MEDICAL FACTS AND MCQ'S

CTEV : congenital talipes equino varus ( club foot )

INTRODUCTION :

Clubfoot can be classified as (1) postural or positional or (2) fixed or rigid. Postural or positional clubfeet are not true clubfeet. Fixed or rigid clubfeet are either flexible (ie, correctable without surgery) or resistant (ie, require surgical release, though this is not entirely true according to the Ponseti experience^1,^2,³).
The Pirani, Goldner, Di Miglio, Hospital for Joint Diseases (HJD), and Walker classifications have been published, but no classification system is

Gustilo Classification
I	Low energy, wound less than 1 cm
II	Wound greater than 1 cm with moderate soft tissue damage
III	High energy wound greater than 1 cm with extensive soft tissue damage
	IIIA	Adequate soft tissue cover
	IIIB	Inadequate soft tissue cover
	IIIC	Associated with arterial injury

Enneking staging of bone tumors

Enneking System for Staging Benign and Malignant Musculoskeletal Tumors

BENIGN

Latent

Active

Aggressive

MALIGNANT

Stage	Grade	Site	Metastases
IA	Low	Intracompartmental	None
IB	Low	Extracompartmental	None
IIA	High	Intracompartmental	None
IIB	High	Extracompartmental	None
III	Any	Any	Regional or distant metastases

Mangled Extremity Severity Score

*LIMB SALVAGE Vs AMPUTATION :

*To predict which limbs will be salvageable after trauma, available scoring systems include the predictive salvage index, the limb injury score, the limb salvage index, the mangled extremity syndrome index, and the mangled extremity severity score. Of these, the Mangled extremity severity score was found to be most useful.

*This system, which is easy to apply, grades the injury on the basis of the energy that caused the injury, limb ischemia, shock, and the patient's age.

*The system was subjected to retrospective and prospective studies, with a score of 6 or less consistent with a salvageable limb. With a score of 7 or greater, amputation was the eventual result.

*Although we do not strictly follow these guidelines in all patients, we do calculate and document a mangled extremity severity score in the chart whenever we are considering primary amputation versus a complicated limb salvage.

*Mangled Extremity Severity Score

Type	Characteristics	Injuries	Points
1	Low energy	Stab wounds, simple closed fractures, small-caliber gunshot wounds	1
2	Medium energy	Open or multiple-level fractures, dislocations, moderate crush injuries	2
3	High energy	Shotgun blast (close range), high-velocity gunshot wounds	3
4	Massive crush	Logging, railroad, oil rig accidents	4
Shock Group
1	Normotensive hemodynamics	BP stable in field and in OR	0
2	Transiently hypotensive	BP unstable in field but responsive to intravenous fluids	1
3	Prolonged hypotension	Systolic BP <90 mm Hg in field and responsive to intravenous fluid only in OR	2
Ischemia Group
1	None	Pulsatile limb without signs of ischemia	0^[*]
2	Mild	Diminished pulses without signs of ischemia	1^[*]
3	Moderate	No pulse by Doppler, sluggish capillary refill, paresthesia, diminished motor activity	2^[*]
4	Advanced	Pulseless, cool, paralyzed, and numb without capillary refill	3^[*]
Age Group
1	<30 y		0
2	>30 – <50 y		1
3	>50 y		2

BP, blood pressure; OR, operating room.

^*	Points × 2 if ischemic time exceeds 6 hours

Differences between Gout and Pseudogout

GOUT	PSEUDOGOUT
1. Smaller joints	1. Large joints
2. Intense pain	2. Moderate pain
3. Joint inflamed	3. Joint swollen
4. Hyperuricemia	4. Chondrocalcinosis
5. Uric acid crystals	5. Calcium pyrophosphate crystals
6. Rod shaped crystals	6. Rectangular shaped crystals
7. Negatively bisfringent	7. Positive bisfringence

Causes of Pseudoarthrosis

*Pseudoarthrosis is a false joint, that may develop after a fracture that has not united properly.

*It may be idiopathic.

*Causes of Pseudoarthrosis (in decreasing order of frequency are) :
1. Non union of a fracture - including pathological fracture.
2. Congenital - in the middle to lower third of the tibia with or without fibula. 50% present in the first year. Later there may be cupping of the proximal bone and pointing of the distal bone end.
3. Neurofibromatosis - seen in 50% of patients with pseudoarthrosis.
4. Osteogenesis imperfecta
5. Cleidocranial dysplasia - congenitally in the femur
6. Fibrous dysplasia
7. Ankylosing spondylitis - in the fused bamboo spine .

Causes of Osteolytic and Osteoblastic metastases

*Causes of Osteolytic metastasis :
- Kidney (expansile) and Thyroid : Expansile lytic osseous metastasis are characteristic of Renal cell carcinoma (kidney) and Thyroid .
- Lung
- Gastrointestinal tract (GIT)
- Breast (occasionally)
- Less commonly melanoma, carcinoma of bronchus and pheochromocytoma may also present with Expansile lytic lesions.

*Causes of Osteoblastic metastasis :
- Prostate
- Breast (may be mixed)
- Seminoma
- Carcinoid
- Neurogenic tumors
- Uterus - Ovary

Pulsating Tumors of the Bone

*Primary tumors that may present as pulsating lesions :
- Telengiectatic Osteogenic sarcoma
- Angioendothelioma/Angiosarcoma of bone
- Aneurysmal bone cyst
- Giant cell tumor (rarely)

*Secondaries/Metastases that may present as pulsating lesions :
- Metastasis from Renal cell carcinoma
- Metastasis from Thyroid carcinoma

Markers of Bone formation and Bone resorption

*MARKERS OF BONE FORMATION :
- Serum Bone specific Alkaline phosphatase
- Serum Osteocalcin
- Serum propeptide or type I procollagen

*MARKERS OF BONE RESORPTION :
- Urine and Serum N-telopeptide
- Urine and Serum C-telopeptide
- Urine total free deoxypyridinoline
- Urine Hydroxyproline
- Serum tartarate resistant Acid phosphatase
- Serum Bone Sialoprotein
- Urine hydroxylysine glycosides

Congenital Dislocation of Hip ( CDH or DDH)

*Synonyms: CDH, Developmental dysplasia of the hip, DDH, dysplasia of the hip

*Developmental dysplasia of the hip (DDH) was formerly referred to as congenital dislocation of hip. DDH is now the preferred term to reflect that DDH is an ongoing developmental process, which is variable in presentation and not always detectable at birth. Developmental dysplasia of the hip refers to a range of developmental hip disorders, from a hip that is mildly dysplastic, concentrically located, and stable, to one that is severely dysplastic and dislocated.

*There is a suggestion that the national screening programme in the UK, that has operated since 1969, has

Milestones of Prenatal development

1^st week of gestation : fertilization and implantation , beginning of embryonic period.

2^nd week of gestation : endoderm and ectoderm appear ( bilaminar embryo ).

3^rd week of gestation : first missed menstrual period, mesoderm appears ( trilaminar embryo ), somites begin to form.

4^th week of gestation : neural folds fuse, folding of embryo into human like shape, arm and leg buds appear , crown-rump length is 4 to 5 mm .

5^th week of gestation : lens placodes,primitive mouth,digital rays on hands.

6^th week of gestation : primitive nose , philtrum ,primary palate , crown-rump length 21 – 23 mm

7^th week of gestation : eyelids begin

8^th week of gestation : ovaries and testes distinguishable

9^th week of gestation : fetal period begins, crown-rump length is 5 cms, weight is 9 grams .

10^th week of gestation : external genitalia distinguishable

20^th week of gestation : usual lower limit of viability ,weight is 460 grams , length is 19 cms

28^th week of gestation : eyes open , fetus turns head down , weight is 1000 grams

38^th week of gestation : term.

Nadas criteria

The assessment of a child for the presence or absence of heart disease can be done with the help of some guidelines suggested by nadas and are called "nadas criteria" . the criteria are divided into major and minor criteria. Presence of one major and two minor criteria are essential for indicating the presence of heart disease.

The major criteria are :

1. systolic murmur grade 3 or more specially with a thrill
2. diastolic murmur
3. cyanosis
4. congestive cardiac failure

The minor criteria are :

1. systolic murmur less than grade 3 in intensity
2. abnormal second heart sound ( S2)
3. abnormal ECG
4. abnormal X-ray
5. abnormal blood pressure

Juvenile Myoclonic Epilepsy ( Janz syndrome )

Here are some important points about Juvenile Myoclonic Epilepsy :

1. Juvenile Myoclonic Epilepsy is otherwise called Janz syndrome .

2. It usually begins between the ages of 12 and 16 year .

3. It accounts for approximately 5 % of all the epilepsies .

4. Patients note frequent myoclonic jerks on awakening , making hair combing and tooth-brushing difficult .

5. As the myoclonus tends to abate later in the morning, most patients do not seek medical advice at this stage and some deny the episodes .

6. A few years later, early morning generalized tonic-clonic seizures develop in association with the myoclonus.

7. The EEG shows a 4-6/sec irregular spike and wave pattern, which is enhanced by photic stimulation .

8. The neurologic examination is normal.

9. Majority of the patients respond dramatically to valproate, which is required lifelong .

10. Discontinuance of the drug causes a high rate of recurrence of seizures .

Live and Killed vaccines

*All the live attenuated vaccines are contraindicated in pregnancy.

Treatment of Vesicoureteral reflux in children

Grade I	Reflux into a non-dilated ureter
Grade II	Reflux into the upper collecting system without dilatation
Grade III	Reflux into dilater ureter and/or blunting of calyceal fornices
Grade IV	Reflux into a grossly dilated ureter
Grade V	Gross dilatation of the ureter, renal pelvis and calyces: Calyces show loss of papillary impression

*TREATMENT RECOMENDATION FOR VUR diagnosed following a UTI :

GRADE	AGE(YRS)	SCARRING	INITIAL TREATMENT	FOLLOW-UP
I-II	Any	-	Antibiotic prophylaxis	-
III-IV	0-5	-	Antibiotic prophylaxis	Surgery
III-IV	6-10	-	U/L: Antibiotic prophylaxis	Surgery
III-IV	6-10	-	B/L: Surgery
V	less than 1	-	Antibiotic prophylaxis	Surgery
V	1-5	No	U/L: Antibiotic prophylaxis	Surgery
V	1-5	No	B/L: Surgery
V	1-5	Yes	Surgery
V	6-0	-	Surgery

Differential diagnosis of Neonatal Jaundice

A. Jaundice consisting of either direct or indirect bilirubin, that is present at birth or appears with in the first 24 hours of life causes :
*Erythroblastosis fetalis (high direct bilirubin - in infants who were given intrauterine transfusions)
*Concealed hemorrhage
*Sepsis
*Congenital infections like syphilis, CMV, rubella and toxoplasmosis
*Jaundice secondary to extensive eccymosis or blood extravasation may occur during the first day or later,

Features of Prematurity

*Premature babies are those babies who are born before 37 completed weeks.

*Physical criteria of prematurity :
- Skin texture : Shiny gelatinous, thin, plethoric skin.
- Lanugo : abundant.
- Plantar creases : Single deep crease over anterior 1/3rd of sole or no deep creases. Sole may be full of superficial creases.
- Genitals : Males : Both testes are at external inguinal ring or above. Empty scrotum with scanty rugosities.
- Genitals : Females : Labia majora are widely separated with labia minora fully exposed with hypertrophied clitoris.
- Breast nodules : Lesser than 5 mm and nipple is small or absent.
- Ear cartilage : Cartilage is deficient or absent with poor recoil.

What is vascular ring ?

vascular ring is a malformation of the aortic arch that results in vascular branches or major blood vessels encircling the trachea and esophagus. In the double aortic arch (one of the most common vascular ring malformation patterns)It is most often due to persistence of the double aortic arch "right-sided aortic arch is present" after the second month of fetal life.

The two arches surround the esophagus and trachea which, if sufficiently constrictive, may cause breathing or swallowing difficulties.

In other vascular ring malformations, vascular remnants such as the ligamentum arteriosum (formerly the ductus arteriosus) may be part of the ring encircling the trachea and esophagus.

The esophagus and trachea are both compressed by the vascular ring made by malformation of the brachial arch vessels.

Systemic Sclerosis

Systemic sclerosis is an autoimmune connective tissue disorder. Other similar diseases include SLE, RA, Sjogren’s syndrome and mixed connective tissue disease. There is a lot of overlap in the symptoms of these diseases. Many will require immunosuppressive therapy.

- You should always consider connective tissue diseases in ill patients with multisystem involvement, when there is no infection present.

- Also remember that, even though the common presentations are discussed below, connective tissue disorders can present with strange symptoms, that can be just about anything!