| Birth
Marks |
Often the vascular skin lesions are broadly
grouped into 2 varieties based on its presentation and
its pathology.
Vascular malformations:
Birth marks that are present since birth . Patch of
any colour other than black on any part of the skin
or mucosa present at or within days after birth. These
vascular malformations are variable in its manifestation
depending on whether its a low flow or high flow vascular
malformation.
1) Low flow: Slow growing or static.
Usually a cosmetic issue than any thing else. Birthmarks
of this nature are easily manageable with various properly
selected LASERs.
2) High flow: Progressive vascular
lesions often with worsening symptoms such as swelling,
pain, overgrowth of the part and beyond, pressure on
the neighboring structures, high output cardiac failure
etc. Often what is seen on the surface is tip of the
iceberg with unpredictable deeper extent. Hence these
high flow vascular malformations require detailed imaging
investigations such CT Angio/MRA etc to study the extent,
type and feeding vessels. The management is often complex
and requires input from interventional radiologist before
surgical removal. Due to extensive nature of this condition,
the removal is often incomplete in order to preserve
the blood supply to the extremities. This is one of
the reasons why they sometimes recur later.
Haemangiomas: Plumply
vascular lesion which appear as a pinkish patch initially
few weeks after birth. This vascular birth mark has
a peculiar growth cycle and spontaneous resolution in
majority of the cases. That means majority of them require
none other than supervision by experts in dealing with
these birth marks. These lesions keep growing until
the child is around 3 years followed by the phase of
spontaneous resolution of about 70% by 7years. Occasionally
residual redundant skin requires tidying up. In minority
of symptomatic cases as a result of pressure from rapidly
growing haemangioma in the vicinity of important parts
such eyes, close monitoring by paediatrician with or
with out surgical intervention is required. Otherwise
it may result in some irreversible damage such as blindness. |
| Clefts |
Clefts are birth defects on the face,
generally the upper lip and palate. these defects are
usually vertical slits of variable extent and associated
with a range of nose or other facial deformities.
Children born with cleft lip and palate in isolation
or in combination poses the eagerly awaiting parents
with emotional distress, a sense of guilt and many questions.
In the ideal world, it is diagnosed during pregnancy
and the parents are given proper counselling. The counselling
aims at educating the parents regarding these congenital
defects, their incidence, modern management programme
that makes these unfortunate babies into nearly normal
individuals.
These babies are better managed by a well co-ordinated
multidisciplinary team (MDT) of specialists that includes
Paediatrician, Plastic Surgeon, Orthodontist, Speech
therapist etc co-ordinated by a cleft care nurse. With
this approach, one can expect the best outcome and experience
in restoring normalcy.
|
| Cranio
Facial anomalies |
Babies born with abnormal shaped head,
which sometimes get worse unless intervened and some
of them get better without any intervention. But the
key is to identify those who need intervention in order
to take them through a timely procedure to restore the
shape, to prevent potential damage to brain and eyes
by relieving undue pressure and exposure.
This again demands a well co-ordinated team of specialists
which includes the team similar to cleft care, in addition,
a Neurologist and Neurosurgeon are to be involved as
a part of the MDT.
Some of these children do present with anomalies in
the hand as a part of the Syndrome. The correction procedures
on the affected hand are timed usually in the preschool
age.
|
| Defects
in the chest & Abdominal wall |
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They are rare but most disturbing for
parents and difficult for treating doctors. Chest wall
defects are much rarer than Abdominal ones. The Abdominal
wall defects are again grouped into two major types,
Gastroschisis and Omphalocele/Exomphalos. They can be
minor or major depending the on the proportion of the
defect. The minor ones are suitable for direct surgical
closure without delay, Where as the major omphloceles
requires special techniques to expand the abdominal
wall to accommodate all the abdominal contents before
the surgical closure can be achieved.
Because these are rare, the parents and family friends
of these unfortunate babies have joined together in
the net as a forum to share the experience and to support
the future victims. You can find them on http://geeps.forumsplace.com.
|
| Hand/Foot
anomalies |
The hand and upper limb develops into
full shape within a period 3-4weeks time during the
later part of the 1st trimester of the pregnancy.
Any disturbance to this process could result in some
kind of anomaly. The list of these hand anomalies run
into pages in the textbooks. But the common ones are
extra fingers, fused fingers, underdeveloped or absent
thumb/fingers. Most complex ones are when the deformity
affecting the whole hand and extending to the remaining
parts of the upper limb to various degrees as in Radial
& Ulnar Club hands. These complex anomalies require
multiple staged procedures which are properly timed
to restore the shape and its function.
Finally, extremely rare condition where the hand is
completely absent at any level below the shoulder. There
are only two options, 1) Hand transplant(rare &
many conditions apply). 2) Prosthesis (Currant practice).
|
| Genital
anomalies(Hypospadias, Vaginal atresia etc) |
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Phimosis:
Commonest of all anomalies. Pinhole opening of the foreskin
of the penis is called Phimosis. This is treated either
by Circumcision or Prepucioplasty where in the foreskin
is adequately preserved while increasing the opening.
Hypospadias: One
of the Common genital anomalies is hypospadias. The
diagnosis of Hypospadias is made when the baby is born
with a urinary passage opening at an abnormal location
undersurface of the penis. The severity of hypospadias
is directly depends on how far is it away from the tip
of the Penis. Further the severe. It is often associated
with Chordee. The penis that bends down upon erection
is called called Chordee. The objectives of treating
Hypospadias includes 1) Relocating the opening to the
tip of the penis, 2) Straight penis upon erection and
3)Minimal scarring.
Epispadias: This
is opposite of hypospadias and rarer. The abnormal opening
is on the upper surface of the penis. The treatment
is based on the same principles as for Hypospadias.
Extrophy of Bladder:
This is very rare and considered to be severe degree
of epispadias. This includes both external and internal
anomalies where in the urinary bladder is open to the
exterior in the lower abdomen via the unfused wide pubic
bones. This requires staged procedures by a team of
Plastic, Orthopaedic and Paediatric surgeons to achieve
optimal results.
Vaginal Atresia: Absent
or poorly developed Vagina. The reconstruction of Vagina
can be achieved by various techniques ranging from skin
grafts to free flaps which are chosen on individual
basis. This is usually timed only in adulthood usually
requires element of cooperation from the prospective
sexual partner/Husband.
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