ODESSA NATIONAL MEDICAL UNIVERSITY

Department of pediatric surgery

Methodical Elaboration
of the practical class for the V1th - years students
Malformations of limbs. Clubfoot: etiology, classification, clinical picture, treatment. Abnormalities of development of the hand: syndactilia, polidactilia.  

Approved at the methodical meeting of the chair 
Protocol № 1 from 27/08/2021
                                                                        Head of the department Losev O.O.

Odessa 2021

	Topic: Malformations of limbs. Clubfoot: etiology, classification, clinical picture, treatment. Abnormalities of development of the hand: syndactilia, polidactilia. Classification, clinical picture, treatment. Dysplastic dislocation of the femur: etiology, pathogenesis, classification, clinical picture, X-ray diagnostics, treatment.”.
	I. Topic actuality: 
Congenital limb deformities constitute approximately 43,6 % of all the pathologies of  muscle-skeletal system. The are characterized not only by cosmetic defects, but also by grave disfunction, morphological and functional immaturity of tissues and organs of a child. Continous process of maturity and growth may be used to correct the deformiies wihout operaion, which is practically impossible in aduls. Congenital clobfoot, congenital elevation of the scapula may be trated conservatively, if diagnosed early. That’s why, early diagnosis of congenital limb deformities is so important.
	II. Studding goal:
	To teach students diagnostic methodsand reatment of congenital limb deformities.
	a) Students must know theoretically:
1.	The classification of congenital limb deformities.
2.	Clinical features of congenital clubfoot.
3.	Treatment of congenital clubfoot.
4.	Typesof syndactyly.
5.	Types of operative treatment of syndactyly.
6.	Clinical manifestations of congenital clubfoot.
7.	Treatment of congenital limb shortening.
8.	Types of polydactyly, tactics of the doctor.
9.	Radiological methods of investigation of congenital limb deformities.
10.	Types of congenital pseudoarthrosis and their etiology.
11.	Treatment of congenital pseudoertnrosis.
b) Students must know to do practically:
1.	To take anamnesis.
2.	To find the main symptoms ofcongenitallimb deformities.
3.	To evaluateradiologicalfindings.
4.	To applay a bandage for tratmrnt of clubfoot.
5.	To apply POP slints.

III. Questions for training.
1.	Right after birth deformation of both the feet is found in a boy (equines, supination, adduction of foot). Your diagnosis. Methods of treatment.
2.	A 6 mjnth old child is treated for clubfoot with application of plaster cast. What will be your decission?
3.	Cutaneous syndactyly between III and IV digits of theright hand iis found in neonate. At which age will you operate syndactyly?

IV.The plan and organization of lesson:
Work type	Time	Activity	Equipment	
		Students	Teacher		
Organization of the practical class	5	Listen	Presence  control	Journal	
Aim setting	5	Listen	Explain		
Checking of the knowledge of students	15	Make	Control	Tests	
Seminar	60	Answer	Control	X-ray-grams	
Clinical discussion	30	Listen	Discuss patients	Patients	
Personal student’s work	60	Make	Control	Patients	
Results of the lesson	10	Listen	Appreciation of each activities		
Task for the next lesson	5	Listen	Explains the task, indicates the literature	

V. Substance of the lesson:
Congenital malformations of limbs are graded to:
1. Insufficiency of development;
2. surplus of development;
3. contractures of the joints on the basis of unsufficiency of development;
4. Systemic deformations.
Among malformations stipulated by developmental insufficiency of a skeleton they determine the following ones: amelia -a complete absence of all limbs, ectromelia -a complete absence of one or several limbs, abrachia is an absence of arms, monobrachia is an absence of one upper limb, apodia is an absence of legs, monopodia is an absence of one lower limb, hemimelia is an-absence of distal part of a limb, phocomelia is an absence of a proximal part of a limb, peremelia is a condition when instead of a limb there is only a minor rudiment, micromelia is characterized by a acute underdevelopment of all limbs, ectrodactylia is a heavy fault of development of a hand, oligodactylia means a reduction of a number of fingers. The malformations  mentioned above are heavy incurable teratisms , but at some cases after rudiments removal and creation of supportable stump, the prosthesis is possible.
Syndactylia is an accretion of the fingers (from the embryologic point of view is it a complete or partial inseparability of fingers) makes of all anomalies of the upper limbs and is met as dermal, membranous, osteal and tip form. Surgical treatment (separation of fingers, displacement of a skin defect with local tissues or with the usage of free dermal plastics, and also by distraction in the apparatus of external fixation implements from 2 years of age, at the terminal form in one year of age. Present time at syndactylia they prefer distractive method of treatment (fig. 40), which does not require a free dermal plastic, i.e. it is softer than other methods (the dermal regenerate allows to model local tissues in any way), provides obtaining of good anatomical and functional outcomes.
Malformations of hands and feet quite often are accompanied by amniotic strangulations, which represent filiform circular and hemicircular compressions down to a bone; they can be watched on proximal parts of segments as well. The membranes are surface and steep, last cause disturbance of lympho and blood supply, trophic disturbances. The operating treatment (deleting of grooves by local tissues plastics) is rotined per the first years of life of the child.
The congenital false joints (pseudoarthrosis) most often are met in the lower third of a crus bones and represent a concequences one of three 3 pathologies: 
a)	Local fibrous a dysplasia; 
b)	neurofibromatosis; 
c)	myeloangiodysplasia. 
The congenital false joints can have latent and manifestated forms. The latent form appears as antecurvation of a cms in the lower third; the deformation progresses with the course of time and step-by-step passes in a manifestated false joint. Clinnical picture at a level of a false joint, the deformation and pathological movability emerges, the axle-load is impossible. At X-ray at false joints the sclerosis of a joint proximal ends takes place, as well as the obliteration of a medullar channel of of a bone ends, which derivates a joint, thinning of joint ends.  At the manifestating forms the surgical treatment is implemented after 2 years. The best outcomes are reached at a polylocal osteosynthesis with deleting of pathological tissues (the broad resection of tissues of a false joint is performed, the ends are confronted; the above segment is cut for further elongation and equalizing of length of a limb, for treatment they pin apparatus of external fixation).
The congenital shortening of a limb can be total or at the expense of a underdevelopment of one segment. At shortening at more than 3 sm. the lengthening by osteotomy and distraction in the apparatus of external fixation is recommended.
Congenital radio-ulnar synostosis is stipulated by inseparability of radial and ulna in an embryogenesis and clinical picture is characterized first by absence of rotatory motions of a forearm, hereinafter - the deformation of bones of a forearm like torsion of joins. Surgical treatment (separation of bones) is performed from 1,5 - 2-years of age.
d)	To malformations stipulated by the excess of development, they refer gigantism and polydactylia. The gigantism can be total and partial (exuberant growth of one part of a limb). The pathology is accompanied by a hypoplasia of lymphatic vessels, lymphostasis. The radical treatment does not exsist. To stop the growth of a bone, probably, by an epiphysiodesis (destruction of growth area). At a partial gigantism (fingers) quite often the amputations are performed. 
Polidactylia often is met at the form of six-fingereness, but can be seven-, eight-, and even ten-fingereness; they distinguish three types of a polydactylia:
1. An additional finger - rudiment;
2. An additional finger (normal form and sizes);
3. An additional finger by bifurcation of main (most often thumb- polex duplex).
If there is an additional finger - rudiment, it is possible to delete it per the first months of the child's life. In all other cases before the operation it is necessary to investigate functional value of fingers (refinement which of the fingers is main for a function, and which one harms it).
To congenital malformations with a leading sign of a joint contracture (one or several) they relate talipomanus, talipes, longitudinal ectromelia, dislocations and so forth.
Talipomanus - manus vara - most often is stipulated by a complete or partial underdevelopment of one of bones of a forearm -ectromelia longitudinale prox., that causes deviation of a hand in the side of malformation (radial talipomanus, ulnar talipomanus). In 50 % of cases a talipomanus is connected with an aplasia of a radial bone; at this malformation the deviation of a hand reaches 180°, as a rule, there is no finger as well, takes place an underdevelopment or absence of muscles, vessels and nerves from the radial side. At a defect of a distal part of a ray, underdevelopment, deformation of a forearm and deviation of a hand are expressed less sharply. A defect of a ulna and ulnar talipomanus are watched 7 times less often, than a radial one. Conservative treatment (redressment correcting splints) starts from the birthday of the child; operating treatment - from 3 years (tenotomy of the truncated muscles, liquidation of a dislocation of a paintbrush, polysisation of its finger, elongation of a segment and so forth).
Except for ectromelia longitudinale pi ox. the talipomanus can be stipulated by amniotic grooves, general diseases (Madelung illness), arthrogryposis or neurologic pathology.
Ectromelia longitudinale dist. is accompanied by rigor contractions in patellar and talocrural joints. At a complete underdevelopment of a tibial bone the cms is truncated, deformed, there is a dislocation in patellar and talocrural joints; foot is in a varus position, the supinations and equinus, quite often there are no I and II toes. At a partial underdevelopment of a tibial bone (the defect of mean and dystal parts) takes place only dislocation in a talocrural joint; truncation and deformation of an anticnemion are expressed in a less degree.
The inherent underdevelopment of a fibular bone is observed 3 times more often, than tibial one. At this malformation a crus is truncated, curved, a foot is in equvino-valgus position, IV and V metatarsus bones and IV and V toes may be absent. These malformations are registered at once after the child's birth. To the age of 6-months they conduct conservative treatment (redressment, correcting splints, bandages), from the age of 6 months the operating treatmentis performed: exposure or deleting of a fibrous cord (if there is no a fibular bone), exposure of the truncated bursa-ligamentous apparatus, tenotomy of the truncated muscles from the age of 2 years, possible elongation) of an extremety with distraction apparatus, transposition of bones and so forth.
The   congenital  talipes   on   frequency  takes   the   first  place   among congenital malformations of a locomotor aparatus it is met among boys twice than in girls. They distinguish the typical and atypical forms of a talipes. The atypical form is observed in 20 % of cases and is stipulated by anomalies of development of limbs (defects of bones of a crus, amniotic grooves), systemic lesions (diastrophic dysplasia, arthrogryposis). The typical talipes is stipulated by an underdevelopment of the medial posterior ligamentous apparatus of the talocrural articulation.
The diagnosis of malformation is made at once after birth. The main clinical signs of a congenital typical talipes are:
1. Supinatio (internal version of a plantar surface);
2. Eguinus (plantar inflection of a foot in a talocrural joint);
3. Adductio (reduction of the anterior part of a foot).
With the child's growth there is a torsion of bones of an crus, plantar inflection of toes (transversal flexion of a foot with formation of a sulcus of Adams), with the beginning of walking on an external surface of a foot the callosities form with a slimy ascus, there is a subdislocation of navicular and cuboid bones, shortening of a foot.
According to T. S. Zatzepin they allocate the following degrees of a talipes:
Mild - subjects to a complete manual correction without the special efforts;
Mean - the manual correction is possible at considerable efforts;
Heavy - the complete 1 manual correction is impossible.
At a X-ray examination (in the children older than 2 years) they study relationship of foot bones, determine talotibial angle, talocalcaneal one; the first of them at a congenital talipes is increased (more than 100°), the second one decreases (less than 35°).
They start conservative treatment at once after birth: the first weeks they execute repressing gymnastics and dressing according to Fink-Attingen, then plastering (the bandages should be changed each 2-3 weeks). At an ineffectiveness of conservative means from 6-months they resort to a surgical intervention (tenoligamentocapsulotomy).
VI. Situational tasks:
1. Limitation of driving back in the left hip joint, linear shortness of the extremity, asymmetry of dermal tucks were revealed during examination of the new-born girl. Other pathologies were not revealed. Your suggested diagnosis is:
А. Metaepiphyseal osteomyelitis
В. Congenital linear shortness of the extremity 
С. Children's cerebral paralysis
D. Birth trauma
Е. An congenital dysplastic dislocation of the left femur
2. A newborn has got a fracture of the left-hand femur in a mean third during labour. Objectively: there are an oedema of soft tissues, deformation and linear shortness of the femur. What medical tactics would you choose?
A. Plaster extension on Shede
  В. Plaster bandage
С. Skeletal traction
D. Bandage on Кrede
Е. Opened reposition
3. An infant, 1 year and 8 month of age, was treated for destructive pneumonia. She began to walk only in 12 months, and thus limped on the right leg. Objectively: the right femur is shortened, the driving back is limited. On the X-ray examination: the left hip joint is without features. The dislocation of the femur takes place on the right, its head is missed. The diagnosis is:
A. The pathological dislocation of the femur
В. Congenital dislocation of the femur
С. Traumatic epiphysiolyisis
D. Pathological epiphysiolyisis
Е. Children's cerebral paralysis
4. A newborn has syndactylia III-IV of fingers of the right hand without deformation; the motions in interphalageal joints are free. At what age is it expedient to operate syndactylia?
А. In the period of newborns
В. Till 1 year
C. At the age from 3 till 6 years
D. At school age
Е. After 6 months
5. Parents with an infant, 1,5 years old, with the diagnosis of talipomanus have applied for consultation to the polyclinic in connection with underdevelopment of the left radial bone.  What do you recommend for treatment of the child?
А. The operative measure is urgent
В. To conduct massage, treatment physical training
C. Conservatively correction with a bandage, operating treatment after 5 years
D. To undertake nothing
Е. Ablation with the subsequent prosthetic repair

Literature:
	
Main literature:
1.	Pediatric surgery: textbook / Losev O.O., Melnychenko M.H., Dilanyan I.R., Samofalov D.O.; edited by Loseva O.O. – Odesa : ONMedU, 2011. — 224 pages
2.	Pediatric surgery (Textbook / Grif of the Ministry of Health of Ukraine / Odesa: ONMedU, 2019, 224 с.) Losev O.O., Melnychenko M.H., and others, 7 people altogether
3.	Kryvchenya D. Y., Lysak S.V, Plotnikov O.M Surgical diseases in children. - Vinnytsya: New book, 2008. - 256 p.
4.	Pediatric surgery. / Edited by Sushka V.I. and co-authors // K. Health. - 2002. - 718 p.
5.	Pediatric surgery. Tutorial. Part 2. Edited by Tolstanova O.K., Rybalchenko V.F., Danilova O.A. and others. Zhytomyr "POLISSYA". - 2016. - p. 225, 322-331

Additional literature:
6.	Emergency surgery of the abdominal cavity (standards of organization and professionally oriented algorithms for medical care) / Edited by Fomina P.D., Usenko O.Y., Bereznytsky Y.S. - Kyiv: ‘Health of Ukraine’ Library, 2018. - 354 p.
7.	Age aspects of acute appendicitis in children and the key to its recognition. Tutorial. 2019. 260 p. Edited by Bodnar B.M., Ribalchenko V.F., Bodnar O.B., Melnichenko M.H., and others. Publishing. ISBN 978-966-697-828-1
8.	Nedelska S.M. Diseases of the hepatobiliary system and pancreas in children. Textbook for 6th-year students of a medical faculty, interns, pediatricians, family doctors / Nedelska S.M., Mazur V.I., Shumna T.E.. - Zaporizhzhia: [ZDMU], 2017. - 113 p.
9.	Violation of defecation in children: constipation and encopresis: Textbook /  Rybalchenko V.F., Berezhnyy V.V. ,Konoplitsky V.S., and others; Edited by professor Rybalchenko V.F., Berezhnyy V.V., Konoplitsky V.S., Rusak P.S. - Kyiv: VIT-A-POL LLC, 2018. - 548 p .: illustrations
10.	Intestinal malrotation in children: from embryogenesis to consequences / Monograph. Edited by Pereyaslova A.A., Rybalchenko V.F., Loseva O.O - K .: PE "INPOL LTM" Printing House "000000000", 2019. - 226 p: illustrations
11.	Intestinal obstruction in children: a textbook for students of the 5th year of medical faculties (specialties: "Medical business", "Pediatrics"). Spahi O.V., Barukhovych V.Y., Kokorkin O.D., Lyaturynska O.V., Pakholchuk O.P., Zaporozhchenko A.H. - Zaporizhzhia. -2015.- 75 p.
12.	Developmental defects in children: a textbook for independent work of students of the VI course of the medical faculty (specialties "Medical business", "Pediatrics"). Solovyov A.E., Lyaturynska O.V., Barukhovych V.Y., Spahi O.V., Shchokin O.V., Makarova M.O., Anikin I.O. Zaporizhzhia, 2013. 165 p.
13.	Bachurin V.I. Malformations of the genitourinary system as a cause of urological pathologies: teaching methodology for independent work of fourth-year students in the specialty: 7.12010001 "Medical business", 7.12010002 "Pediatrics", 6.120102 "Laboratory diagnostics" / Bachurin V.I.. - Zaporizhzhia: ZSMU, 2017. - 86 p.
14.	Shmakov A.P. Workshop on pediatric surgery: manual / Shmakov A.P.- Vitebsk, VSMU, 2016 .-- 309 p.
15.	Makarov A.V. Examination of the respiratory organs in children. Training manual. Makarov A.V., Danilov O.A., Sokur P.P., Rybalchenko V.F., Yurchenko M.I. - C .: Business entity Kolyada O.P., 2005. - 160 p.