ARTHROGRYPOSIS

DR G AVINASH RAO
FELLOW IN HAND AND MICROSURGERY
A SYNDROME – NOT
A DISEASE
 INTRODUCTION
 1st depicted in 1841 by A.W. Otto, he called - congenital myodystrophy.
 Subsequently termed “multiple congenital contractures” by Schantzin 1897,

 Arthrogryposis” by Rosenkranz.
 Greek, literature - meaning "curving of joints" (arthron "joint"; grȳpōsis "hooking /
bent").
 Arthrogryposis Multiplex Congenita term coined by WG Stern in 1923
 Scheldonin 1932 described clinical features of congenital multiple contractures in
a child and used for the first time the name “amyoplasia congenita”
 Other terms were amyoplasia congenita and congenital arthromyodysplasia
 Pathological Anatomy

 Arthrogryposis affects the entire musculoskeletal system. 
 Children with arthrogryposis have absent or hypotrophic muscles
but may retain the associated tendons and overlying fascia.
 As  the  child  grows,  these  noncontractile  tissues  can 
tether  the  joints, which may lead to joint contractures. 
 Because the bones  do  not  have  the  normal  load  of 
muscle  activity  or  weight  bearing, they may be severely 
osteopenic. 
 Cortices in these children tend to be quite thick, but the 
bones are of small diameter and the medullary canal is 
nearly absent. 
 The motor and mixed nerves are also small in caliber
compared with those in other children of the same size. 
 With thin bones and a hypoplastic musculature, the
subcutaneous fat encompasses - a larger than expected percentage
of the bulk of the arms.
 DEFINITION

 The term arthrogryposisis used to denote non progressive conditions

characterized by multiple joint contractures found at birth & It involves
contractures of at least two joints in two different body regions.

 Incidence: Varies Considerably (from country to country)

 Any child with congenital joint contractures in two or more limbs can be
classified as having arthrogryposis multiplex congenita (AMC); the term
does not specify any certain cause for the contractures.
 Arthrogryposis can result from environmental or genetic causes but is most
often idiopathic with wide range of levels of severity and anatomic
involvement.

 Arthrogryposis can also result from myriad disorders that compromise the 
central or peripheral nervous system or the musculature; currently, over 
300 types have been identified.

 Muscles are either absent or hypoplastic, often replaced by fibrofatty tissue.

Tendons and overlying fascia are often preserved even when no muscle is
present, leading to tethering at the joints.
 Children with amyoplasia have a characteristic appearance 
with high cheeks and button noses and “stork bite”
hemangiomas over the occiput and the bridge of the nose.
 Children tend to have average to above average intelligence.
 Terminal limb deficits are more common in amyoplasia 
patients than in the general population. 
 The family history is usually positive in DA and in other
inheritable neuromuscular conditions, but it is otherwise negative.
 Etiology

 It usually occurs due to absence of active fetal movements (akinesia), normally

appearing in the eighth week of fetal life

 Fetal akinesia lasting over 3 weeks may be sufficient to result in absence of

normal stretching of muscles and tendons acting on the affected joints, and cause

reduced compliance of the joint capsule and periarticular ligaments

 Consequently fetal akinesia leads to fibrosis and contractures of the affected

joints determined by the passive position of the limb.

 The direct etiological factor causing akinesia in humans remains unknown.

Hall’s Classification of AMC

1) Primarily Limb Involvement

2) Limb involvement+ other body areas.

3) Limb + CNS involvement

 Pathogenesis – Intrinsic Factors
 Neurologic Deficit  Maternal Considerations
1. Disorders of Cerebrum 1. Multiple Sclerosis

2. Anterior Horn Cell deficiency 2. Diabetes Mellitus

3. Abnormalities of nerve function or structure 3. Myasthenia Gravis

(central and peripheral) 4. Maternal Infection

 Genes resulting in abnormal development of 5. Maternal Hyperthermia

myocytes - congenital muscular dystrophy 6. Drug Exposure
 Connective Tissue/Skeletal Deficit 7. Myotonic Dystorphy
1. Primary disorder of joint/connective
tissue  Intrauterine Vascular Compromise
2. Diastrophic dysplasia. 1. Severe bleeding
3. Collagen disorders - Larsen's syndrome, 2. Failed termination
multiple pterygium syndrome,
3. Monozygotic twins
congenital arachnodactyly, and Beals
syndrome 4. Amniotic Bands
 Pathogenesis – Extrinsic Factors

1) Intrauterine mechanical obstruction

2) Fetal crowding: multiple births

3) Oligohydramnios

4) Uterine myomas

5) Amniotic bands

6) Trauma
 Genetics of Arthrogryposis

 Arthrogryposis is a group of clinical symptoms that can be observed in many

different genetic syndromes :

1) Sporadic

2) Single-gene mutations (e.g. autosomal dominant, autosomal recessive and

X-linked recessive inheritance patterns).

3) Chromosomal disorders (e.g. trisomy 18) such as deletion, translocation, or

duplication, and mitochondrial disorders.
Approach to diagnosis

 Family history

 Pregnancy history

 Delivery history

 Physical exam

 Multidisciplinary approach
 Upper limb
 Shoulder

 Adducted and internally rotated.

 Deltoid muscle function is deficient.

 Elbow

 Extension contracture of the elbows with deficient brachialis and biceps brachii
function, resulting in absent or significantly deficient elbow flexion.

 Flexion contracture of the elbow is less commonly observed. The elbow

joint is cylindrical in appearance and devoid of any skin creases .

 Forearm in neutral to slight pronation

Wrist

 Characteristic palmar flexion contracture with ulnar deviation and pronation of the hand.
 Patients with myogenic arthrogryposis / DA may present with extension contracture of the
wrist.

Hand

 Flexion contractures of interphalangeal joints (most common).

 Metacarpophalangeal joints relative extension contractures.
 Thumb is usually adducted. Finger contractures are usually stiff and most patients have
significant deficiency of active finger movements
 In syndromic arthrogryposis - “clenched fist” with “thumb in palm” deformities may be
observed.
Lower limb
 Hip
1. Mostly flexion, abduction, and external rotation contractures of varying degrees of
severity.
2. Unilateral or bilateral hip dislocation is observed in approximately 1/3 of patients.

 Knee –

1. The most common deformity is flexion contracture of varying severity, Flexion

contracture is usually associated with weak quadriceps and a “dimple” over the patella.

2. An extension contracture is less commonly observed and may be accompanied by knee

dislocation.
 Ankle joint And Foot
 These deformities are observed in nearly all arthrogryposis patients.

 Severe talipes equinovarus (most common).

 Less frequently vertical talus observed.

These deformities are characterized by usually extreme severity, difficulties in

treatment and high tendency to relapse.
 Spine
 Abnormal curvatures in approximately 28% to 67% of patients

 Simple long thoracolumbar curves without concomitant vertebral malformations

 The curves often rapidly progress.

 Extra skeletal manifestations
 Facial skeleton –
 Hypoplasia of the mandible (micrognathia).

 Contracture and limited function of temporo-mandibular joints.

 Extraskeletal clinical signs and symptoms

 Normal intelligence

 Hemangioma on the forehead.

 Abdominal wall abnormalities(inguinal hernia or gastroschisis)

 Varying abnormalities of the reproductive.

 DISTAL ARTHROGRYPOSIS

 Inheritance is autosomal dominant

 Contractures limited mainly to the distal portions of the limbs, i.e. to wrists, hands,
ankles, and joints of the foot.

 Contractures of other joints are low-degree or absent.

 According to Bamshad 10 types of distal arthrogryposis had been described The 

most common DAs are types 1, 2A, and 2B (DA1,  DA2A, 
and DA2B.
Bamshad M, Van Heest AE, Pleasure D: Arthrogryposis: a review and
update. J Bone Joint Surg Am 91(Suppl 4):40–46, 2009.
 Other Arthrogryposis

 Pterygium syndromes
 These are a separate class of genetically mediated congenital contractures,
characterized by the presence of pterygia: these are skin webs located in the area of
a joint and causing limitation of its range of motion.
 Skin webs may also be found in lateral portions of the neck, and be accompanied
by cleft palate or lip, syndactyly or atypical fingerprints.
 Many variations have been described with varying inheritance patterns of clinical
features including autosomal dominant or recessive, e.g. lethal Bartsocas-Papas
syndrome
 Escobar's syndrome (multiple pterygium syndrome)

 Neck webs are evident at birth but are not always severe.

 Clinically the Escobar syndrome is characterized by facial dysmorphism, neck (bucco-

sternal) webs, and hand contractures. With age, the neck webs may increase in size; the
neck mobility is limited due to concomitant congenital vertebral malformations.

 The lumbar lordosis increases with age as well; in adolescence, lumbar lordosis and
popliteal and cubital webs increase in size.

 The inheritance pattern is autosomal recessive, sometimes autosomal dominant; the

syndrome may be associated with mental retardation.

 The lethal multiple pterygium syndrome is autosomal recessive; features include severe
contractures, hypertelorism, cervical pterygia, narrow chest, and hypoplastic lungs.
Popliteal Pterygium

Multiple Pterygium
 Larsen syndrome

 A genetically mediated, autosomal dominant syndrome with an incidence of 1/100,000 live

births, caused by a mutation of the gene encoding filamin B (FLNB), a component of the
actin complex in the cell protein cytoskeleton.

 The clinical features of Larsen syndrome may include multiple contractures, most
commonly in the form of talipes equinovarus.

 The dominant features are hypermobility and congenital dislocations of multiple joints: hips,
knees, and elbows. Cervical spine instability and kyphosis may be present, leading to
potentially life-threatening cervical cord injuries.

 Other features include: laryngomalacia and/or subglottic stenosis, low body stature, central
facial hypoplasia, and accessory metacarpal and metatarsal bones. Mental development is
usually normal.
.
 Bruck syndrome
 Extremely rare, autosomal recessive form of arthrogryposis, with combined
clinical features of osteogenesis imperfecta and congenital contractures; this
disease was historically described by Alfred Bruck in 1897
 Investigations
 Routine radiographs are unnecessary for patients with 
arthrogryposis. Some experts advocate lateral finger radiographs to 
evaluate phalangeal condyle hypoplasia in cases of camptodactyly. 

 Neither computed tomography nor magnetic resonance imaging is 
necessary for the diagnosis or treatment of AMC. 

 Electrodiagnostic  studies  are  rarely  necessary  other  than  in 

evaluating specific neurogenic contracture syndromes. 

 Muscle biopsy rarely yields useful information unless a specific, 
unusual  diagnosis is being pursued.
 Treatment

 The principal treatment goal in arthrogryposis is optimization of quality of life: this

includes communication capabilities, unassisted activities of daily living, social
participation capacity, independent ambulation, and consequently independent
living.

 In order to achieve these goals, management must be initiated as early as possible,

and optimally in the neonate and infant.
 The goal of any treatment plan for a child with arthrogryposis  is to 
maximize functional independence for that child. 
 For the lower extremities, the goals are ambulation and sitting.
 For the upper extremity, the primary goals are to have “one hand to eat 
and one to wipe.” 
 They can be by the same hand.
 The child must be able to acquire the food and Grip it firmly enough to 
bring it to the mouth, and release it. Beyond that, the  wrist  and  the 
elbow  combined  must  have  enough  extension to reach the plate or 
the table and sufficient flexion to allow the hand to reach the mouth. 
 Forearm and shoulder rotation need to allow the hand to face the plate 
and then the  mouth. 
 Perineal care can be performed through the legs or around 
the back. 
 Children who wipe their buttocks through the legs require 
wrist flexion, elbow extension, and shoulder internal  rotation. 
 Children who wipe their buttocks around the back require 
forearm  supination,  elbow  extension,  and  shoulder  extension.
 This comprehensive approach is based on a triad of treatment tools:

 Firstly, rehabilitation including physiotherapy, manipulation of contractures,

and later social and occupational rehabilitation.
 Secondly, individually tailored orthotic management, whether for
maintenance or correction of joint mobility, and for prevention of
recurrent deformities.
 Thirdly, a broad spectrum of surgical techniques for correction of
musculoskeletal deformities, typically found in congenital contractures
 Rehablitation and Physiotherapy

 The parents of a child with arthrogryposis often place the greatest importance on
independent ambulation and concentrate their attention on this ability in the treatment
program .
 It is therefore extremely important that the treatment plan and its objectives – both immediate
and long-term – be communicated to both the patient and the parents.

 At birth - Gentle stretching and ROM exercises

 Passive stretching exercise followed by serial splinting with custom made thermoplastic
splints
 Existing joint motion to be preserved and placed in most functional position

 Stiff joints placed for functional advantage

 2 major goals

 Plantigrade standing and walking

 Restoring function of upper limb to carry out daily living activities

 Shoulder
 Typically present with adduction and internal rotation contracture.
 Capsular releases – not helpful.
 Best – Derotation Osteotomy of humerus.
 Contraindication - Absence  of  pectoralis  function.  Children who 
lack pectoralis function but retain triceps function will use an 
obligatory crossover grasp pattern that requires  internal rotation.
 Medial / DeltoPectoral / anterolateral / Posterior approach.
 Desired position of between neutral and 15 degrees of 
internal rotation - Sufficient internal rotation  should be preserved 
to allow for midline function, such as perineal care
Humerus Ext-Rot Osteotomy

.
 For the child with a severe elbow extension contracture (<30 0 of elbow
flexion) distinguishing between an internal rotation contracture at 
the elbow and a pronation contracture at the forearm can 
be  difficult. 
 For these children, we will perform an elbow release first, 
followed by a humeral rotational osteotomy.   
 Bilateral procedures in these children rarely performed because 
the margin of error is so small for losing midline function.
 Complications - Overrotation  or  underrotation  is  the  most 
common complication. Typically, younger children will correct 
for overrotation but will not correct for underrotation
Elbow
 Range of motion exercises ,Early splinting & Serial casting.

 Stiff flexed – surgery not indicated

 Elbow Extension Contractures :

 One side to be treated at a time

 Posterior capsulotomy and triceps tendon lengthening

 Transfer of triceps, pectoralis, or latissimus dorsi to maintain Elbow stability in

extension.

 Steindler flexorplasty Improves active flexion if passive flexion ≥ 90 °

Elbow – Extension Contacture Release
 Elbow Flexion < 30 - Caretakers and health care professionals are 
discouraged from performing passive range of motion of these 
elbows  because  inadvertent  collateral  ligament  damage  or 
physeal injuries.
 Elbow Flexion > 30 - Impressive gains in elbow range of motion 
can be made with Passive ROM.
 Indications for surgical release - less than 30 degrees  of 
elbow flexion by 12 to 18 months of age and failure to 
achieve  sufficient elbow flexion for self-feeding at any age.
 Contraindicated - contralateral  side elbow flexion contracture that 
prohibits perineal care and Absent Triceps function ( flexion contracture).
 Posterior approach (Dominant Hand First)– Ant.Transposition of Ulnar Nerve
 Distally based V-Y advancement – IF INADEQUATE - Turndown flap /
Autograft /allograft.
 Additional Release of Medial head of Triceps all the way upto Spiral groove
subperiostially – Helps.
 Flexion if inadequate – Perform Ant.Closed Wedge Humeral Osteotomy
proximal to Olecronon fossa.
 Release till flexion is B/W – 90 – 120 degree.
 Hour-Glass Splint Maintains the Flexion.
 Avoid Forceful Elbow Flexion – might cause Transphyseal Fractures.
Complications – Triceps Insufficiency and Elbow Stiffness.
 In a child with passive motion who would benefit from active 
elbow  flexion,  there  are  several  options  for  achieving  active flexion.
 Transfer of the entire triceps to the biceps has proven to be a poor 
option.
 Transferring only the long head of the triceps – proponents of this 
technique have  yet to demonstrate a substantial improvement.
 Other  options  for  restoring  active  elbow  flexion  are  a  Steindler 
flexorplasty, a unipolar or bipolar pectoralis major transfer, a bipolar 
latissimus dorsi transfer, and a free muscle  transfer.
 When present, the latissimus dorsi is the best choice for restoring
active elbow flexion
 Bipolar Latissimus Dorsi Transfer
 Contraindicated – Poor / Absent Triceps function due to previous
lengthening and Passive ROM of elbow < 90 degree.
 Expected outcome – power of 3+ and above can be achieved. It depends on
the quality of harvested LD.
 As long as the latissimus dorsi is in good condition, the 
bipolar latissimus muscle transfer is a fairly reliable technique
for achieving active elbow flexion. 
 Another viable option is a free gracilis with a spinal
accessory nerve transfer. 
 Wrist
 Wrist Deformities:
 Volar flexion and ulnar deviation
 Splinting shortly after birth
 Surgical indication -

 For fixed wrist contractures interfering with function

 Release of:
 Volar wrist capsule.

 Flexor Carpi Ulnaris tendon transfer to Extensor Carpi Radialis Brevis.

 Osteotomy of distal radius.

 Intracarpal extension osteotomy.

 Post-op splinting ….. to improve dorsiflexion.

 Arthrodesis - Near skeletal maturity in slight palmar flexion.
 Radial Osteotomy With And Without Ulna Osteotomy

 Forearm in case of Arthrogryposis – most ofen Neutral.

 It is Performed in cases of forearm pronation contracture.
 Differentiate between shoulder internal rotation contracture and forearm pronation
contracture – to avoid mismanagement.
 It is difficult to differentiate both in case of elbow contracture in extension .
 So, before doing humerus or forearm osteotomy – perform an elbow release.
 A radial osteotomy is usually sufficient for rotational correction 
below 45 degrees. Beyond that, an ulnar osteotomy can be 
added for corrections up to 90 degrees. 
 Alternatively, a onebone forearm is more predictable and easier to 
accomplish.
 Contraindicated - A child who requires pronation for perineal care 
should not  undergo a neutralization osteotomy of the forearm.
 A one-bone forearm is the most predictable operation for
correcting  forearm  rotational  deformities.  Union  in  children 
is rapid and robust, and there is no risk of loss of correction 
over time.
 Correction of a pronation deformity can be a life-changing event 
if it allows the child to self-feed for the first time. If it takes 
away or prohibits the acquisition of independence in the bathroom, 
 Loss of forearm pronation can have lifelong consequences.
 Carpal wedge osteotomy
 The carpal wedge osteotomy is a common upper limb procedure for 
children with arthrogryposis. 
 However, the indications are few  and  the contraindications  are  many. 
 Decisions about altering a child’s wrist  position require a  thorough 
assessment of the child’s overall functional goals and limitations
 Most patients with DA tend to have wrist extension contractures, 
whereas patients with amyoplasia have flexion contractures.
 Surgical interventions in the  wrist  and  hand  have  not  been 
demonstrated  to  have  better  outcomes at a younger age, 
surgery can be delayed until the child’s functional needs and
capabilities are better defined
 Extension contractures at the wrist are well tolerated if 
the following criteria are met: 

(1) Finger extension is sufficient to acquire and release objects, 

(2) Forearm supination and elbow flexion are sufficient to get 
food  to the mouth.

(3) Forearm supination, shoulder extension,  and  elbow 

extension  are  sufficient  to  be  able  to  wipe  the 

perineum from behind. 
 If the child has good finger extension and has sufficient pronation 
and elbow flexion to reach the mouth without the need of  wrist 
flexion, and can perform perineal care around the back, the  most 
reliable  procedure  to  provide  wrist  extension  is  a carpal wedge 
osteotomy with ECU to ECRB transfer.
 The osteotomy cut should correct both wrist ulnar deviation and 
flexion.
 The best candidates for this - reciprocal deformity of wrist flexion and 
MP extension.
 In cases where one hand is to be used for wiping and the 
other for eating, the eating hand can be considered for a carpal 
wedge osteotomy.
 Contraindications - to correcting a child’s wrist 
flexion contracture
1. Poor finger extension in maximal preoperative  wrist extension,

2. Obligatory wrist flexion for hand-to-mouth  movement,

3. Obligatory  wrist  flexion  for  perineal  care,

4. Use of the back of the wrist for scooting along the
ground  for  ambulation  or  for  balance  and  support 
during  standing or sitting.

5. Complications – Undercorrection, Loss of finger extension and increased

finger stiffness, Nonunion (rare).
Thumb
 Thumb Reorientation and
Metacarpophalangeal Chondrodesis
 The classic clasped thumb in arthrogryposis consists of extension at 
the first carpometacarpal (CMC) joint and flexion at the MP joint. 
This is more commonly seen in distal arthrogryposis. CMC joint 
generally follows the position of the  wrist.
 Children  with  wrist  flexion  contractures,  as  are  more  commonly 
seen  with  amyoplasia,  typically  have  CMC  joint  flexion 
contractures with the MP joint in extension.
 Surgical options should seek to place the thumb in a position where 
it can best provide pinch and / or grasp, regardless of appearance. 
 Thumb reorientation osteotomy is used to correct CMC flexion.
 Thumb MP chondrodesis is used to correct MP flexion.
 Patients with no  digital  motion  usually  lack  any  active 
motion  in  the  upper  extremity. 
 If FPL function is preserved despite no other function - The 
thumb orientation prevents useful prehension, and these patient are
good candidate for a thumb reorientation osteotomy.
 The goal of the osteotomy is to place the thumb into a position
where it can best take advantage of the child’s existing finger 
and thumb motion to provide pinch and / or grasp. 
 Both type 1 and type 2 thumbs benefit from a first web 
space contracture release at the same time as a chondrodesis 
or reorientation osteotomy. 
 MP chondrodesis alone will not address a CMC flexion contracture. A
combination of MP chondrodesis and reorientation osteotomy may be
required.
 Using a reciprocating saw, make an incomplete transverse osteotomy 
just distal to the physis.
 Referencing the thumbnail, make a second cut to create a 
dorsal closing wedge at the desired angle  Complete both 
cuts.
 Reduce the osteotomy by extending, abducting, and pronating 
the thumb
 The  stiletto  flap  is  the  workhorse  flap  for  the  thumb  in 
arthrogryposis, allowing both expansion of the first web and 
resurfacing of the volar MP joint. 
 It is rare in these children to require only first web space 
expansion - so a four-flap Z-plasty or an Abdel Ghani flap is less 
often used.
 For children with fixed thumb-in-palm deformities who can flex their 
fingers over the thumb, the standard operation is the Thumb MP 
chondrodesis with first web space expansion. 
 The functional and aesthetic gains are typically dramatic, and most 
patients will ask for the same operation on the contralateral side once 
they realize the results.
 For children whose fingers do not reach the thumb and a 
thumb  that  does  not  reach  the  palm,  pinch  is  not 
possible  without reorientation of the thumb. 
 The fingers must actively flex sufficiently to reach the thumb 
in its planned position in order to achieve postoperative pinch.

Contraindications –
 Fixed extension contracture at the CMC joint that would preclude 
opposition after the MP chondrodesis. 
 Children with stiff fingers that would not reach the thumb even 
after reorientation or MP fusion are not candidates for thumb 
surgery.
 Camptodactyly Release
 Camptodactyly is more common in DA and can be a defining characteristic of
specific syndromes such as Beals syndrome (contractural arachnodactyly).
 Camptodactyly can interfere with capturing objects in the hand, with some 
children developing  a  compensatory  pinch-and-grasp  pattern  against  the 
dorsum of the contracted fingers 
 Radiographs are not useful for camptodactyly in young children. In older 
children, the condyles at the PIP joint will often  appear hypotrophic
 Children with passively correctable camptodactyly are more likely to have well
formed condyles than children with fixed flexion contractures. In general, the 
more severe the condylar hypoplasia, the less  correctable the deformity.
 Standard treatment for camptodactyly is releasing the pterygium by
Z-plasty along with FDS tenotomy (if the FDP is functional 
and the FDS is tight ).
 If the camptodactyly is passively correctable and there is 
good FDS excursion, the FDS can be transferred to the 
lateral band to augment the extensor mechanism. 
 Results  of  camptodactyly  release  are  unpredictable,  despite 
some  favorable  results  in  the  literature.  
 It is rare that a patient will be worse off, unless one is 
attempting extensor mechanism augmentation.
 Contraindications - A  relative contraindication for FDS tenotomy 
is lack of the flexor digitorum profundus (FDP) and intrinsic 
function.
 Complications - Stiffness is the most common complication after 
any camptodactyly release. Digital nerve injury and vascular injury 
are  possible  and  are  most  often  traction  injuries  caused 
by straightening a bent finger.
 Summary
 Arthrogryposis remains a challenging disease for the patient, the parents, 
and the treating practitioner.
 Fortunately, the initial joint contractures tend to improve over time with 
stretching and splinting. 
 Each child follows his or her own path, however with some returning to 

full function and other less fortunate children never developing any muscle  

tone. 

 There is little margin  for error in these children, and parents 
should be aware that any surgical intervention may risk
compromising existing function. 
 Surgery should be pursued only if the potential benefits in 
function outweigh the potential losses.
 Operating on one arm at a time provides a mechanism for 
the surgeon, patient, and family to judiciously assess the
outcome.
 Although the options are often limited and the results of 
surgery are not always predictable, helping these children 
transition  from  dependence  to  independence  is 
tremendously  rewarding. 
 Sterling Bunnell’s famous words, “To someone who has
nothing,  a little is a lot”   particularly  apply  to  these 
children.