By Z. Tippler. New England College of Optometry.
The congenital developmental disorder progresses slowly These lesions increase in size as the patient grows discount combivent 100 mcg amex. The as the child grows and normally comes to a halt on condition can affect a single or multiple bones and buy combivent 100 mcg, very completion of growth discount 100mcg combivent with amex. The condition is usually diagnosed rarely generic 100mcg combivent visa, is associated with endocrine abnormalities such as during the first decade, although often not until the sec- precocious puberty, premature physeal closure and hy- ond decade. When associated with hormonal disorders but most commonly affect the proximal metaphysis of the condition is known as Albright syndrome. Several lesions can also occur dystrophia fibrosa, osteitis fibrosa disseminata, Mc- simultaneously in one bone. The condition progresses Cune-Albright syndrome, Jaffé-Lichtenstein disease asymptomatically unless a pathological fracture occurs or bowing is outwardly visible (⊡ Fig. Historical background Bowing particularly affects the proximal femur, where the Polyostotic fibrous dysplasia was first described by Lich- soft bone can bend into the shape of a »shepherd’s crook« tenstein in 1938. Clinically relevant leg length discrepancies merged the polyostotic and monostotic forms under can also occur. The osteolytic areas, the cortex is thin and bulges out, usually individual lesions are generally asymptomatic and do the whole bone is widened and the basic structure shows a not require treatment. Only if symptoms, fractures or ground glass opacity in the osteolytic zones. This ground pronounced bowing occurs are therapeutic measures in- glass pattern is attributable to the formation of new bone. A suitable Osteolytic and sclerotic components appear next to each solution for stabilization is an intramedullary load-bear- other. The cortical bone is eroded and the bone is wid- ing implant in the area of the femoral neck, or so-called ened as a result of new periosteal bone formation. Mi- »Y nail« or Gamma-nail (also known as a »Zickel nail«) crofractures also occur and can lead to painful episodes. In children with open epiphyseal plates tively signal-rich in both the T1- and T2-weighted images, the new telescopic Gamma-nail can be used (⊡ Fig, 4. In the McCune-Albright syndrome the polyostotic fi- If doubt exists, the diagnosis must be confirmed by bi- brous dysplasia is accompanied by abnormal skin pigmen- opsy before such a radical measures is undertaken. Since tations that resemble the café-au-lait spots in neurofibro- fracture and osteotomy healing is not usually impaired, matosis. Girls experience a precocious puberty, resulting surgical measures are only rarely indicated. A biopsy is in a small stature as a result of the premature epiphyseal only indicated prior to surgical procedures. Other hormonal disorders such as hyperthyroid- are asymptomatic, the current imaging procedures are ism can occur, and cortisone metabolism may also be sufficient for establishing the diagnosis. Fibrodysplasia ossificans progressiva Differential diagnosis > Definition Individual foci are not always easy to differentiate radio- This is an autosomal-dominant inherited disorder (gene logically from solitary bone cysts, since the latter also show locus 4q27–31) characterized by progressively increasing swelling of the bone with an osteolytic lesion and inter- calcification and ossification of the fasciae, aponeuroses, vening bone trabeculae. However, the ground glass opac- tendons and ligaments and shortening of the great toes. Note that both lesions can show a strong signal on the MRI scan because of the fluid content. The etiology involves the abnormal induction of en- Another important differential diagnosis to consider chondral osteogenesis in connective tissue. This bone morphogenic protein content (BMP) of the cells condition occurs almost exclusively on the tibia and is is increased. If a polyostotic form of fibrous ly ossifications – occur primarily in the interstitial dysplasia is present, however, there is little possibility of connective tissue and in the tendons and ligaments, confusion. Enchondromatosis and histiocytosis, which also but not in the actual muscles. The use of the term affect multiple bones, usually differ markedly in their ap- »myositis« in this context is therefore misleading. Enchondromas only produce mini- Occurrence: The disease is very rare with only a few mal swelling of the bone and tend to form calcifications. A total of 44 cases were While the appearance of histiocytosis varies considerably, described in a meta-analysis.
The barium enema examination is indicated for any pathology that may result in large bowel obstruction (e quality 100 mcg combivent. A low osmolar iodine- based contrast agent should be used in preference to a barium preparation when examining neonates and young infants or when bowel perforation is suspected buy combivent 100 mcg low price. However combivent 100mcg low price, if a high osmolar contrast agent is used then care should be taken to avoid dehy- dration of the neonate purchase 100mcg combivent with visa. The child’s age and suspected pathology inﬂuence the choice of radiographic technique employed. When examining very young children, a single-contrast examination will provide a diagnosis in the majority of cases whereas in the examination of older children, or where inﬂammatory bowel disease is sus- pected, a double-contrast technique should be used. In a single-contrast examination, the patient should lie on their left side with their hips and knees ﬂexed. A soft rubber catheter is gently inserted into the rectum and taped into position. The patient maintains the lateral position while a 30–100g/100ml suspension of barium sulphate10, warmed to body tempera- ture, is introduced slowly under gravitational force. Progress of the contrast agent through the bowel is monitored ﬂuoroscopically and images taken to demonstrate large bowel anatomy. Routine images might include a lateral pro- jection of the rectum, right and left posterior oblique projections for the splenic and hepatic ﬂexures and an antero-posterior projection to demonstrate the caecum and terminal ileum. A double-contrast technique is similar to the above except that a higher con- centration barium sulphate suspension, 60–120g/100ml, is used and the tech- nique also includes air insufﬂation. Antero-posterior projections in the prone position, with 45° caudal angulation of the central ray to show the sigmoid colon, and lateral decubitus projections, may be required for a complete study, but are not routinely taken. In these cases, anti- spasmodic agents may be given prior to examination to relax the bowel after which air at a pressure not exceeding 80mmHg is insufﬂated over 3 minutes. The child should be rested for 3 minutes before repeating this procedure. At no time should the pressure exceed 120mmHg10 and a maximum of three attempts should be made. In a successful examination, ﬂuoroscopy will demonstrate air bubbling through the site of the intussusception. Surgical reduction may be required if the image-guided reduction attempt fails, and surgical staff should be made aware of the procedure in case of a surgical emergency. Contraindica- tions to the air enema are suspected perforation or peritonitis. Renal tract examinations Intravenous urography Ultrasound is the initial imaging examination of choice for renal tract pathology in the child and intravenous urography (IVU) is required only when less inva- sive procedures have failed to provide adequate diagnostic information. Prior to administration of a contrast agent, the child should be weighed and the dose calculated in accordance with the manufacturer’s instructions on 90 Paediatric Radiography volume and concentration in terms of iodine content per kilogram of body weight. A topical local anaesthetic should be applied to several potential injection sites at least 1 hour prior to radiographic examination to facilitate intravenous puncture or, alternatively, the contrast agent may be administered through an existing intravenous line where one is already in situ. It is standard practice to starve the patient for 4 hours prior to the adminis- tration of a contrast agent in order to ensure that the stomach is empty. However, it is important that patients, particularly children, remain well hydrated and clear ﬂuids should not be restricted. Flexibility in examination appointment times, particularly for infants and young children, will be necessary so that the examination can be timed for when the stomach is likely to be empty (i. Following contrast agent injection, infants may be bottle-fed to help pacify them. The ﬂuid-ﬁlled stomach will effectively form a radiographic ‘window’ facilitating the visualisation of the renal area. Each IVU examination should be tailored to the individual patient and directed to answer a speciﬁc clinical question13 thereby ensuring that the number of radiographic images taken is kept to a minimum. Ideally, the renal tract should be visualised free from overlying bowel gas and faeces, and the use of ureteric compression and oblique projections may be required to achieve this. Oral car- bonated drinks can be used in older children to distend the stomach and provide a gaseous ‘window’ through which the kidneys may be visualised; the antero- posterior projection with the patient supine demonstrating the left kidney while a right posterior oblique will demonstrate the right kidney.