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Paediatric Disease in Pictures by dr fadhil

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Paediatric Disease in Pictures by dr fadhil Powered By Docstoc
					Diseases in pictures By

  Dr. fadhil salman
Consultant pediatrician




          0
                                   Index


1.   Staphylococcal skin scalded           38.   Malar flush
     syndrome                              39.   Malar rash
2.   Ehlers-Danlos syndromes               40.   Malrotation with midgut
   (EDS)                                       volvulus
3.   Tonsillitis                           41. Microcephaly
4.   Telangiectasia                        42. Measles
5.   Tetra logy of fallout                 43. Micrognathia
6.   Achondroplasia                        44. Fifth disease
7.   Acrodermatitis                        45. Molluscum contagiosum
   enteropathica                           46. Mumps
8.   Female                                47. Osteopetrosis
   pseudohermaphrodite                     48. Metaphyseal
9.   Café au lait spots                        Chondrodysplasia
10. Cavernous haemangioma                  49. Retinoblastoma
11. Clubbing                               50. Neonatal oral thrush
12. Diaphragmatic Hernia                   51. Rickets
13. Exanthema subitum                      52. Herpes Zoster
14. Caroli’s Disease                       53. Umbilical sepsis
15. Actinomycosis                          54. Umbilical granuloma
16. Cornelia De Lange                      55. Dextrocardia
   Syndrome                                56. Goodpastures' syndrome
17. Chicken pox                            57. Mitral incompetence
18. Empyema                                58. Pleural effusion
19. Canavan Disease                        59. Hyperlucent lung
20. Kassabach-Merritt                      60. Cystic Hygroma
   Syndrome                                61. Histiocytosis (LCH)
21. Klippel Trenaunay Weber                62. Hydranencephaly
   Syndrome                                63. Hurler's Syndrome
22. Systemic lupus                         64. Foreign body aspiration
   erythematosus                           65. Mangolian Spot
23. Pradder Willi Syndrome                 66. Monilial diaper dermatitis
24. Exomphalos                             67. Sternocleidomastoid
25. Gastroschisis                              tumour
26. Hypothyroidism                         68. Congenital lung cysts
27. Stevens-Johnson syndrome               69. Congenital lung cysts
28. Idiopathic                             70. Congenital Diaphragmatic
   Thrombocytopenic Purpura                    Hernia
29. Henoch-Schonlein purpura               71. Renal Agenesis
30. Meningococcemia                        72. Cruozon craniofacial
31. Urticaria                                  dystosis
32. Abscess: Brain                         73. Moya Moya Disease
33. Abscess: Lung abscess                  74. Jaundice
34. Abscess: Neonatal mastitis             75. Bilary atresia
35. Abscess: Iatrogenic                    76. Herpetic Gingivostomatits
36. Orbital cellulitis                     77. Marasmus
37. Pustules: Neonatal                     78.    Acute myeloid leukemia


                                     1
79. Xanthoma                         93.    Cleft Lip
80. Hydrocephalus                    94.    Rachitic Rosary
81. Kayser-Fleischer ring            95.    Inguinal Hernia
82. Pierre Robin Syndrome            96.    Orbital Ecchymosis
83. Meningocele and                  97.    Gianotti Crosti Syndrome
   Myelomeningocele                  98.    HIV Children
84. Rheumatoid Arthritis             99.    Club Foot
85. Gangrene of Limb                 100.   Nephrotic Syndrome
86. Bladder Exstrophy                101.   Muscular Dystrophy
87. Carpopedal Spasm in              102.   Lesch-Nyhan Syndrome
   Tetany                            103.   Hypothyrodism
88. Subconjunctival                  104.   Chylothorax
   Haemorrhage                       105.   Thymoma
89. Obesity
90. Erb-duchenne paralysis
91. Giant congenital pigmented
   navi
92. Ascites




                                 2
1. Staphylococcal skin scalded syndrome:

   In neonates, young children, and immunocompromised individuals enough
   toxin can be produced by a localized infection and released into the blood
   stream to cause widespread superficial exfoliation resulting in staphylococcal
   scalded skin syndrome. Most individuals seem to acquire neutralizing
   antibodies to this toxin during childhood and thus this disease tends to be
   restricted to the young or the immunoimpaired. Since the toxin is primarily
   eliminated through the kidneys, patients with renal failure may also be at risk.
   It should not be confused with the much more serious form of epidermal
   sloughing, toxic epidermal necrolysis (TEN), which involves the loss of the
   entire epidermal surface, not just the stratum corneum. TEN is a dermatologic
   emergency treated like a widespread burn injury and is most often secondary
   to a drug reaction.




2. Ehlers-Danlos syndromes (EDS):

   The Ehlers-Danlos syndromes (EDS) are a heterogeneous group of heritable
   connective tissue disorders characterized by articular hypermobility, skin
   extensibility and tissue fragility.




                                       3
   3. Tonsillitis:

       The typical findings are

           o   redder-than-normal tonsils
           o   a yellow or white coating on the tonsils
           o   a funny-sounding voice
           o   swollen glands in the neck
           o   fever
           o   bad breath




   4. Telangiectasia:

Telangiectasias are found in areas of cutaneous inflammation. For example, lesions of
discoid lupus frequently have telangiectasias within them. Ataxia-telangiectasia (AT)
is an autosomal recessive genetic disorder characterized by cerebellar ataxia,
oculocutaneous telangiectasia, and immunodeficiency.




                                           4
5. Tetralogy of fallot:

·   A cyanotic congenital heart disease characterized by right ventricular
    hypertrophy, pulmonary stenosis, ventricular septal defect (VSD), and
    dextroposition of the aorta.

·   X-ray: boot-shaped (coeur en sabot)
        o concavity of L cardiac border (no PA)
        o normal heart size
        o RVH -> elevation of apical shadow
·   diminished pulmonary vascularity
·   aortic arch to R in 20%




6. Achondroplasia:

    A non-lethal type of congenital dwarfism characterized by typical skeletal
    dysplasias (rhizometric micromelia), a large head, and neurological
    manifestations. There may be recurrent and multiple fractures. Limbs:
    rhizometric micromelia,shortened limbs, proximal > distal shortening, elbows
    - lack of full extension and supination ,legs - genu varum (bowleg), hands -
    trident (splayed), deviated towards ulna, short and broad .




                                       5
7. Acrodermatitis enteropathica:

   Acrodermatitis enteropathica is a rare autosomal recessive disorder
   characterized by abnormalities in zinc absorption. Clinical manifestations
   include diarrhea, alopecia, muscle wasting, depression, irritability, and a rash
   involving the extremities, face, and perineum. The rash is characterized by
   vesicular and pustular crusting with scaling and erythema. In addition,
   hypopigmentation and corneal edema have been described in these patients.




8. Female pseudohermaphrodite:

   Overall, Congenital adrenal hyperplasia (CAH) is the most frequent cause of
   ambiguous genitalia in the newborn, constituting approximately 60% of all
   intersex cases. CAH produces a female pseudohermaphrodite, which is a
   gonadal female with a virilized phenotype.
   The basic biochemical defect is an enzymatic block that prevents sufficient
   cortisol production. Biofeedback via the pituitary gland causes the precursor to
   accumulate above the block. Clinical manifestation of CAH depends on which
   enzymatic defect is present.




                                       6
9. Café au lait spots:

   Neurofibromatosis (VON RECKLINGHAUSEN'S DISEASE) 1 is
   characterized by cutaneous neurofibromas, pigmented lesions of the skin
   called cafe au lait spots, freckling in non-sun exposed areas such as the axilla,
   hamartomas of the iris termed Lisch nodules, and pseudoarthrosis of the tibia.
   Neurofibromas are benign peripheral nerve tumors composed of proliferating
   Schwann cells and fibroblasts. They present as multiple, palpable, rubbery,
   cutaneous tumors. They are generally asymptomatic; however, if they grow in
   an enclosed space, e.g., the intervertebral foramen, they may produce a
   compressive radiculopathy or neuropathy. Aqueductal stenosis with
   hydrocephalus, scoliosis, short stature, hypertension, epilepsy, and mental
   retardation may also occur.




10. Cavernous haemangioma:

   A "cavernous hemangioma" is not a hemangioma but a venous malformation
   in which there is a dearth of smooth muscle in the wall of a large thin venous
   structure lined by endothelium. These never regress spontaneously, and
   neither glucorticoids nor IFN-? are effective.




                                       7
11. Clubbing:

   Hypertrophic osteoarthropathy (HOA) is characterized by clubbing of digits
   and, in more advanced stages, by periosteal new bone formation and synovial
   effusions. HOA occurs in primary and familial forms and usually begins in
   childhood. The secondary form of HOA is associated with intrathoracic
   malignancies, suppurative lung disease, congenital heart disease, and a variety
   of other disorders and is more common in adults. Clubbing is almost always a
   feature of HOA but can occur as an isolated manifestation. The presence of
   clubbing in isolation is generally considered to represent either an early stage
   or an element in the spectrum of HOA. The presence of only clubbing in a
   patient usually has the same clinical significance as HOA.




12. Diaphragmatic Hernia:

   Congenital Diaphragmatic Hernia (CDH) occurs in about one in every 2,500
   live births. Absence of the diaphragm may occur on the left, right or both
   sides, but the left side is most common. There is a wide discrepancy between
   the “visible” mortality reported from children’s centers, which treat only those
   infants who survive gestation, birth, resuscitation, transport and often major
   surgery, and the true mortality, based on all prenatally diagnosed cases, which
   has been called the “hidden mortality” of CDH.




                                      8
13. Exanthema subitum:

   Exanthema subitum had been speculated to be a viral disease although its
   pathogen is unknown. Human herpesvirus 6 (HHV-6), first isolated in 1986,
   was proved by Yamanishi et al. to be the causal agent of exanthema subitum.
   The typical presentation is appearance of generalized macular rash when the
   fever subsides without other localizing signs. The common age group affected
   are less than two years.




14. Caroli’s Disease:

   Caroli disease is a nonobstructive dilatation of the intrahepatic bile ducts. This
   is a rare congenital disorder that classically causes saccular ductal dilatation,
   which usually is segmental. Caroli disease is associated with recurrent
   bacterial cholangitis and stone formation.
   Caroli disease also is known as communicating cavernous ectasia or
   congenital cystic dilatation of the intrahepatic biliary tree. It is distinct from
   other diseases that cause ductal dilatation caused by obstruction. It is not one
   of the many choledochal cyst derivatives.




                                       9
15. Actinomycosis:

   Actinomycosis is a chronic, suppurating, granulomatous condition caused by
   bacteria producing branching hyphae, such as Actinomyces israelii. Most
   commonly affecting the cheek or mandibular skin, lesions can also be located
   in the thorax or elsewhere. Actinomyces, produces a chronic fibrotic
   necrotizing process that crosses tissue planes and may involve the pleural
   space, ribs, vertebrae, and subcutaneous tissue, with eventual discharge of
   sulfur granules (macroscopic bacterial masses) through the skin (empyema
   necessitatis).
   Typically, there is a group of dull red nodules, with sinuses draining colonies
   of organisms called "sulfur" granules.
   In the mouth and elsewhere, the lesion extends from a focus of bone
   involvement, or from some deeper focus.
   Treatment:
   - Long term (more than a year), high dose (10 million units daily) penicillin
   may be required, with or without surgical debridement.
   - Alternatives include imipenem and erythromycin.




16. Cornelia De Lange Syndrome:

   A disorder of unknown etiology resulting in a syndrome characterized by
   specific dysmorphic features. The following clinical pictures are seen:

1. Head and Neck:
   microcephaly +/- brachycephaly ,micrognathia ,low hairline ,low set ears
   ,dilated veins on temples +/- forehead ,synophris (confluent eyebrows) ,long
   curly eyelashes ,broad and/or depressed nasal bridge, anteverted nostrils,
   prominent philtrum ,thin upper lip, downturned angles of mouth ,high arched
   palate, delayed eruption of teeth ,bluish tinge around eyes, nose +/- mouth.




                                      10
2. Extremities:
   limitation of extension at elbows ,small hands and feet with short digits ,single
   transverse palmar crease, clinodactyly of fifth fingers, proximally placed
   thumbs, webbing of 2nd and 3rd toes .




3. Others:
   mental retardation ,growth retardation ,hirsutism ,cutis marmorata, birth
   weight <2500g ,neonatal difficulty with feeding +/- respiration.

17. Chicken pox:

   Varicella-zoster virus (VZV) causes two distinct clinical entities: varicella
   (chickenpox) and herpes zoster (shingles). The incubation period of
   chickenpox ranges between 10 and 21 days but is usually between 14 and 17
   days. Secondary attack rates in susceptible siblings within a household are
   between 70 and 90%. Patients are infectious approximately 48 h prior to the
   onset of the vesicular rash, during the period of vesicle formation (which
   generally lasts 4 to 5 days), and until all vesicles are crusted.
   Clinically, chickenpox presents as a rash, low-grade fever, and malaise,
   although a few patients develop a prodrome 1 to 2 days before onset of the
   exanthem. In the immunocompetent patient, this is usually a benign illness
   that is associated with lassitude and with body temperatures of 37.8 to 39.4?C
   (100 to 103?F) of 3 to 5 days' duration. The skin lesions?the hallmark of the
   infection?include maculopapules, vesicles, and scabs in various stages of
   evolution. These lesions, which evolve from maculopapules to vesicles over
   hours to days, appear on the trunk and face and rapidly spread to involve other
   areas of the body. Most are small and have an erythematous base with a
   diameter of 5 to 10 mm. Successive crops appear over a 2- to 4-day period.




                                       11
18. Empyema:

   Pleural empyema is a common complication of pneumonia in children and
   increases the already-considerable morbidity associated with this infection.
   The commonest aetiological agent is S. aureus which cause pneumonia
   without predisposing epidemiologic or host factors that favor colonization of
   the respiratory tract and/or that impair defense mechanisms. Tuberculous
   empyema is a less common complication of pulmonary tuberculosis. All of the
   cases needs drainage with decortication which improves the lung function and
   reduces the duration of the hospital stay.




19. Canavan Disease:

   Canavan disease is a severe progressive leukodystrophy characterized by
   swelling and spongy degeneration of the white matter of the brain. It is an
   autosomal recessive disease found more frequently among Ashkenazi Jews.
   This MRI is of a one and half year old female child from Myagdi, Gandaki
   Zone, who presented with the history of difficulty in walking for two months
   duration. The clinical features are thse of severe mental retardation with
   inability to gain developmental milestones. Hypotonia, head lag and
   macrocephaly are characteristic of Canavan disease and become apparent after
   5-6 months of age. Massive excretion in the urine of N-acetylaspartic acid is
   the biochemical marker for Canavan disease, which is caused by deficiency of
   the enzyme aspartoacylase. Computed Tomography (CT) scan of the head or
   Magnetic Resonance Imaging (MRI) of the brain reveal diffuse white matter
   degeneration. There is no treatment.




                                    12
20. Kassabach-Merritt Syndrome:

   Kassabach-Merrit (KM) syndrome is a consumption coagulopathy that occurs
   as a complication of angiomatous nevi. Angiomatous nevi leading to KM
   syndrome are generally single, deep lesions of large size, but the disorder can
   vary occasionally and occur in association with smaller and more superficial
   lesions. The responsible angiomas most commonly occur on the trunk, neck
   and proximal parts of the limbs, particularly the thighs and shoulders, and are
   relatively uncommon on the head. This picture is of an 2 month old infant who
   was brought to the pediatric outpatient department for evaluation of a swollen
   left arm since birth. On examination the limb was enveloped by a rapidly
   growing hemangioma. Although the child appeared well and the hemangioma
   was not symptomatic, a small ulcer was noted near the elbow, and the platelet
   count was less than 10,000.




21. Klippel Trenaunay Weber Syndrome:

   Klippel-Trenaunay syndrome is characterized by a triad of port-wine stain,
   varicose veins, and bony and soft tissue hypertrophy involving an extremity.
   KTWS generally affects a single extremity, although cases of multiple
   affected limbs have been reported. The leg is the most common site followed
   by the arms, the trunk, and rarely the head and the neck. This hemangioma has
   a distinct, linear border that respects the midline. Hemangioma is often noted
   on the lateral aspect of the limb.It is typically of the nevus flammeus type, but
   cavernous hemangiomas or lymphangiomas may also occur. Nevus flammeus
   is a salmon pink patch, sometimes with a verrucous quality, which evolves to
   a deep purple color with time. Unlike strawberry hemangiomas, the port-wine
   stain hemangioma possesses neither a proliferative nor a regressing phase.




                                      13
22. Systemic lupus erythematosus:

   Systemic lupus erythematosus (SLE) is a disease of unknown etiology in
   which tissues and cells are damaged by pathogenic autoantibodies and
   immune complexes. The abnormal immune responses include (1) polyclonal
   and antigen-specific T and B lymphocyte hyperactivity, and (2) inadequate
   regulation of that hyperactivity. At onset, SLE may involve only one organ
   system (additional manifestations occur later) or may be multisystemic. The
   malar ("butterfly") rash is a photosensitive, fixed erythematous rash, flat or
   raised, over the cheeks and bridge of the nose, often involving the chin and
   ears.




23. Pradder Willi Syndrome:

   Prader-Willi Syndrome (PWS) is a chromosomal (chromosome 15) disorder,
   which can affect children of both sexes and of any race or country. Three
   Swiss doctors -Prader, Labhart and Willi - first described it in 1956. Major
   characteristics: obesity, hypotonia, hypogonadism, hyperphagia, cognitive
   impairment, difficult behaviors.




                                      14
24. Exomphalos:

   It is a herniation of intra-abdominal contents through the umbilical ring into
   the umbilical cord. It could be associated with Beckwith-Wiedemann
   syndrome (exomphalos, macroglossia and gigantism).




25. Gastroschisis :

   It is a complete defect through all layers of the anterior abdominal wall
   extending up to 3 cm in length and usually lying to the right of the normally
   attached umbilical cord. At delivery it is essential the baby is placed in a
   plastic bag extending above the level of defect leaving the head and upper
   extremity exposed and urgently referred to neonatal surgical unit.




                                      15
26. Hypothyroidism:

   This condition could be congenital or acquired. At birth the diagnosis is not
   always possible but as the neonate grows the condition is recognizable by
   hypothermia, hoarse cry, prolonged jaundice, rough dry skin, macroglossia,
   and sparse, dry and straight hair. Constipation, delayed milestones of
   development, delayed closure of cranial sutures and failure to gain height are
   other features seen subsequently if not treated.




27. Stevens-Johnson syndrome:

   It is a blistering disorder that is usually more severe than erythema
   multiforme. Initial presentation is often a sore throat, malaise, and fever.
   Within a few days, in addition to erosions of multiple mucous membranes,
   small blisters developing on dusky or purpuric macules or atypical target
   lesions characterize this eruption. Total percent of body surface area blistering
   and eventual detachment is less than 10%.




                                      16
28. Idiopathic Thrombocytopenic Purpura:

   It is common in children and accounts for 90% of the pediatric cases of
   immunologic thrombocytopenia. The classic presentation of ITP is that of a
   previously healthy 1–4 yr old child who has the sudden onset of generalized
   petechiae and purpura. Often there is bleeding from the gums and mucous
   membrane, particularly with profound thrombocytopenia (platelet count < 10
   × 109 /L). The two commonest differential diagnoses are aplastic anaemia and
   malignancy. The physical examination is normal other than the finding of
   petechiae and purpura. Splenomegaly is rare.




29. Henoch-Schonlein purpura:

   It is also referred to as anaphylactoid purpura, is a distinct systemic vasculitis
   syndrome that is characterized by palpable purpura (most commonly
   distributed over the buttocks and lower extremities), arthralgias,
   gastrointestinal signs and symptoms, and glomerulonephritis. It is a small
   vessel vasculitis. Most patients recover completely, and do not require
   therapy.




                                       17
30. Meningococcemia:

   Fulminant Meningococcemia (Purpura Fulminans) is one of the most rapidly
   lethal form of septic shock in children. It differs from most other forms of
   septic shock by the prominence of hemorrhagic skin lesions (petechiae,
   purpura ) and the consistent development of DIC. Many times the CSF is
   clear. It presents as acute onset of purpuric rash in a child with fever. There
   may be signs of meningitis. Unless treated urgently with steroids and
   penicillin it is fatal.




31. Urticaria:
    Urticaria (hives) are transient lesions that are composed of a central wheal
    surrounded by an erythematous halo. Individual lesions are round, oval, or
    figurate and are often pruritic. The common physical urticarias include
    dermographism, solar urticaria, cold urticaria, and cholinergic urticaria




                                       18
32. Abscess: Brain :
    A brain abscess is a focal, suppurative process within the brain parenchyma; it
    begins in an area of devitalized brain tissue as a localized area of cerebritis and
    develops into a collection of pus surrounded by a well-vascularized capsule.
    One organism is cultured in the majority of abscesses (70%), two in 20%, and
    three or more in 10% of cases. Abscesses associated with mucosal infections
    (sinusitis) frequently have anaerobic bacteria. Clinical signs include non
    specific symptoms as fever, letharg during early stage. Later signs of raised
    intracranial pressure vomiting, severe headache, seizures and papilloedema
    develops. Focal neurological signs is seen subsequently. When the aetiological
    agent is not known the combination of a third-generation cephalosporin and
    metronidazole is used for 3-6 weeks. A large abscess should be drained
    surgically.




33. Abscess: Lung abscess:
    The commonest causes of lung abscess is pneumonia and aspiration. Both
    anaerobic and aerobic organisms can cause lung abscesses. The most common
    symptoms of pulmonary abscess are cough, fever, tachypnea, dyspnea, chest
    pain, vomiting, sputum production, weight loss, and hemoptysis. It is treated
    with 2–3 wk course of parenteral antibiotics for uncomplicated cases, followed
    by a course of oral antibiotics to complete a total of 4–6 wk. Antibiotic should
    include aerobic and anaerobic coverage




                                        19
34. Abscess: Neonatal mastitis:
    Enlargement of breast tissue in new born infants is a common physiologic
    hormonal phenomenon. Excessive manipulation can result in secondary
    infection. It is treated with systemic antibiotics with advise to avoid further
    mani-pulation. Brain abscess following neonatal masttits have been reported.




35. Abscess: Iatrogenic:
    Post injection abscess are commonly seen in hospital admitted children. It is
    often seen due to the extra venous spoiling of drugs from an i.v canula. This
    hospital acquired infection some times difficult to treat because of resistant
    microorganism. It may complicate with distant metastasis.




                                       20
36. Orbital cellulitis:
    It can occur at any age or sex. There will be noticeable lid edema and redness,
    distention, proptosis, and significant pain upon palpation. Additionally, there
    will be diplopia from extraocular motility limitations. There typically will be a
    precipitating factor such as penetrating lid trauma, mucormycosis, orbital
    medial wall blow-out fracture, severe lid infectious disease, bite wounds,
    meningitis, sinusitis and sinus infection, septicemia, ketoacidosis, or dental
    abscess. Vision loss and an afferent pupil defect may often be present. The
    patient will also be systemically ill and have a fever. The sagital sinus
    thrombosis is known complication




37. Pustules: Neonatal:
    It contains purulent material. These are commonly seen in neonatal period as
    skin infections. Few pustules could be treated with local antibiotics and
    regular follow-up. Large and numerous pustules should be treated with
    antibiotics for one week, preferably by iv antibiotics.




                                       21
38. Malar flush

   It is commonly seen in children with high fever, polycythaemia and severe
   mitral stenosis. Malar fllush with high fever is often seen in typhoid,
   pneumonia and pyelonephritis.




39. Malar rash

   Patients with systemic lupus erythematosus typically develop a sharply
   defined, erythematous eruption in a butterfly distribution on the cheeks (malar
   rash). It is also seen in infection caused by the spirochete Borrelia burgdorferi,
   Graft versus host disease and impetigo.




                                       22
40. Malrotation with midgut volvulus

   Malrotation is incomplete rotation of the intestine during fetal development
   The midbowel (distal duodenum to midtransverse colon) begins to elongate
   and progressively protrudes into the umbilical cord until it lies totally outside
   the confines of the abdominal cavity. An acute presentation of small bowel
   obstruction in a patient without previous bowel surgery is usually a result of
   volvulus associated with malrotation. This is a life-threatening complication of
   malrotation and the main reason that symptoms suggestive of malrotation
   should be investigated. The diagnosis is by ultrasound or contrast radiographic
   studies. The abdominal plain film is usually nonspecific but may demonstrate
   evidence of duodenal obstruction with a double-bubble sign.




41. Microcephaly

   Microcephaly is defined as a head circumference that measures more than
   three standard deviations below the mean for age and sex. Although there are
   many causes of microcephaly, abnormalities in neuronal migration during fetal
   development, including heterotopias of neuronal cells and cytoarchitectural
   derangements, are found in many brains. Microcephaly may be subdivided
   into two main groups: primary (genetic) microcephaly and secondary
   (nongenetic) microcephaly. A precise diagnosis is important for genetic
   counseling and for prediction for future pregnancies.




                                      23
42. Measles

   Measles (rubeola) is an important childhood disease which causes high
   morbidity and mortality in developing countries. Infants acquire immunity
   transplacentally from mothers who have had measles or measles
   immunization. This immunity is usually complete for the first 4–6 mo of life
   and wanes at a variable rate. The essential lesion of measles is found in the
   skin, conjunctivae, and the mucous membranes of the nasopharynx, bronchi,
   and intestinal tract. Measles has three clinical stages: an incubation stage, a
   prodromal stage with an enanthem (Koplik spots) and mild symptoms, and a
   final stage with a maculopapular rash accompanied by high fever. The
   incubation period lasts approximately 10–12 days to the first prodromal
   symptoms and another 2–4 days to the appearance of the rash; rarely, it may
   be as short as 6–10 days. Body temperature may increase slightly 9–10 days
   from the date of infection and then subside for 24hr or so. The patient may
   transmit the virus by the 9th–10th day after exposure and occasionally as early
   as the 7th day, before the illness can be diagnosed.




43. Micrognathia

   Often the growth of the mandible will achieve a normal profile within 4–6 yr.
   The feeding of infants with mandibular hypoplasia requires great care and
   patience but can usually be accomplished without resorting to gavage. Pierre
   Robin sequence consists of micrognathia usually accompanied by a high
   arched or cleft palate. The tongue is usually of normal size, but the floor of the
   mouth is foreshortened. Obstruction of the air passages may occur, particularly
   on inspiration, and usually requires treatment to prevent suffocation. The
   infant should be maintained in a prone or partially prone position so that the
   tongue falls forward to relieve respiratory obstruction.




                                      24
44. Fifth disease

   Anderson and colleagues identified B19 as the cause of erythema infectiosum
   or fifth disease in 1983. The incubation period for erythema infectiosum
   ranges from 4 to 28 days (average, 16–17 days). The prodromal phase is mild
   and consists of low-grade fever, headache, and symptoms of mild upper
   respiratory tract infection. The hallmark of erythema infectiosum is the
   characteristic rash, which occurs in three stages that are not always
   distinguishable. The initial stage is an erythematous facial flushing, often
   described as a “slapped-cheek” appearance. The rash spreads rapidly or
   concurrently to the trunk and proximal extremities as a diffuse macular
   erythema in the second stage. Affected children are afebrile and not ill-
   appearing.




45. Molluscum contagiosum

   Discrete, pearly, skin-colored, dome-shaped, smooth papules vary in size from
   1–5?mm. They typically have a central umbilication from which a plug of
   cheesy material can be expressed. The papules may occur anywhere on the
   body, but the face, eyelids, neck, axillas, and thighs are sites of predilection.
   Lesions on patients with AIDS tend to be large and numerous, particularly on
   the face; exuberant lesions may also be found in children with leukemia and
   other immunodeficiencies. Molluscum contagiosum is a self-limited disease;
   the average attack lasts 6–9 mo.




                                      25
46. Mumps

   It is caused by an RNA virus of the genus Paramyxovirus in the family
   ParamyxoviridaeThe incubation period ranges from 14–24 days, with a peak
   at 17–18 days. Approximately 30–40% of infections are subclinical. In
   children, prodromal manifestations are rare but may be manifest by fever,
   muscular pain (especially in the neck), headache, and malaise. The onset is
   usually characterized by pain and swelling in one or both parotid glands. The
   parotid swells characteristically; it first fills the space between the posterior
   border of the mandible and the mastoid and then extends in a series of
   crescents downward and forward, being limited above by the zygoma. There is
   no specific antiviral therapy; treatment is entirely supportive




47. Osteopetrosis

   Bone-in-bone appearance that is characteristic of osteopetrosis. Most of the
   manifestations are due to failure to remodel growing bones. This leads to
   narrowing of cranial nerve foramina and encroachment on marrow spaces,
   which results in secondary complications, such as optic and facial nerve
   dysfunction, and anemia accompanied by compensatory extramedullary
   hematopoiesis in the liver and spleen. Skeletal radiographs reveal a
   generalized increase in bone density and clubbing of metaphyses.




                                      26
48. Metaphyseal Chondrodysplasia

   It is a rare autosomal dominant disorder of endochondral ossification of long
   bones. Clinical diagnosis is based on presence of bowed legs and short stature
   during infancy and early childhood. Waddling gait is often the presenting sign
   in second year of life. There is marked widening of the joints with expansion
   of the long bones of forearms and legs. The biochemical parameters serum
   calcium, serum phosphorus, alkaline phosphatase and 24 hrs urinary excretion
   of calcium and phosphate are normal.




49. Retinoblastoma

   Only about 10% of retinoblastomas are detected by routine ophthalmologic
   screening in the context of a positive family history. Overall, about 60% of
   cases are unilateral and nonhereditary, 15% unilateral and hereditary, and 25%
   bilateral and hereditary. Bilateral involvement is found in 42% of those
   presenting when younger than 1 yr of age but in only 21% of those presenting
   during 1 yr of age and is even less common at older ages of
   presentation.Retinoblastoma classically presents with leukocoria, a white
   pupillary reflex. Case like this is late presentation. Most unilateral disease
   presents as a large tumor. Enucleation is undertaken if there is no potential for
   useful vision.




                                      27
50. Neonatal oral thrush

   Thrush of the mouth occurs in healthy infants. Transmission of fungi from
   maternal vaginal moniliasis to the infant's oral mucosa is the primary means of
   infection in healthy newborns. Secondary cases may develop in the hospital
   nursery as a result of direct or indirect contact with infected infants,
   caretakers, or contaminated supplies.




51. Rickets

   In rickets, defective bone growth results from retardation or suppression of
   normal epiphyseal cartilage growth and calcification. These changes result
   from a deficiency of calcium and phosphorus salts in the serum. Cartilage cells
   fail to complete their normal cycle of proliferation and degeneration, with
   subsequent failure of capillary penetration, which occurs in a patchy manner.
   The result is a frayed, irregular epiphyseal line at the end of the shaft. Failure
   of osseous and cartilaginous matrix to mineralize in the zone of preparatory
   calcification, followed by deposition of newly formed uncalcified osteoid,
   results in a wide, irregular, frayed zone of nonrigid tissue (the rachitic
   metaphysis.




                                      28
52. Herpes Zoster

   Herpes zoster is manifested as vesicular lesions clustered within one or less
   commonly two adjacent dermatomes Unlike zoster in adults, zoster in children
   is infrequently associated with localized pain, hyperesthesias, pruritus, and
   low-grade fever. In children, the rash is mild, with new lesions appearing for a
   few days; symptoms of acute neuritis are minimal; and complete resolution
   usually occurs within 1–2 wk. In contrast to adults, postherpetic neuralgia is
   very unusual in children. In Nepal there is a belief that if faces of tigers are
   painted towards the end of vesicles it will not spread.




53. Umbilical sepsis

   Although aseptic delivery and routine cord care (daily application of triple dye
   to the umbilical stump and surrounding skin) decrease the risk of umbilical
   infection, the necrotic tissue of the umbilical cord is an excellent medium for
   bacterial growth. Omphalitis may remain localized or may spread to the
   abdominal wall, the peritoneum, the umbilical or portal vessels, or the liver.
   Infants with abdominal wall cellulitis or those with necrotizing fasciitis have a
   high incidence of associated bacteremia. Portal vein phlebitis may develop and
   result in the later onset of extrahepatic portal hypertension.




                                      29
54. Umbilical granuloma
    The umbilical cord usually dries and separates within 6–8 days after birth. The
    raw surface becomes covered by a thin layer of skin, scar tissue forms, and the
    wound is usually healed within 12–15 days. The presence of saprophytic
    organisms delays separation of the cord and increases the possibility of
    invasion by pathogenic organisms. Mild infection or incomplete
    epithelialization may result in a moist granulating area at the base of the cord
    with a slight mucoid or mucopurulent discharge. Good results are usually
    obtained by cleansing with alcohol several times daily.




55. Dextrocardia
    Positional anomalies of heart refer to conditions in which the cardiac apex is
    in the right side of the chest (dextrocardia), or at the midline (mesocardia), or
    in which there is a normal location of the heart in the left side of the chest but
    abnormal position of the viscera (isolated levocardia). Knowledge of the
    position of the abdominal organs and of the branching pattern of the main
    stem bronchi is important in categorizing these malpositions. When
    dextrocardia occurs without situs inversus, when the visceral situs is
    indeterminate, or if isolated levocardia is present, associated, often complex,
    multiple cardiac anomalies are usually present. In contrast, mirror-image
    dextrocardia is usually observed with complete situs inversus, which occurs
    most frequently in individuals whose hearts are otherwise normal.




                                        30
56. Goodpastures' syndrome
    Goodpastures' syndrome, a disease characterized by lung hemorrhage and
    severe glomerulonephritis, represents an example of antibody binding leading
    to local activation of complement and neutrophil accumulation and activation.
    Early in the disease, it is alveolar in nature, more prominent at the bases and
    perihilar regions — simulates pulmonary edema Within 2-3 days, the blood is
    absorbed in to the interstitium and the pattern changes to interstitial reticular
    By about 10 days, the reticular disease disappears With repeated bleeds, there
    is hemosiderin deposit in the lungs and progressive
    pulmonary fibrosis occurs.




57. Mitral incompetence
    Rheumatic etiology is the commonest cause in developing countries. It takes
    several years to develop mitral valvular lesions. The typical symptoms are due
    to pulmonary edema and reduced cardiac output. The specific clinical signs
    are: displaced apex beat out side the midclavicular line and apical pansystolic
    murmur radiating towards the axilla. The chest x-ray shows: enlarged left
    atrium, prominent pulmonary conus, enlarged left ventricle making the
    straight left heart border and plethoric lung fields.




                                       31
58. Pleural effusion:
    Pleural effusion could be serous, purulent, hemorrhagic or chylous. It could be
    unilateral or bilateral. Infections causes unilateral effusion where as bilateral
    effusions are due to conditions leading to hypoproteinemia. The commonest
    cause of unilateral effusion in Nepal is pneumonia and tuberculosis. The
    typical clinical signs are: diminished thoracic movement on the involved site,
    fullness of intercostals spaces, dullness on percussion and diminished breath
    sounds. PA view of the chest x-ray shows dense uniform opacity in the lower
    and lateral part of the hemithorax.




59. Hyperlucent lung
    Swyer-James-Macleod Syndrome is a variant of post-infectious bronchiolitis
    obliterans that is most commonly the sequela of a viral (adenovirus) infection
    during infancy or early childhood. There is a necrotizing bronchiolitis which
    damages the terminal and respiratory bronchioles and prevents the normal
    development of their alveolar buds. Additionally, fibrosis (the result of the
    healing process) results in bronchiolar obstruction that in turn leads to air
    trapping. It may also be seen following measles, pertussis, tuberculosis, and
    mycoplasma infections . The syndrome is characterized by unilateral
    hyperlucency with associated decrease in the size and number of pulmonary
    vessels on the involved side. Patients may be asymptomatic, or complain of
    recurrent URI's or dyspnea on exertion. Although classically involving an
    entire lung, the disorder can be lobar or subsegmental. Although the cxr is
    confused with pneumothorax, the trachea is not shifted and the child looks
    comfortable. Sharma PR. Swyer-James-Macleod Syndrome. ( Journal of
    Institute of Medicine, vol.25, Issue 2, 2003




                                       32
60. Cystic Hygroma

   During developmental period around the sixth week of gestation lymphatic
   channels are formed from a series of clefts that develop in the mesenchyme.
   From these channels, sacs are formed that establish drainage with the venous
   system. Failure to establish venous drainage results in dilated disorganized
   lymph channels, which, in the largest form, present as cystic hygromas. The
   masses are usually large soft structures without clear margins. The most
   important sign is the illumination test.




61. Histiocytosis (LCH)

   There are generally first signs of LCH and frequently become manifest as
   scaly, erythmatous, seborrhea like brown to red papules, especially
   pronounced in inter-triginous zones. Hepatosplenomegaly is the indication of
   presence of organ involvement by LCH or it may indicate obstructive disease
   caused by enlarged nodes in the porta hepatis. It may also reflect kupffer cell
   hypertrophy and hyperplasia as an indicator of generalized activation of the
   cellular immune system. Cough, tachypnea/dyspnea, cyanosis, pneumothorax,
   or pleural effusion are attributable to the disease rather than to superimposed
   infection. Increasing number of cysts form honeycomb lungs and in later
   stages large bullae. This child had fever and was pale. The seborrhoeic
   dermatitis lesions were seen over scalp and abdomen. He had cervical
   lymphadenopathy. Abdomen was soft on palpation with liver 8 cm below
   costal margin and spleen 4 cm below costal margin. Preliminary blood counts
   show hemoglobin 6.5 gm%, TLC 6300, Platelet count 68,000, DLC N49 L46
   M02 My01 MM02.




                                     33
62. Hydranencephaly

   Hydranencephaly is a rare condition in which the brain's cerebral hemispheres
   are absent and replaced by sacs filled with cerebrospinal fluid. An infant with
   hydranencephaly may appear normal at birth. The infant's head size and
   spontaneous reflexes such as sucking, swallowing, crying, and moving the
   arms and legs may all seem normal. However, after a few weeks the infant
   usually becomes irritable and has increased muscle tone. After a few months
   of life, seizures and hydrocephalus (excessive accumulation of cerebrospinal
   fluid in the brain) may develop.




63. Hurler's Syndrome

   Gertrud Hurler, 1889-1965, Austrian paediatrician first described this disease.
   It is caused by deficiency of the enzyme deficiency alpha-L-iduronidase.
   There is excess urinary excretion of dermatan sulphate and heparan sulphate.
   Clinically, there is severe mental retardation which is progressive. The
   children have a coarse facies, are short statured, have a protruberant abdomen,
   hernias, joint contractures and a thoracolumbar gibbus. There is also
   hepatosplenomegaly, cardiomyopathy and cardiac failure. Radiologically,
   there is a large skull with a J-shaped sella, shallow orbits, beaking and
   flattening of the anterior portion of the vertebral bodies at the thoracolumbar
   junction, wide, thick ribs, thick clavicles, widening of the shafts of the long
   bones with flaring of relatively small iliac wings with acetabular roofs. All the
   mucopolysaccharidoses share in common pointing of the bases of the
   metacarpals.




                                      34
64. Foreign body aspiration

   Foreign body aspiration is common children as they have the habit of keeping
   things in the mouth. The commonest material that is reported in the literature
   is peanuts. Common symptoms is persistent cough and respiratory distress
   (depends upon the type and size of the ingested material). This child presented
   with the history of cough for three weeks and persisten tachypnoea. On
   examination he had bronchial breath sound in the right infrascapular region.
   He was treated with various antibiotics. The chest xray shows the collapse
   consolitatin in the right lower zone and CT scan shows the changes. He
   coughed out the small piece of olive while in hospital the repeat chest x-ray
   shows the cleared previous area.




                                     35
65. Mangolian Spot:

   This is present at birth in more than 80% of Asian infants. Typically there will
   be a single lesion rarely multiple can occur. This is an asymptomatic benign
   condition which fades during the first one to two years of life. Three to four
   percent often persists for life. No melanomas have been reported to occur. No
   treatment is required.




66. Monilial diaper dermatitis:

   This is common in children below 3 years of age. Candida albicans is the
   causative organism. It is usually present in area covered by diaper. Topical
   nystatin or clotrimazole is the treatment. No treatment is required.




                                      36
   67. Sternocleidomastoid tumour:

This is due to the bleeding within the sternicleidomastoid muscle caused by the
injury during difficult delivery. The formation of hematoma within the
sternocleidomastoid further damages the muscle causing ischemia. Ultimately the
fibrous tissue replaces the ischemic fibres. This will produce congenital torticollis.
Passive stretching will usually be effective in restoring the range of motion. This
tumours may be present any where in the line of sternocleidomastoid.




   68. Congenital lung cysts :

       Congenital lung cyst is also a rare condition, which presents in the neonatal
       period. It was first described by Stock in 1897. The etiology of this condition
       is not known. But it is recognized by the absence of bronchotubular glands and
       cartilage and the presence of tall columnar mucinous epithelium. There is
       overproduction of luminal bronchiolar structure in the form of cysts of various
       size. These present as respiratory distress of various degrees. Sometimes, there
       may not be any symptoms for as long as 5 to 6 months. Normally male babies
       are affected more often than female.
       It is usually unilateral affecting the left upper lobe followed by the right
       middle and right upper lobes. Involvement of more than one lobe is rare.
       Similarly involvement of lower lobe is also extremely rare. The differential
       diagnosis was CLE, congenital diaphragmatic hernia (CDH), pneumothorax
       and lung cyst. Chest X-ray of shows hyperlucency on the affected side with
       multiple cystic shadows.




                                         37
   69. Congenital lung cysts

Ch'in and Tang first described cystic adenomatoid malformation (CAM) as a distinct
entity in 1949. CAM is a developmental hamartomatous abnormality of the lung with
adenomatoid proliferation of cysts resembling bronchioles. CAM represents
approximately 25% of all congenital lung lesion. CAM is believed to result from focal
arrest in fetal lung development before the seventh week of gestation secondary to a
variety of pulmonary insults. CAM is subdivided into 3 major types.

                        Type I                    Type II                   Type III
 Morphology Multiple large cysts or Multiple, evenly spaced         Multiple small cysts less
            single large cyst       cysts not over 1.2 cm           than 0.5 cm




 Contents       Air or fluid            Air                         Solid

 Wall           Smooth muscle and       Terminal bronchioles        Adenomatoid
                connective tissue                                   hyperplasia or bronchial




 B. Symptoms
 · Progressive respiratory distress in a newborn infant
 · Tachypnea, subcostal retraction, cyanosis

CT findings

   ·    Areas of small cysts (<2 cm in diameter) appearing with other abnormalities (a
        larger cystic area, consolidation, or low attenuation) are the most frequent
        findings.
   ·    Multiple large cystic lesions (>2 cm in diameter) are seen alone or with other
        abnormalities (areas of small cysts, consolidation, or low attenuation).
   ·    Low-attenuation areas are clusters of microcysts




                                          38
70. Congenital Diaphragmatic Hernia :

   This rare condition is seen in 1:5,000 live births. Commonly it is seen on the
   left side. It is often associated with pulmonary hypoplasia and malrotation of
   the intestine. It is important to examine these children for other congenital
   anamolies. It is often present in trisomies. Most of this condition is diagnosed
   due to the presence of severe respiratory distress.




71. Renal Agenesis :

   Bilateral agenesis is not compatible with post natal life. Potter phenotype is
   the presentation in newborn with the typical facial abnormality. Unilateral
   agenesis is often diagnosed during the course of investigation. All newborn
   with single umbilical artery should be suspected for renal agenesis. The
   contralateral kidney goes into compensatory hypertrophy. Most cases are often
   associated with other anamolies. Children are at risk of hypertension and
   proteinuria. These IVP and isotopic renogram was from a boy who presented
   with persistent hypertension.




                                      39
72. Cruozon craniofacial dystosis:

   This was originally described in 1912 by Crouzon. The commonest
   abnormalities is ocular proptosis with or without hypertelorism due to shallow
   orbits. The other abnormalities are hypoplasia of maxilla and craniosynostosis.
   It is transmitted as autosomal dominant with variable expression.




73. Moya Moya Disease:

   It is a rare, progressive cerebrovascular disorder characterized by the
   narrowing or occlusion of major blood vessels leading into the brain, and the
   formation of abnormal blood vessels called moyamoya vessels. (ie "puff of
   smoke") characterizes the appearance on angiography of abnormal vascular
   collateral networks that develop adjacent to the stenotic vessels. The steno-
   occlusive areas are usually bilateral, but unilateral involvement does not
   exclude the diagnosis. Cerebral angiography in this case has shown poorly
   opacified intracranial internal carotid artery and its branches. The CT Scan
   shows multiple infarcts in bilateral cerebral hemisphere new and old.




                                     40
    74. Jaundice:
        This is a clinical condition due to the abnormally raised bilirubin in serum.
        Typically the sclera becomes yellowish. As the serum bilirubin increases the
        skin also becomes yellow. The various causes could be hemolytic , hepatic or
        obstructive. The commonest cause is the infective hepatitis, where the sclera is
        mild to deep yellow. The greenish yellow tinge is suggestive of obstructive
        jaundice.




    75. Biliary atresia

The most common form is the absence of entire extrahepatic biliary tree at or above
the porta hepatis. This results in the absence of bile in the stool that typically results in
raised conjugated bilirubin and clay coloured stool. It is essential to differentiate this
condition from Idiopathic Neonatal Hepatitis. Hepatobiliary scintigraphy using
imidodicetic acid analogs is helpful to differentiate these two conditions. This
scintigraphy shows good intake in liver but no excretion in the intestine.




    76. Herpetic Gingivostomatits

It is the commonest form of stomatitis in children. Children present with insidious
onset of high fever preceeding the oral lesions by 1-2 days. The acute phase lasts for
one week. There is no specific treatment. Symptomatic treatment with paracetamol
makes difficult to counsel parent on its self limited course.




                                             41
   77. Marasmus

This condition is seen in children due to severe malnutrition. As the child fails to gain
weight and looses weight the wrinkles of skin are seen specially over buttock. The
child’s face retain a normal appearance till late. Hairs are sparse. Atrophy of the arm
and thigh muscles are visible easily with distension of abdomen. With proper
nutritional therapy the look completely changes.




   78. Acute myeloid leukemia

This rare leukemia in children occurs 5:1000000. Certain genetic disorders predispose
to it e.g. trisomy 21, Diamond-Blackfan anaemia and Fanconi anaemia. Typically it
presents with anaemia, fever, bleeding, hepatosplenomegaly. A localized mass of
leukemic cells (chloroma) may develop at any site as seen in this child. With
aggressive therapy the cure rate is 30-40%.




   79. Xanthoma

Xanthoma is exceedingly rare in children aged 10 years or younger. Papular
xanthoma (PX) is a very rare skin disorder. Histologic studies shows diffuse
monomorphous infiltrate of foamy cells in the upper dermis.




                                           42
   80. Hydrocephalus:


It is not a specific disease but complications of many intracranial acquired or
congenital diseases. It results from impaired circulation and absorption of CSF. The
clinical features depends upon the duration and rate of rise of intracranial pressure.
The earliest sign in a child could be enlarging occipitofrontal circumference and the
late sign could be setting sun eye sign as in this child. Long tract signs due to
stretching of the pyramidal tract is also seen.




   81. Kayser-Fleischer ring

This finding is seen in due to the abnormal copper deposition in the stromal layer of
limbic region of eye. Kayser-Fleischer rings consist of copper granules in the stromal
layer of the eye. Color changes are visible only in the Descemet membrane and
typically are described as a golden brown, brownish green, bronze, or tannish green
color seen in the limbic area of the eye. The rings fade and disappear with appropriate
therapy. The typical condition is most commonly seen in Wilson’s Disease.




   82. Pierre Robin Syndrome

This syndrome consist of micrognothia, glossoptosis and high arch or cleft palate.
One of the most important difficulty the child feels is difficulty in breathing when
lying supine. This is because the tongue falls back causing obstruction over the
pharynx. The infant should be maintained in a prone or partially prone position to
relieve respiratory obstruction. The feeding of infants in this sequence requires great
care. Often the growth of mandible will achieve an essentially normal profile within
4-6 year.




                                           43
   83. Meningocele and Myelomeningocele

A meningocele is formed when the meninges herniated through a defect in the
posterior vertebral aches or midline neuraxis. The spinal chord is usually normal.
Myelomeningocele is the severe form of dysraphism more commonly involving the
lumbosacral region but can occur anywhere along the neuraxis. It is covered by thin
layer of partially epithelialized tissue and remnants .




   84. Rheumatoid arthritis:

This chronic arthritis due to immunological process involves the large and small joints
of limbs and other parts of body. It is characterized by chronic nonsuppurative
inflammation of synovium. If untreated the joints gets deformed and takes the typical
Swan Neck deformity as seen in the following pictures.




   85. Gangrene of limb

Gangrene of limb may be with oedema or without oedema. If it is oedematous mostly
the cause is infective or venous obstruction. If the gangrene is not associated with
oedema then the common causes are vasospastic or thrombo-embolic. Raynauds
phenomenon could be due to collagen diseases, Burger’s disease, cold agglutinin,
cryoglobulinaemia , neurogenic etc.




   86. Bladder exstrophy




                                          44
This rare congenital abnormality is found in every 40,000 births. The severity ranges
from small abdominal fistula to the complete exstrophy of the cloaca resulting in the
exposure of the bladder. In males there is complete epispadias with a wide and
shallow scrotum. Females have wide duplication of the clitoris. Management of this
condition should start at birth by covering the area with a Sialistic shield or another
appropriate plastic dressings and should be urgently transferred to a surgical unit.




   87. Carpopedal spasm in tetany

This is the typical sign of peripheral hyperexcitability of motor nerves due to the
spasm of muscle of the wrists. The wrists are flexed , fingers are extended and thumbs
adducted over the palms. The commonest cause is hypocalcemia. In this child it is
secondary to the intake of large amount of fluid containing sodium bircabonate.




   88. Subconjunctival haemorrhage

The bleeding in the subconjunctival area remains bright red due to the continuous
oxygenation of the haemoglobin. It may result from trauma or inflammation and may
be unilateral or bilateral. It may occasionally result from whooping cough or bleeding
disorders as thrombocytopenia..




   89. Obesity



                                           45
Extreme obesity in children are mostly due to the endocrine or genetic causes or due
to syndromes (cohen and carpenter). This two and half year old child weighed 30 Kg.
One of the complication of this extreme obesity is pickwickian syndrome. In this
syndrome there severe cardiorespiratory distress with alveolar hypoventilation, which
is manifiested by polycythemia, somnolence and features of congestive cardiac
failure.




   90. Erb-Duchenne paralysis

This condition is due to the injury to the 5th and 6th cervical nerves during birth. The
characteristic positon is adduction and internal rotation of the arm with pronation of
the forearm. The power of the forearm and the hand grasp are usually preserved. The
presence of hand grasp is a favorable sign. Treatment consists of partial
immobilization and appropriate positioning to prevent contractures.




   91. Giant congenital pigmented nevi

This rare skin condition is seen in <1:20,000 births. This occurs mostly on the trunk.
This nevi needs special attention as there may be association with leptomeningeal
melanocytosis. If associated with neural melanosis there may be features of mental
retardation with seizures. Management of this condition is controversial however a
team of pediatrician, dermatologist and plastic surgeon should discuss with parents.




                                           46
   92. Ascites

In this condition there is collection of fluid in the peritoneal cavity. Clinically it is
diagnosed by eliciting Puddle’s sign, shifting dullness or fluid thrill depending upon
the amount of fluid. In huge collection the diaphragm is pushed up making the child
difficult to lie flat in bed. The prominent veins on the abdominal wall signifies
inpaired venous flow through the portal vein or inferior vena cava . The three
commonest cause are tubercular, portal vein and inferior venacaval obstruction.




   93. Cleft lip:
       Cleft lip can occur with or without cleft palate. The incidence is 1:600 births.
       This incidence is highest amongst Asians due to unknown reason. If it is
       associated with cleft palate there is recurrent aspirations and chocking can
       occur, often the child fails to thrive. Special teats are available for cleft palate
       to feed the child, often orthodontic appliance are needed. Surgical closure of
       lip is usually performed by 2 months of age.




   94. Rachitic rosary


In advanced rickets the enlargement of the costochondral junctions may become
prominent. In many cases the beading of the ribs is not only palpable but also visible
as in this case. The sides of thorax become flattened and longitutional grooves
develop posterior to the rosary. The sternum with its adjacent cartilages appears to be


                                            47
projected forward, producing the so called pigeon breast deformity. Harrison’s
groove, which corresponds to the costal insertions of the diaphragm.




   95. Inguinal Hernia

It is one of the commonest cause of swelling in the groin. Approximately 50% present
before the age of one year. In children it is the result of persistent patency of
processus vaginalis. Parent often notice this swelling during crying. The treatment of
choice is operative repair. Operation is not indicated in a child with a isolated
hydrocele in tunica vaginalis.




   96. Orbital ecchymosis


Head injury is frequently seen in children. This child had injury in baby walker and
large hematoma was developed in the forehead which descended to the eye lids giving
this appearance. Baby walker should not be used for children.




                                         48
   97. Gianotti-Crosti Syndrome

This is a rare, self limited eruption, characterized by the onset of nonpruritic lichenoid
papules. This is commonly seen in face and limb. In this child the site is unusual often
confused with necklace dermatitis (itchy). Physical examination rveals monomorphus
papules, flesh colored, flat topped and 1-10 mm in size. It is self limiting.




   98. HIV children

The first cases of AIDS were reported in Nepal in 1988. Surveillance data is scarce in
Nepal. However, limited data indicate that HIV prevalence is currently around 0.5
percent in the general population. As of June 2002, the Ministry of Health (MoH) has
reported 606 cases of AIDS and 2,392 HIV infections. These children were in one of
the medical college who are seropositive




   99. Club foot


Talipes Equinovarus is a deformity of the foot which may be congenital or positional.
The cause of congenital is not known. It is considered to be multifactorial.
Conservative methods of treatment include taping and use of malleable splints. Serial


                                           49
plaster casts are the major nonoperative treatment. Complete correction both clinically
and radiologically should be achieved by three months of age. If surgery is needed it
is done between 6-12 months of age.




   100.        Nephrotic syndrome:
      It is characterized by oedema, proteinuria, hypercholesteraemia and
      hypoalbuminaemia. Idiopathic, minimal change histology is the commonest
      type. It responds to steroids. First the periorbital swelling is noted and ascites
      subsequently follows. The frothy urine signifies massive proteinuria.




   101.        Muscular dystrophy
      The term dystrophy means abnormal growth. Muscular dystrophy term was
      first used by Erb in 1891. Duschenne type is the commonest heriditory
      muscular disorder. It is sex linked recessive inherited. Gower’s sign is the
      typical sign. Cardiomyothy is a constant feature of this disease. The serum CK
      is consistently greatly elevated.




                                           50
   102.       Lesch-Nyhan Syndrome:
      This X-linked condition is due to the defective purine metabolism. In infant
      the serum uric acid concentration is in the gouty adult range. It is characterized
      by compulsive self-destructive behaviour as seen in this case: chewing of lip
      and tongue leading to mutilation. These children may chew their fingers.
      Other neurological signs like extrapyramidal choreoathetoid movements,
      hyperreflexia, ankle clonus and spasticity of the legs develop.




   103.        Hypothyrodism

This condition is due to the deficiency of thyroid hormone either congenital or
acquired. It could be due to the deficiency of iodine , abnormality in thyroid gland or
low thyroid stimulating hormone from hypothalamus. The affected child has the
typical facial appearance as seen bellow. After two weeks of thyroid hormone there is
drastic change in appearance. Often the treatment is continued for life long. Every
neonate with prolonged jaundice should be investigated for hypothyroidism because
mental retardation could be prevented.




   Before treatment                                   After two weeks of treatment




                                          51
   104.        Chylothorax:

It is due to the escape of chyle from the thoracic duct in the pleural cavity. It may be
due to the rapture of thoracic duct due to the surgery or malignancy or congenital
anamolies. The clinical manifestations are similar to the pleural effusion or pyothorax.
The diagnosis is established when thoracentesis reveals a milky white fluid. The
biochemical analysis shows high triglyceride level. The bed side test for chyle may be
done by shaking the chyle with alkali or ether. If it is a chyle it becomes clear.




   105.        Thymoma:

Embryologically, thymic epithelium is derived from both the ectoderm and the
endoderm of the 3rd & 4th branchial cleft and pharyngeal pouches. Thymoma is a
tumor arising from epithelium of thymus gland. Majority of thymoma looks
histological benign. Usually it follows indolent course in patient surviving for years.
34% of thymomas invades their own capsules, extending to surrounding structures.
Less than 10% behave like invasive epithelial malignancies. Incidence 0.15 cases per
100,000 accounting 0.2 to 1.5% of all malignancies Thymoma constitute about 50%
of anterior mediastenal neoplasm in adult, Lymphoma -25% Peak age of incidence –
4th to 6th decades of life. Children very rarely affected.




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