Osteogenesis imperfecta
OI and sometimes known as Brittle Bone Disease, or ‘Lobstein syndrome’.Osteogenesis imperfecta is disorder of congenital bone fragility caused by mutations in the genes that codify for type I procollagen. It is a common heritable disorder of collagen synthesis that results in weak bones that are easily fractured and are often deformed. It is also known as Brittle Bone Disease, or ‘Lobstein syndrome’. This condition affects an estimated 6 to 7 per 100,000 people worldwide. Several distinct subtypes have been identified. All of them lead to micromelic (short-limbed) dwarfism of varying degree. Depending on severity, the bone fragility may lead to perinatal death or cause severe deformities that persist into adulthood. A wide array of clinical manifestations of the disease may be seen. These partly depend on the genetic subtype. Types I and IV are the most common forms of osteogenesis imperfecta, affecting 4 to 5 per 100,000 people.

The following 4 types of osteogenesis imperfecta have been reported. Type I - mild forms, type II - extremely severe, type III, severe type IV – undefined.

People with this disease are born with defective connective tissue, or without the ability to make it, usually because of a deficiency of Type-I collagen. This deficiency arises from an amino acid substitution of glycine to bulkier amino acids in the collagen triple helix structure. The larger amino acid side-chains create steric hindrance that creates a "bulge" in the collagen complex. As a result, the body may respond by hydrolyzing the improper collagen structure. If the body does not destroy the improper collagen, the relationship between the collagen fibrils and hydroxyapatite crystals to form bone is altered, causing brittleness. Another suggested disease mechanism is that the stress state within collagen fibrils is altered at the locations of mutations. These recent works suggest that osteogenesis imperfecta must be understood as a multi-scale phenomenon, which involves mechanisms at the genetic, nano-, micro- and macro-level of tissues.
In osteogenesis imperfecta, the modes of inheritance, family history, clinical features, and radiologic findings vary.Four distinct types are identified: type I, which is the dominantly inherited form with blue sclerae; type II, which is the perinatal lethal form; type III, which is the progressively deforming form with normal sclerae; and type IV, which is the dominantly inherited form with normal sclerae.
In general, type I is the mildest form of disease; type IV, type III, and type II, respectively, increase in severity.



As a genetic disorder, Ti is an autosomal dominant defect. Most people with OI receive it from a parent but it can be an individual (de novo or "sporadic") mutation. Osteogenesis imperfecta is relatively rare. In some cases, the parent has osteogenesis imperfecta and the condition has been genetically transmitted to the child. But, the child's symptoms and the degree of disability could be very different from that of the parent. In some children, neither parent has osteogenesis imperfecta. In these cases, the genetic defect is a spontaneous mutation.
The primary pathology in osteogenesis imperfecta is a disturbance in the synthesis of type I collagen, which is the predominant protein of the extracellular matrix of most tissues. In bone, this defect of extracellular matrix causes osteoporosis, which leads to an increase in the tendency to fracture. Besides bone, type I collagen is also a major constituent of dentin, sclerae, ligaments, blood vessels, and skin; therefore, individuals with OI may also have abnormalities of these structures.
The process of collagen molecule formation starts with the synthesis of procollagen. This precursor consists of a long triple-helix protein flanked by 2 propeptides at its 2 terminals. Procollagen is synthesized and then secreted into the extracellular compartment, where the amino- and carboxy-terminal propeptides are cleaved; thus, the functional collagen molecule is formed. These molecules then assemble into an ordered fibril. Mutations that interfere with expression of the collagen gene, formation of the triple helix (amino acid sequencing), or procollagen secretion affect the structure and function of collagen fibrils, resulting in a form of OI.
Electron microscopic studies of OI demonstrate a decrease in the diameter of the collagen fibril, relative to the collagen fibril of healthy persons, and smaller-than-normal apatite crystals.
A number of genetic defects cause the abnormal type I collagen synthesis that leads to OI. OI generally arises from mutations in 1 of 2 genes that encode for the synthesis and/or structure of type I collagen: the COL1A1 gene on chromosome 17, and the COL1A2 gene on chromosome 7. Mutations in these genes may cause abnormal collagen to be produced and may lead to a decrease in the production of normal collagen. The varying degree to which these 2 factors manifest themselves results in the different phenotypic expressions of OI. Milder forms of OI are caused primarily by a decrease in production of normal collagen, whereas more severe forms are caused primarily by the production of abnormal collagen. These abnormalities may be dominantly inherited, or they may be the result of sporadic mutation.
Common causes of nonorthopedic morbidity in type I and type IV OI are joint hypermobility, which causes chronic joint pain, hearing impairment, and brainstem compression.Children with type III OI often require orthopedic care because of their progressive deformities. Standing and walking are often impossible because of spinal compression fractures and scoliosis. Progressive thoracic deformities are associated with recurrent pneumonias that often limit the patient's lifespan.


Type I: The life expectancy of patients with all forms of OI other than type III is often assumed to be shortened. However, according to Paterson et al, the life expectancy of patients with OI type IA is the same as that of the general population. Type IA is a subtype of type I OI in which dentinogenesis imperfecta (tooth abnormalities) does not occur. Type IB is a rare form of type I OI in which dentinogenesis imperfecta does occur. In types IB and IV, mortality is modestly increased in comparison with that of the general population; there is no statistically significant difference in life expectancy. Type II: This form of OI is fatal in the perinatal period.

Type III: Only in type III OI is life expectancy affected. However, patients with type III OI who survive beyond the age of 10 years have a better outlook than other patients with OI.
Osteogenesis imperfecta does not seem to have a predilection for any particular race. No known sex predilection is reported for osteogenesis imperfect. The onset of fractures and deformities varies according to the type of osteogenesis imperfecta (OI) that is present.
For type I, the age of onset is variable. This form most commonly appears during the preschool years when the child is starting to stand. Onset after puberty is uncommon, although fractures may recur in adulthood after menopause or after periods of inactivity, such as after childbirth. Type II occurs in utero. In type III, abnormalities are present at birth (ie, abnormalities develop in utero) in more than 50% of patients. Fractures are frequent during the first 2 years of life.Type IV abnormalities are present at birth in approximately 30% of patients. The onset of this form is during infancy or the preschool years.
The clinical features of osteogenesis imperfecta (OI) depend on the type, but bone fragility with multiple fractures and bony deformities are the common hallmark of all types.
The major presenting signs and symptoms of OI include blue sclerae, hearing loss, tooth abnormalities (dentinogenesis imperfecta), joint laxity, and abnormal skin texture (smooth and thin skin). Other features that are common to multiple OI types include bleeding diathesis (easy bruising) and respiratory distress.
OI is classified into 4 distinct types: I-IV. Some cases of OI do not fit easily into any of the 4 types. A type V category has been added to include patients with osteoporosis or interosseous membrane ossification of the forearms and legs, as well as patients who are prone to the development of hypertrophic calluses.




The type 1 prototypical and most common form of OI is associated with the best prognosis. The mode of inheritance is autosomal dominant. The distinguishing clinical features of type I are blue sclerae, which occurs in patients of all ages, and presenile conductive hearing loss; in addition, most patients with type I OI have a family history of hearing loss. Bone fragility is mild, and there are minimal bony deformities. The stature of patients with type I OI is often normal or near normal. Ligamentous hyperlaxity, resulting in joint hypermobility or subluxation, is common. Approximately 20% of patients have kyphoscoliosis.
Dentinogenesis imperfecta is present in some families but not in others.12 Therefore, type I OI is subclassified to distinguish patients without dentinogenesis imperfecta (type IA, more common) from those with dentinogenesis imperfecta (type IB, rare). Some investigators have suggested that these 2 subgroups are biochemically distinct and that individuals with OI type IB, whose bodies make structurally abnormal collagen, are more similar to those with OI type IV than to those with other types of OI, including type IA.
Type II is the most severe form of OI. It is characterized by extreme bone fragility that almost invariably leads to intrauterine or early infant death. The cause of death is most often respiratory failure. The mode of inheritance is autosomal recessive. The sclerae are blue and occasionally dark blue or black. Clinically distinguishing features include intrauterine growth retardation, thin and beaded ribs, crumpled long bones, and limited cranial and/or facial bone ossification. Limbs are short, curved, and angulated.
Type II OI can be further subdivided into types IIA, IIB, and IIC on the basis of the radiographic features of the long bones and ribs. Patients with type IIA or IIC inevitably die in the perinatal period; rarely, patients with type IIB survive into early childhood.
Type III is the next most severe form of OI after type II. It is the most severe form in which survival extends beyond the perinatal period.
Its hallmark feature is severe bone fragility and osteopenia, which is progressively deforming. The mode of inheritance is thought to be autosomal recessive. Multiple fractures and progressive deformity affect the long bones, skull, and spine and are often present at birth. Postnatal growth failure is severe. Kyphoscoliosis is common. Sclerae are either normal from birth, or they progress from pale blue in infancy to a normal appearance by adolescence.
Type III OI is probably the form that is best known to radiologists and orthopedic surgeons. Children with type II OI tend to have severe dwarfism caused by spinal compression fractures, limb deformities, and disruption of growth plates.
Type IV OI is distinguished from type I OI by the slightly increased, though still variable, severity of bone fragility and by the presence of normal sclerae. The mode of inheritance is autosomal dominant. Mild to moderate bony deformity of the long bones and spine is present; the incidence of fracture is variable. Basilar impression of the skull, with consequent brainstem compression, is common; it is reported in 70% of patients.


Hearing loss or a family history of hearing loss is noted in patients with this type of OI, as is dentinogenesis imperfecta. Type IV OI is also subclassified to distinguish patients without dentinogenesis imperfecta (type IVA) from those with it (type IVB). Compared with type I OI, hearing loss is less common in type IV, and dentinogenesis imperfecta (type IVB) is more common. Some authors have distinguished a self-limiting variant of OI, known as temporary brittle-bone disease. Its clinical features are identical with those found in cases of child abuse.
While there is no cure for osteogenesis imperfecta, there are opportunities to improve the child's quality of life. Treatment must be individualized and depends on the severity of the disease and the age of the patient. Care is provided by a team of health-care professionals, including several types of doctors, a physical therapist, a nurse-clinician and a social worker.
In most cases, treatment will be nonsurgical.
Medical bisphosphonates, given to the child either by mouth or intravenously, slow down bone resorption. In children with more-severe osteogenesis imperfecta, bisphosphonate treatment often decreases the number of fractures and bone pain. These medications must be administered by properly trained doctors and require close monitoring.
Casting, bracing, or splinting of fractures is necessary to immobilize the bone so that healing can occur. Movement and weight bearing are encouraged as soon as possible after fractures to increase mobility and decrease the risk of future fractures.
In surgical treatment, repeated fractures of the same bone, deformity, or fractures that do not heal properly are all indications that surgery may be necessary. Metal rods may be inserted in the long bones of the arms and legs. Some rods are a fixed length and must be replaced as the child grows. Other rods are designed like telescopes so they can expand along with the bone growth. However, other complications may occur with telescoping rods.
In many children with osteogenesis imperfecta, the number of times their bones fracture decreases significantly as they mature. However, osteogenesis imperfecta may become active again after menopause in women or after the age of 60 years in men. Scoliosis, or curvature of the spine, is a problem for many children with osteogenesis imperfecta. Bracing is the usual treatment for scoliosis, but it is often ineffective in children with osteogenesis imperfecta. Spinal fusion, in which the vertebrae are realigned and fused together, may be recommended to prevent excessive curvature.
At present there is no cure for OI. Treatment is aimed at increasing overall bone strength to prevent fracture and maintain mobility.
There have been many clinical trials performed with Fosamax (Alendronate), a drug used to treat women experiencing brittleness of bones due to osteoporosis. Higher levels of effectiveness apparently are to be seen in the pill form versus the IV form, but results seem inconclusive.

Bone infections are treated as and when they occur with the appropriate antibiotics and antiseptics.
Physiotherapy used to strengthen muscles and improve motility in a gentle manner, while minimizing the risk of fracture. This often involves hydrotherapy and the use of support cushions to improve posture. Individuals are encouraged to change positions regularly throughout the day in order to balance the muscles which are being used and the bones which are under pressure.
Children often develop a fear of trying new ways of moving due to movement being associated with pain. This can make physiotherapy difficult to administer to young children. With adaptive equipment such as crutches, wheelchairs, splints, grabbing arms, and/or modifications to the home many individuals with OI can obtain a significant degree of autonomy.
Spinal fusion can be performed to correct scoliosis, although the inherent bone fragility makes this operation more complex in OI patients. Surgery for basilar impressions can be carried out if pressure being exerted on the spinal cord and brain stem is causing neurological problems.
Because osteoporosis and multiple fractures are hallmark features of osteogenesis imperfecta (OI), other disorders that cause multiple fractures or decreased bone mineralization may be considered in the differential diagnosis. Such disorders including juvenile osteoporosis, steroid-induced osteoporosis, menkes (kinky-hair) syndrome, hypophosphatasia, battered child syndrome (syndrome X), temporary brittle-bone disease.

References:
http://emedicine.medscape.com/article/947588-overview

http://emedicine.medscape.com/article/411919-overview

http://ghr.nlm.nih.gov/condition=osteogenesisimperfecta

http://orthoinfo.aaos.org/topic.cfm?topic=A00051

http://hwmaint.jmg.bmj.com/cgi/content/abstract/16/2/101

N.B.: This article is excerpted from the book MUSCULOSKELETAL INJURIES for UNDERGRADUATES

Date Palm Synovitis

Date Palms




The date palm is common in the Sultanate of Oman and neighboring Gulf countries. The history goes back 600 years to Eridu in lower Mesopotamia where the first evidence of date cultivation has been found. Closer to this oasis, the Hilli settlements of Al-Ain in the Arabian Peninsula seems to be cultivated dates some 5000 years back. In Bidiyah, in the eastern region of the Sultanate of Oman, dates are the main-stay of the economy. Date growing is labor intensive in Oman and very traditional in its method.

To fertilize the female flowers (pistils), each date palm must be climbed and the pistils, which are clustered to the center of the leaves, pollinated. Each date palm provides the farmer with natural steps to climb the trunk. These are formed from the base of the previous season’s leaf stalks which were cut off. The technique of climbing is simple but since a date palm can be 30 meters tall, a key requirement is good head for height. The worker has to climb the tree once more in the later months in order to obtain the fruit that is now ready to be harvested.

Synovitis

Synovitis is the medical term for inflammation of the synovial membrane. This membrane lines joints which possess cavities, known assynovial joints. The condition is usually painful, particularly when the joint is moved. The joint usually swells due to synovial fluid collection.
Synovitis may occur in association with arthritis as well as lupus, gout, and other conditions. Synovitis is more commonly found in rheumatoid arthritis than in other forms of arthritis, and can thus serve as a distinguishing factor, although it can present to a lesser degree in osteoarthritis. Long term occurrence of synovitis can result in degeneration of the joint.

Date Palm Synovitis

Joint inflammation associated with intra-articular retention of a date palm thorn.

Synonyms

Date Palm Knee

Incidence

Uncommon in the northern hemisphere and in developed countries. It is much more common in Sultanate of Oman and neighbouring Gulf countries where traditional agricultural practices require climbing palm trees. Because the original injury may have been forgotten, this diagnosis should be considered in mono-articular inflammation in children.

Differential Diagnosis

Septic arthritis

( This can be differentiated by doing a simple blood investigation.
There will be normal WBC and ESR in Date Palm Synovitis, meanwhile in septic arthritis there will increase in both WBC and ESR)

Pathogenesis

A penetrating injury into the joint (usually the knee) results from a minor wound from a the thorn. The date palm tree bears thorns 10-15cm long, which can easily pierce the joint cavities.



If the thorn breaks off inside the joint, an acute, sub-acute or chronic inflammation of the joint may result. Many infective agents have been associated with date palm thorn. with no one predominating bacterium. Staphylococcus aureus has been found commonly but this is thought to be secondary infection following attempts at self-treatment.

Pathology

The arthritis may be either septic or sterile. It is unknown whether the primary features are due to infection or to an immune response to the foreign material in the vegetable matter. The reason for this is not clear, but alkaloids in the thorns are a possible cause (Stromqvist, Edlund and Lidgren 1985). The pathological features are those of acute inflammatory synovitis. Chronic synovitis develops if the condition persists.

Macroscopically,
• Redness, swelling, tenderness, loss of range of motion
• May settle to a chronic effusion with thickened boggy synovium
• May progress to a septic arthritis. There may also be a soft tissue infection leading to fasciitis. Examine for local, distant and systemic signs of infection – pyrexia, malaise, lymphadenopathy, cellulitis
• Rare presentation as locking, mimicking IDK with the thorn itself causing the locking




Microscopically,


Synovium from to two cases requiring partial synovectomy showed a non-specific synovitis. (Haematoxylin and eosin)



Synovial tissue from knee of patient with thorn-induced synovitis (hematoxyline- phloxine-saffron). Top: Heavy fibrin deposits (F) on surface and intenae infiltration of inflammatory cell (original maginification x 120, reduced approximately 25%). Bottom: Foreign material (arrow) in synovium, surrounded by numerous giant cells, seen under polarized light, material is highly refractile, consistent with plant thorn matter (original magnification x 540)

Stages

Acute
Infected
Inflammatory
Sub-acute (> 1 week)
Chronic
Non-specific

Classification

None encountered in the literature. Useful classifications could be devised using time, aetiological agent, infected/sterile or extent of the condition.

Clinical Features

Palm thorn synovitis is usually mild, the initial symptoms are often intolerated, delaying presentation for treatment.
The clinical features are:

• Puncture wound or history (may be absent)
• Redness, swelling, tenderness, loss of range of motion
• May settle to a chronic effusion with thickened boggy synovium
• May progress to a septic arthritis. There may also be a soft tissue infection leading to fasciitis. Examine for local, distant and systemic signs of infection – pyrexia, malaise, lymphadenopathy, cellulitis
• Rare presentation as locking, mimicking IDK with the thorn itself causing the locking

Investigation

CRP, aspiration and culture may identify an infective process and an organism but treatment of the infection may not resolve the problem unless the presence of the foreign body is detected. But normally, the WBC and ESR is within normal limits and no organisms was grown from any joint aspirate.

Depending on the stage fluid aspirated from the joint will have acute or chronic inflammatory cells but other rheumatological investigations will be negative.
Xrays are most often negative apart from a synovial effusion as thorns cannot be seen on radiographs. CT scan has been claimed to be diagnostic. MR scan is reliably diagnostic for this condition as the foreign body ( thorns) shows up well.

Prognosis untreated

In the acute infected case the prognosis is that of acute septic arthritis
For sterile cases and indolent infections the condition may settle to a chronic mono-arthritis with eventual secondary OA.
Since the condition is provoked by the presence of foreign material it will not settle completely until the foreign material is removed or eliminated.

Non-Operative Treatment

Appropriate antibacterial treatment.
Symptomatic treatment with analgesics and anti-inflammatory medication.
Steroid injection contra-indicated

Operative Treatment

Transarthroscopic excision of the loose body
Open or transarthoscopic synovectomy
Surgical treatment of septic arthritis

Complications

Chronic arthritis
Secondary OA
Sepsis

Outcomes

Favourable outcome after early recognition and surgical treatment
Literature suggests that synovectomy may be necessary after development of chronic synovitis i.e. that removal of the foreign body may not be enough.

Bibliography

1. Clough J.F.M. (1999) Cactus Knee Orthopaedic Rare Conditions Internet Database (ORCID) http://www.orthogate.org/orcid/aspercases.htm
Has an extensive bibliography on this subject

2. Maillot F, et al.
Plant thorn synovitis diagnosed by magnetic resonance imaging.
Scand J Rheumatol. 1994;23(3):154-5.


3. Doig SG, et al.
Plant thorn synovitis. Resolution following total synovectomy.
J Bone Joint Surg [Br]. 1990 May;72(3):514-5.

4. Klein B, et al.
Thorn synovitis: CT diagnosis.
J Comput Assist Tomogr. 1985 Nov-Dec;9(6):1135-6.


5. Ramanathan EB, et al.
Date palm thorn synovitis.
J Bone Joint Surg [Br]. 1990 May;72(3):512-3.

6. Olenginski TP, et al.
Plant thorn synovitis: an uncommon cause of monoarthritis.
Semin Arthritis Rheum. 1991 Aug;21(1):40-6.


7. Vaishya R.
A thorny problem: the diagnosis and treatment of acacia thorn injuries.
Injury. 1990 Mar;21(2):97-100.

8. Adams CD, Timms FJ, Hanlon M.
Phoenix date palm injuries: a review of injuries from the Phoenix date palm treated at the Starship Children's Hospital. Aust N Z J Surg. 2000 May;70(5):355-7.


9. Miller EB, Gilad A, Schattner A.
Cactus thorn arthritis: case report and review of the literature.
Clin Rheumatol. 2000;19(6):490-1.

10. Labbe JL, Bordes JP, Fine X.
An unusual surgical emergency: a knee joint wound caused by a needlefish. Arthroscopy. 1995 Aug;11(4):503-5.

This article was contributed by Ms Maisrah as an e learning exercise

ORTHOPEDIC MANIFESTATIONS DURING PREGNANCY

Almost all pregnant women experience musculoskeletal discomfort during pregnancy,
with a good portion of them suffering from severe disability. The enlarging gravid uterus alters
the maternal body's center of gravity, mechanically stressing the axial and pelvic systems, and compounds the stresses that hormone level fluctuations and fluid retention exert. While the pregnant woman
is prone to many musculoskeletal injuries, most can be controlled conservatively, but some require emergent surgical intervention.

Often, the source of these musculoskeletal problems can be traced to an endocrine disorder.
For example, carpal tunnel syndrome is not uncommon in patients who are pregnant or
have diabetes, hypothyroidism, or acromegaly. Joint problems and arthritis
are other common findings in diabetes, pregnancy, and hyperparathyroidism. Muscle weakness or stiffness is seen in both hypothyroidism and hyperthyroidism, and muscle wasting is a characteristic of adrenocorticoid insufficiency. Bone disorders are common with glucocorticoid excess, acromegaly, and hyperparathyroidism. Some presentations are a classic picture of a specific endocrine condition and are readily recognized if the index of suspicion is appropriately high.

During pregnancy, certain anatomical and hormonal changes occur that produce
increased stress on the pelvic
articulations resulting in the development of pelvic girdle relaxation. Pelvic girdle relaxation during pregnancy is physiological and is caused by hormonal and biomechanical factors. When a pregnant woman presents as
a patient with low back or pelvic pain, walking dysfunction and with reproduction
of pain with sacroiliac provocation, the diagnosis of symptomatic pelvic girdle relaxation can be madeThe gravid uterus
and the compensatory lordosis that it
causes create a tremendous mechanical
burden on the lower back. Joint laxity increases during pregnancy. The hormone relaxin has been identified as a major contributor to joint laxity during pregnancy. It decreases the intrinsic strength of the connective tissue allowing it to expand and lose its rigidity, resulting in increased widening and sliding mobility of the joints, thus causing potential instability. This occurs especially in the ligaments of the sacroiliac and pubic symphysis joints,
but may also occur in peripheral joint.
This may result in pubic symphysis pain,
low back pain or hip pain.

Pubic Symphysis Pain

Pathology: Separation of the pubic symphysis joint (diastasis or symphysiolysis), as a result of pelvic girdle relaxation, is thought to be the main cause of pubic symphysis pain. Relaxin levels were found
to be significantly higher in pregnant
women with pelvic pain and joint laxity.
The highest level was found in those women with the most severe clinical symptoms, who also took a longer time to recover after pregnancy. Swelling within the joint, ligament disruption and hemorrhage have
also been suggested to cause pubic symphysis pain. The severity of these conditions varies from mild self-limiting pain to a severe disabling condition. Lack of awareness and failure of recognition of these complications by obstetricians not only results in women feeling very lonely and misunderstood, but may also result in long-term morbidity.

Presentation: Patients may present during pregnancy (usually in the second or third trimester), during labour or 24-48 hours postpartum, with a sudden or insidious
onset of pain of variable severity in the pubic area or groin which may radiate to
the medial aspect of the thigh and increases on weight-bearing. Pain may occur also in the hips, suprapubic area or the lower back and be aggravated by walking, standing, stairs climbing, parting of the legs or turning in bed.

Clinically, a waddling gait or limp may be noticed. The woman may not be able to stand comfortably on one leg. Abduction of the thigh is usually painful. Point tenderness in the region of the pubic symphysis and pain on compression of the pelvis by simultaneous pressure on both trochanters are usually present. Care must be taken as exquisite pain may occur on palpation of
the pubic symphysis, which may also reveal
a gaping pubic defect and edema.

The symptoms (and their severity) experienced vary, but include:

. Present swelling and/or inflammation over joint.

. Difficulty lifting leg.
. Pain pulling legs apart.
. Unable to stand on one leg.
. Unable to transfer weight through pelvis and legs.

. Pain in hips and/or restriction of hip movement.

. Transferred nerve pain down leg.
. Can be associated with bladder and/or bowel dysfunction.

. A feeling of symphysis pubis giving way.
. Stand with a stooped over back.
. Mal-alignment of pelvic and/or back joints.

. Struggle to sit or stand.
. Pain may also radiate down the inner thighs.

. You may waddle or shuffle.
. Aware of an audible ‘clicking’ sound coming from the pelvis.

Psychosocial impact - interferes with participation in society and activities of daily life; the average sick leave due to posterior pelvic pain during pregnancy is 7 to 12 weeks. In some cases patient may also experience emotional problems such as anxiety over the cause of pain, resentment, anger, lack of self-esteem, frustration and depression; she is three times more likely to suffer postpartum depressive symptoms. Other psychosocial risk factors associated with woman experiencing PGP include higher level of stress, low job satisfaction and poorer relationship with spouse.

Diagnosis of pubic symphysis separation is based on the clinical presentation and the response to therapy. Imaging (X-ray, ultrasound o r magnetic resonance [MRI])
may be useful in confirming the diagnosis. Ultrasound examination using a 7.5 MHz or 5 MHz linear array transducer may demonstrate widening of the interpubic gap in excess of 10 mm. Ultrasound has many advantages over conventional X-ray, as it can be done during pregnancy and can be repeated safely for follow-up. However, the amount of symphyseal separation does not always correlate with the severity of the symptoms, or the degree of disability, nor does it appear to
predict outcome.

Severity - The severity and instability of the pelvis can be measured on a three level scale.

Pelvic type 1: The pelvic ligaments support the pelvis sufficiently. Even when the muscles are used incorrectly, no complaints will occur when performing everyday activities. This is the most common situation in persons who have never been pregnant, who have never been in an accident, and who are not hyperactive.
Pelvic type 2: The ligaments alone do not support the joint sufficiently. A coordinated use of muscles around the joint will compensate for ligament weakness. In case the muscles around the joint do not function, the patient will experience pain and weakness when performing everyday activities. This kind of pelvic often occurs after giving birth to a child weighing 3000 grams or more, in case of hyperactivity,
and sometimes after an accident involving the pelvis. Type 2 is the most common form of pelvic instability. Treatment is based on learning how to use the muscles around the pelvis more efficiently.

Pelvic type 3: The ligaments do not support the joint sufficiently. This is a serious situation whereby the muscles around the joint are unable to compensate for ligament weakness. This type of pelvic instability usually only occurs after an accident, or occasionally after a (small) accident in combination with giving birth. Sometimes a small accident occurring long before giving birth is forgotten so that the pelvic instability is attributed only to the childbirth. Although the difference between Type 2 and 3 is often difficult to establish, in case of doubt an exercise program may help the patient. However, if Pelvic Type 3 has been diagnosed then invasive treatment is the only option: in this case parts of the pelvic are screwed together.

Treatment: One of the main factors in helping women cope with the condition is with education, information and support. Other coping strategies include physical medicine and rehabilitation, physiotherapy, osteopathy, chiropractic, psychologist, prolo therapy or platelet-rich plasma therapy, massage therapy, acupuncture and alternative medicine. Mobility aids such
as a wheelchair, walker, elbow crutches
and walking stick can be very useful. Medication dispensed by a qualified health care provider can also be used to manage:

• Chronic pain
• Anxiety
• Depression
• Post Traumatic Stress Disorder (resulting from birth trauma/ pregnancy)
• Musculo-skeletal disorders.

Conservative treatment is effective in most cases, including those women with the most severe symptoms at presentati0n. A clear explanation of the condition and its management, to both the woman and her partner, is vital. The aim is to avoid abduction of the hip joint and encourage immobilization of the pubic symphysis joint. In cases presenting during pregnancy or after birth, women should be advised to rest as much as possible in the lateral decubitus position: avoid prolonged weight bearing and stairs and keep her legs together in activities such as turning in bed or getting into a car. Since immobilization is a primary risk factor for deep vein thrombosis, isometric exercises should be encouraged. Anti-embolism stockings and heparin may be required. Analgesics can be given on demand. If the above measures fail to improve the
symptoms, referral to an obstetric physiotherapist should be arranged. Pelvic support by a tight binder or tubular
bandage and the use of a walker or elbow crutches may be required. The maximum hip abduction possible without pain (pain-free gap) should be measured before labour, to avoid over-abduction of thighs in labour, especially when regional anesthesia is
used. Some pelvic joint trauma will not respond to conservative type treatments
and orthopedic surgery might become the
only option to stabilize the joints.

Surgery is rarely indicated, but may be considered for those who have inadequate reduction, recurrent diastases or
persistent symptoms. External skeletal fixation is the treatment of choice. The symphysis is compressed using a frame
which can be removed once stability has returned. Prognosis is uniformly good.
Mild cases typically resolve within 2 days to eight weeks of delivery with no lasting sequelae. However, some women require as much as eight months before they are free
of pain when walking. During this time the pain may be worse during the secretory phase of the menstrual cycle. In a recent survey of Norwegian women registered as having pregnancy-initiated pelvic joint pain, it was found that pelvic pain worsened with subsequent pregnancy in 81.4% of the responding. However, in the absence of specific obstetric indications, prior pubic symphysis separation should not be considered a strong indication for subsequent operative delivery.

Low Back Pain

Pathology: Symptomatic back pain in pregnancy is caused by the mechanical
burden created on the lower back by the pregnant uterus and compensatory lordosis. Relaxation of the sacroiliac joint and
pubic symphysis plays an important part.
The highest levels of relaxin during pregnancy have been found in women with incapacitating low back pain. Very occasionally, low back pain may be attributable to a herniated vertebral disc.
Presentation: The usual presentation is
that of low back pain or posterior pelvic pain that is aggravated by activity and relieved by lying down, sitting and the use of supportive pillow. The pain may radiate to the posterior aspects of the thighs. Examination reveals accentuation of the lumbar lordosis and the cephalad part of
the spine thrown backwards to compensate
for the increased size of the abdomen. Tenderness is usually greatest over the sacroiliac joints. Indirect bimanual compression over the iliac crest also produces discomfort in the sacroiliac
joints.

Management: Each patient should be questioned carefully about neurological compromise as very occasionally radicular signs or even a cauda equina syndrome may
be identified. Most patients with classic symptoms and signs limited to low back strain or sacroiliac instability can be managed without radiographic evaluation. Radiographic evaluation of patients with unusual or severe symptoms may be carried out after the first trimester and can include a three view spine series. However, MRI appears to be a safe way to image the pelvic regions during pregnancy and will give direct information about any disc prolapse without irradiation. This should now be the investigation of choice if indicated.

Treatment: Relief of symptoms of low back pain in pregnancy can be achieved by the patient limiting her physical activity, wearing low-heeled shoes, resting in bed with pillows under the knees and applying heat. Lying on the back with the feet propped approximately two feet above the hips for about 20 minutes four times a day usually relieves muscle spasm, decreases lumbar lordosis and relieves acute pain.
In addition, the pain can be partially relieved if the patient keeps the pelvis
in a flexed position, thereby improving spinal alignment. Exercise to increase the tone of the back and abdominal muscles should be commenced as soon as the pain decreases. A sacroiliac corset or trochanteric belt can relieve symptoms. Surgical treatment of low back pain is contraindicated in pregnancy, except when
a herniated disc is producing bowel or bladder incontinence. Pain relief can be achieved with simple analgesics but anti-prostaglandins are relatively contraindicated in pregnancy.

Hip Pain

Two relatively rare conditions, osteonecrosis of the femoral head and transient osteoporosis of the hip, both
seem to occur with somewhat greater frequency during pregnancy and present with pain in the hip or groin. The diagnosis of these conditions is often missed initially because pain is easily taken for pelvic girdle relaxation or round ligament pain. Early diagnosis and treatment are the keys for a successful outcome and prevention of secondary degenerative changes or fracture in the joints of these young women.

1) Osteonecrosis of the femoral head
Presentation: Symptoms usually begin in the third trimester or shortly after a difficult delivery, with sudden or gradually increasing pain of variable severity, usually unilateral and deep in the groin. The pain may radiate to the knee, thigh or back. Elderly primigravida are most at risk. On examination, painful limitation of
active or passive movements of the hip joint, especially with movement, can be noticed. The exact aetiology is not known. But it has been speculated that the rise in unbound cortisol, oestrogen and
progesterone in late pregnancy, the increased interosseous pressure and a
direct injury to the femoral joint by the compression of the growing uterus or during a difficult delivery may all act together
to produce insufficiency of blood supply
to the fernoral head at some point.

Management: Plain radiography may demonstrate arc-like subchondral
radiolucent areas and other pathological changes in the femoral head, but MRI has been used recently for earlier diagnosis with apparent safety during pregnancy.
Early diagnosis, rest and avoiding weigh-bearing are very important. Aspiration of the hip joint may occasionally be
required. The prognosis after early diagnosis and conservative treatment
seems to be good, although secondary degenerative or osteoarthritic changes
may develop and require surgical treatment at a later age.

Figure: Subchondral separation Figure: Osteonecrosis of femoral head on plain X-ray

2) Transient osteoporosis of the hip
Presentation: This is a poorly understood and frequently undiagnosed syndrome of unknown aetiology. It occurs in the third trimester and presents with pain in the
hip, anterior thigh or groin, which progressively increases and is made worse
by weight-bearing. The left hip is more frequently involved but bilateral involvement can also occur. On examination, pain and limitation of range of mobility on passive abduction and rotation of the affected joint is usually noticed.

Management: X-rays of the hip show advanced osteoporosis of the femoral head and neck and, occasionally, the acetabulum, but
with preservation of the joint space. These changes are present three to eight weeks after the onset of symptoms. MRI can be
use for early diagnosis. Bone mineral density (BMD) of the femoral neck of symptomatic women has been shown to be 20% lower than the average of age-matched controls. The great concern with regard
to this disorder is that continued unprotected weight-bearing can result in
a fracture of the femoral neck. The aim
of treatment is to avoid unprotected
weight bearing by the use of crutches until the symptoms resolve completely and radiography shows reconstitution of bone
in the proximal part of the femur. Given
the decrease in BMD that occurs during pregnancy and lactation, it might appear prudent to recommend cessation of lactation in these patients.

During pregnancy, circulating total calcium concentration drop slowly but consistently and parallel with decreasing albumin concentration. Reaching a nadir in the middle third of the third trimester. An early hypothesis was that pregnancy is a state of maternal physiologic hyperparathyroidism. According to this theory, transfer of calcium to the fetus induces secondary hyperparathyroidism
in the mother, which leads consequently
to increased 1,25-dihydroxyvitamin D production. Another theory says the
increase in circulating levels of 1, 25-dihydroxyvitamin D is the primary
event in calcium metabolism alterations during pregnancy, subsequently stimulating intestinal calcium absorption and possible additional effects on other target tissues. With these alterations in calcium metabolism, pregnancy may exacerbate or simply coexist with the number of
conditions that may result in maternal hypercalcemia. These conditions include primary hyperparathyroidism, vitamin A or
D intoxication, systemic sarcoid, hyperthyroidism, milk-alkali syndrome, familial hypocalciuric hyoercalcemia, immobilization, malignancy with or without bone metastasis or ectopic PTH secretion.
On the other hand, alterations in calcium and parathyroid hormone metabolism may
also results in hypoparathyroidism and hypocalcemia. Hypoparathyroidism results from inadequate secretions of PTH or defective production of biologically active PTH. Pseudohypoparathyroidism results from end-organ insensitivity to the hormone.
The diminished PTH activity in the kidney and bone leads to hypocalcemia and hyperphosphatemia. Patient with mild hypoparathyroidism may be asymptomatic or may experience only subtle manifestation
of the disease. In more severe forms of the disorder, symptoms and signs related to decreased serum ionized calcium concentrations may occur. Increased neuromuscular excitability, which can be elicited on physical examination by a positive result for Chovstek's sign
(tapping along facial nerve including contractions of the eye, mouth and nose)
or Trousseau's sign (inflating a blood pressure cuff above systolic pressure causing spasm of the hands within minutes), can uncommonly progress from weakness and paresthesia to the development of seizures, tetany, or laryngospasm. Papilloedema, elevated cerebrospinal fluid pressure and neurologic sign that mimic a cerebral
tumor may be found. A spectrum of mental status changes, from irritation to psychosis, can occur. Abnormalities in the cardiac conduction, particularly prolongation of
QT interval and T wave changes, may be present. Radiographs of the skull may demonstrate intracranial calcifications, which are sometimes associated with a parkinsonian-like syndrome. Additionally,
if the disease has been long standing, physical examination may reveal dental abnormalities or cataracts.
Untreated maternal hypoparathyroidism with its associated hypocalcemia leads to a high incidence of maternal, fetal and neonatal complications. Generalized skeletal demineralization, osteitis fibrosa cystica and fetal or neonatal death can occur. Although the secondary hyperparathyroidism is transient and generally resolves in the neonatal period, the infant may not
achieve normal bone mineralization until
6 months of age.

Deficiency of vitamin D and disorders of vitamin D absorption or metabolism can
lead to hypocalcemia and also to
subsequent disorders of bone
mineralization, such as osteomalacia and tetany. Derangements in vitamin D
metabolism may also explain the osteopenia associated with heparin treatment during pregnancy.

References:

1. Medical Complications During Pregnancy by Burrow and Duffy 5th edition

2. http://en.wikipedia.org

3. http://www.maitrise-orthop.com /corpusmaitri/orthopaedic/mo72_hernigou/index.shtml

4. http://www.ncbi.nlm.nih.gov/pubmed/18199383?dopt=AbstractPlus

5. http://www.ncbi.nlm.nih.gov/pubmed/1946104?ordinalpos=1&
itool=EntrezSystem2.PEntrez.Pubmed. Pubmed_ResultsPanel.Pubmed_ SingleItemSupl.Pubmed_ Discovery_RA&linkpos=4&log$=relatedreviews &logdbfrom=pubmed

N.B. This article was contributed by Medical Student Ms Azreena Baizura bt Ariffin from Melaka Manipal Medical College , Malaysia as an E learning Exercise.