GROUP 6 POLYDACTYL AND SYNDACTYL - PowerPoint Presentation

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GROUP 6

POLYDACTYL AND SYNDACTYL

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GROUP MEMBERS

OGUNNIYI OLUWATOYIN 17/MHS02/111

OLALERE FATIMAH 17/MHS02/113

SANI RIHANAT 17/MHS02/114

SULEIMAN BINTA 17/MHS02/115

SHAIBU FAWZIYAT 15/MHS02/050

OJO LOVETH 14/MHS01/102

AKPOSIVWODOR ESEROGHENE 15/MHS02/012

OLUWASOLA BOLANLE 15/MHS02/043

MOSHESHE PRUDENCE 15/MHS02/034

BAMBE CHIOMA 15/MHS02/018

ADEOLU DAMILOLA 16/MHS02/053

ADEDIGBE OLUWAPELUMI 15/MHS02/002

 

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INTRODUCTION

Polydactyl is characterized by more than five fingers/digits in hand/foot. It can present alone or as part of many syndrome due to genetic disorder. The defects may be due to

autosomal

recessive or 

autosomal

dominant disease. The present work was conducted to

analyse

the type, pattern of involvement, associated anomalies, plan of treatment and outcome of this malformation.

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From Raphael's paintings it was  found that the father (St. Joseph) and son had postaxial

polydactyly

which is an

autosomal

 dominant trait. St. Joseph found shoes uncomfortable because of his polydactyl. Pre and postaxial polydactyl are also found in

Acrocallosal

Syndrome, an

autosomal

recessive condition characterized by agenesis of corpus

callosum

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Molecular aspect of limb development (4) has an identifiable molecular basis. Hand surgeons should beware of the basic molecular pathways controlling limb development because they are in a unique position to be able to identify patients with such deformities.

If Ultrasonographic examination can be done during 14-16 weeks of gestation, fetuses with polydactyl may be observed. USG is a valuable tool for identification and early management since there is no biochemical or

histopathological

markers. In

utero

 auto-amputation (5) of extra digits can be done. 

Pregnancies, having polydactyl associated with other anomalies, should be terminated. Whereas isolated fetal postaxial polydactyl type II is associated with a

favourable

outcome

.

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EPIDEMIOLOGY

The condition has an incidence of 1 in every 500 live births. Postaxial hand polydactyl is a common isolated disorder in African black children, and

autosomal

dominant transmission is suspected. Postaxial polydactyl is more frequent in native Africans living in the Eastern and Central than the Caucasians and Mongoloids and is more frequent in male

children.In

contrast, postaxial polydactyl seen in white children is usually

syndromic

and associated with an

autosomal

recessive transmission. This study showed incidence of all types of polydactyl to be 2.3 per 1000 in Caucasian males, 0.6 per 1000 in Caucasian females, 13.5 per 1000 in African males, and 11.1 per 1000 in African females.

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Syndactyly and polydactyl—respectively characterized by fused and supernumerary digits—are among the most common congenital limb malformations, with syndactyly presenting at an estimated incidence of 1 in 2,000–3,000 live births and polydactyl at a frequency of 1 in approximately 700–1,000 live births. Despite their relatively regular manifestation in the clinic, the etiologies of

syndactyl

and polydactyl remain poorly understood because of their phenotypic and genetic diversity

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TYPES OF POLYDACTYL

Five types of polydactyl were encountered, 

Type I-

cutaneous

nubbin,

Type II-

pedunculated

digit, 

Type III- articulating digit with fifth metacarpal,

Type IV-fully developed digit with sixth metacarpal

Type V-

polysyndactyl

.

Type I and II

ulnar

polydactyl are more prevalent

.

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TYPES OF

POLYDACTYL

There are 3 types of polydactyl based on where the digit is:

1.  

Ulnar

or postaxial polydactyl or small finger duplication: This is the most common form of the condition, where the extra finger is on the outside of the little finger. This side of the hand is known as the

ulnar

side. When this form of the condition affects the toes, it is called fibular polydactyl.

2. Radial or

preaxial

polydactyl or thumb duplication: This is less common, occurring in 1 in every 1,000 to 10,000 live births. The extra finger is on the outside of the thumb. This side of the hand is known as the radial side. When this form of the condition affects the toes, it is called

tibial

polydactyl.

 3. Central polydactyl: This is a rare type of polydactyl. The extra finger is attached to the ring, middle, or most often index finger. This form of the condition has the same name when it affects the toes.

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CAUSES AND SYMPTOMS

Polydactyl and

syndactyl

are conditions that occur in the developing fetus. Most often these conditions are caused by genetic factors. Both polydactyl and

syndactyl

can be caused by the presence of an

autosomal

dominant trait. An

autosomal

dominant trait is a gene that is not related to the chromosome that determines gender; therefore, it affects boys and girls equally. Because the gene is dominant, when one parent has the gene, each of his or her children has a 50 percent chance of having polydactyl or

syndactyl

.

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The primary symptom of polydactyl is the presence of extra digits on the hands or feet. Polydactyl rarely causes any difficulties for the child. The extra fingers and/or toes are usually removed for cosmetic reasons. In children with

syndactyl

of the hand, finger function may be impaired and, in cases where fingers of different lengths are connected by tissue, finger growth may be limited unless surgery to separate the fingers is performed

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During normal embryonic development (while the baby is still in the womb), the hand initially forms in the shape of a paddle; then — at about the sixth or seventh week of gestation — splits into separate fingers.

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SIGNS AND SYMPTOMS

The way

polydactyly

presents can vary. 

- It may appear as a small, raised lump of soft tissue, containing no bones (called a nubbin) partially formed finger or toe containing some bones but no joints.

-The extra fingers are usually smaller and abnormally developed than normal and can be made up

of:skin

and soft tissue—the simplest to remove

-Skin, soft tissue and bone but no joint

-Skin, soft tissue and bone with joint (closer to a fully-formed finger—most challenging to remove/remodel)

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SIGNS AND SYMPTOMS CONTD

Each person with syndactyly tends to experience different symptoms, as webbed toes may be:

-Unilateral or bilateral: Impacting only one side of the body or both.

-Severe, moderate, or mild: With digits almost entirely fused, with digits partially fused, or with only minor webbing between digits.

- Symmetric or asymmetric: Appearing alike and in the same region on both sides of the body, or appearing dissimilar or in different places on each side of the body.

- Simple or complex: Involving only two digits or a few bones, or including multiple digits or bones.

- Painful or asymptomatic

.

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Minor cases may not interfere much with toe or foot movement and function. However, if the toes are severely webbed or fused, the condition can be disabling.

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DIAGNOSIS OF POLYDACTYL

Polydactyl can be seen by ultrasound prenatally and by eye (i.e. physical examination) at birth. Your doctor will use x-rays to assess the underlying structure of your baby’s finger and determine a course of treatment.

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Syndactyly may be seen by ultrasound prenatally and is apparent at birth. Your doctor will use x-rays to assess the underlying structure of your baby’s fingers and determine a course of treatment. If the syndactyly is associated with a genetic syndrome, doctors will evaluate the baby’s entire upper extremity, chest feet and head/face to detect other abnormalities

.

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TREATMENT OF POLYDACTYL

Treatment for polydactyl depends on how and where the extra digit is connected to the hand or foot. In most cases, the extra digit is removed in the child’s first two years. This gives the child typical use of their hand and allows their feet to fit into shoes.

Sometimes adults will have the surgery to improve the appearance or function of their hand or foot.

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Surgery is usually outpatient, with local or topical anesthesia. Different surgical techniques are a subject of ongoing research.

Removal of an extra little finger or toe is usually a simple procedure.

In the past, nubbins were usually just tied off, but this often left a bump. Now surgery is preferred.

The child will receive stitches to close the wound. The stitches dissolve within two to four week

s.

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Fifth digit:

Removal of an extra little finger or toe is usually a simple procedure.

In the past, nubbins were usually just tied off, but this often left a bump. Now surgery is preferred.

The child will receive stitches to close the wound. The stitches dissolve within two to four weeks.

Thumb or big toe:

Removal of an extra thumb may be complex. The remaining thumb must have an optimal angle and shape to be functional. This may require some remodeling of the thumb, involving the soft tissues, tendons, joints, and ligaments.

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Central fingers or toes:

This surgery is usually more complex and requires remodeling of the hand to ensure it’s fully functional. It may require more than one surgery, and the child may need to wear a cast for a few weeks after surgery.

Sometimes a pin will be inserted to hold the bones together while they heal.

The doctor may prescribe physical therapy to reduce scarring and help improve function.

This procedure is typically performed when the child is between 1 and 2 years old. At this age, the child is old enough to tolerate anesthesia and surgery but is not at risk for missing developmental milestones such as grasping (

prehension

).

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In general, the skin is split evenly between the two fingers with

zig-zag

incisions (z-

plasty

). Only one side of a finger is separated at a time in order to avoid complications related to the skin coverage and blood supply of the affected finger

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THANK YOU