disease), while lymphangioleiomyomatosis is not. However, more recent (2003) work suggests that there is considerable overlap between the appearance of lymphangioleiomyomatosis and Langerhans cell histiocytosis and that CT can't be reliably used to differentiate the two.

References

• Bonelli FS, Hartman TE, Swensen SJ, Sherrick A. Accuracy of high-resolution CT in diagnosing lung diseases. AJR Am J Roentgenol. 1998 Jun;170(6):1507-12.
• Koyama M, Johkoh T, Honda O, Tsubamoto M, Kozuka T, Tomiyama N, Hamada S, Nakamura H, Akira M, Ichikado K, Fujimoto K, Rikimaru T, Tateishi U, Müller NL. hronic cystic lung disease: diagnostic accuracy of high-resolution CT in 92 patients. AJR Am J Roentgenol. 2003 Mar;180(3):827-35.

Hypoxic-Ischemic Encephalopathy

Hypoxic-ischemic encephalopathy (HIE) is one of the most common causes of severe neurologic deficit in children and occurs in up to about 10/1,000 live births. HIE is thought to occur due to insufficient cerebral blood flow in combination with decreased oxygen content, which leads to loss of normal cerebral autoregulation and diffuse brain injury.

Different patterns of injury have been described in preterm (< 36 weeks) and term (> 36 week) neonates and based on severity. They include:

• Mild-to-moderate hypotension in preterm infants: The periventricular regions are most affected. MR shows areas of T1 hyperintensity within larger areas of T2 hyperintensity. Periventricular leukomalacia (cavitation and periventricular cyst formation) develops 2 to 6 weeks later. End-stage periventricular leukomalacia refers to progressive necrosis of the periventricular tissue with ex-vacuo enlargement of the ventricles and manifests on cross sectional imaging as ventriculomegaly with irregular margins of the bodies and trigones of the lateral ventricles, loss of periventricular white matter with increased T2 signal, and thinning of the corpus callosum.
• Severe hypotension in preterm infants: The thalami, brainstem, and cerebellum in are most affected due to their high metabolic activity in the immature brain. Ultrasound shows hyperechogenicity, CT shows hypoattenuation, and DWI shows restricted diffusion in these regions. T2 signal is variable.
• Mild-to-moderate hypotension in term infants (shown above): The intervascular watershed zones between the anterior and middle cerebral arteries and between the middle and posterior cerebral arteries and the border zone are affected. The parasagittal cortex and subcortical white matter demonstrate restricted diffusion.
• Severe hypotension in term infants: The lateral thalami, posterior putamina, hippocampi, brainstem, corticospinal tracts, and the sensorimotor cortex are affected. These areas may be hyperechoic on ultrasound and low attenuation on CT. The CT findings may be subtle, with the hypoattenuation of the basal ganglia and thalami manifesting as isoattenuation compared to adjacent white matter. MRI will show abnormal T1 hyperintensity, variable T2 signal, and restricted diffusion in these areas.

References

Chao CP, Zaleski CG, Patton AC. Neonatal hypoxic-ischemic encephalopathy: multimodality imaging findings. Radiographics. 2006 Oct;26 Suppl 1:S159-72.

Endobutton

Endobuttons are used to secure the upper end of a tendon graft, for example in anterior cruciate ligament (top images) or biceps tendon (bottom images) reconstruction. A strip of polyethylene tape (black) is looped around the tendon graft (red) on one end and looped around the center two holes of the Endobutton on the other.

References

• Illinois Sportsmedicine & Orthopaedic Centers (video)
• Smith & Nephew.

Pilomatricoma

Pilomatricomas (pilomatrixoma, calcifying epithelioma of Malherbe) are benign calcifying tumors that arise from skin appendages and are the most common solid cutaneous tumor in patients under 20 years of age. Imaging is generally not used in the evaluation of pilomatricomas, since they are usually superficial, small, and well-circumscribed.

Pilomatricomas are slow-growing subcutaneous tumors that are usually less than 3 cm in diameter. The majority are found in the head and neck, followed by the upper extremities.

Radiographic features of pilomatricomas include regular contours, sharp demarcation from surrounding fat, and a normal overlying skin. Calcifications, when present, are usually speckled or sand-like, but can be large and dense in some cases.

Different ultrasound features have been described. Classically, these are hypoechoic solid nodules that are either fully or partially calcified. However, Pilomatricomas may also have internal fluid signal and a thick-wall.

MRI reveals a mass that is homogeneously isointense to muscle on T1-weighted images. Pilomatricomas can be hypointense to muscle on T2-weighted images with patchy hyperintense areas and striations that radiate from the center toward a hyperintense peripheral rim. Post-contrast images may reveal patchy or reticular enhancement centrally and peripheral enhancement of a connective tissue capsule. A case pilomatricoma without enhancement has also been reported. Peritumoral edema may be seen in some cases.

The case shown here is from a young woman with a longstanding history of a painless, hard bony mass in the left preauricular region. The images are not very exciting, as they are basically blacked out by the calcium, which goes along with the history of a hard bony mass.

References

• De Beuckeleer LH, De Schepper AM, Neetens I. Magnetic resonance imaging of pilomatricoma. Eur Radiol. 1996;6(1):72-5.
• Gustin AF, Lee EY. Pilomatricoma in a pediatric patient. Pediatr Radiol. 2006 Oct;36(10):1113.
• Haller JO, Kassner G, Ostrowitz A, Kottmeler K, Perfschuk LP. Pilomatrixoma (calcifying epithelioma of Malherbe): radiographic features. Radiology. 1977 Apr;123(1):151-3.
• Hoffmann V, Roeren T, Möller P, Heuschen G. MR imaging of a pilomatrixoma. Pediatr Radiol. 1998 Apr;28(4):272.
• Lim HW, Im SA, Lim GY, Park HJ, Lee H, Sung MS, Kang BJ, Kim JY. Pilomatricomas in children: imaging characteristics with pathologic correlation. Pediatr Radiol. 2007 Jun;37(6):549-55.
• Solivetti FM, Elia F, Drusco A, Panetta C, Amantea A, Di Carlo A. Epithelioma of Malherbe: new ultrasound patterns. J Exp Clin Cancer Res. 2010 May 6;29(1):42.

Duodenal Filling Defects

Small

• Lymphoid hyperplasia: Multiple Little (1 mm - 2 mm) filling defects. May be normal in children. May be associated with giardiasis in adults with hypogammaglobunimeia.
• Heterotopic gastric mucosa: Multiple mini (few mm) filling defects clustered in a mosaic pattern.

Large

• Pancreatic rest: Ectopic pancreatic tissue. Smooth (round or lobulated) filling defects 1 cm - 2 cm in diameter. Classical appearance of a central dimple of barium that can be confused with an ulcer. Usually found in the bulb or descending duodenum.
• Brunner gland hyperplasia: Multiple, Big (up to 1 cm or so), rounded filling defects at the bulb that can extend to the descending duodenum. Often produce a cobble stone appearance.

Miscellaneous/Uncommon

• Flexural pseudopolyp: Usually in thin patients. Due to sharp angle between the apex of the duodenal bulb and the descending duodenum, which causes mucosa to bunch up at the apex of the bulb.
• Adenoma: Uncommon. Mucosal polypoid lesions with high potential for malignant degeneration. Usually smaller than 1 cm. Villous adenomas can get larger than 1 cm and are more likely to be found in the periampullary region.
• Mesenchymal tumors: Leiomyoma or lipoma. Most common benign lesions of duodenum. Smooth, submucosal lesions. Ulceration uncommon.
• Kaposi: Multiple polypoid and ulcerated lesions.
• Carcinoid: Typically in the descending duodenum, peripapillary region.
• Choledochocele: Causes extrinsic compression.
• Papillitis: Enlarged major papilla due to inflammation, usually recent passage of a calculus.