10
&~mt J. PMi,#. 4e: 114, 1975 FOR GENERAL PRACTITIONERS t EXAMINA'I'ION OF THE NEWBORN Y^m P~UL Jodhpur infants may be clue to inlraeranial Head haemorrhage, subdural baematoma or The shape of the head at the time meninghis. If bulging appears o:, the of birth is very variable due to moulding third or fourth day it is morelikelyto during the process of birth and so the be due to meningitis. A d,p, essed or diagnosis of any torm of c, anio+ya:~stosts sunke, l~man+lle o(:cu, s in det~yd~ation. should not be attempted at the time of Capwt succeda~:eum I, ceph;~lic p'ese,ta- birth, tiou especially in p:imiparity or when The size o['thehead also varies to the head is large and the outlet is smait, some extent, due to mouldil~g during the the labour is p]olongvd and there isa birth process. The average head sob tbsue swelling over the presen+irg circumference in the t~ewborn is 33 cm. part. the SUtUres override one another and is bigger by' 3cm. than the and the to-tanelles are tiny or circumference of the chest. [fthehead obliterated. The swelling gradually is smaller titan the chest ~r if the head disappeart. is bigger than the che.~t by more than Cepha.'haematoraa. Factors responsible 3cm, one may think in terms of for cephJthaema,oma are almost the microcephaly or macrocephaly respec- same as for the caput, but the sw~lling tively. Absence of sutural separation appears generally around the fourth day (synostosis) or excessive separation are and is limited sharoly to one cranial both abnormal and rrquire re-assessment bone. Rarely the,e may be more than after a few days. one haematoma. Depending upon the Funlanelle. Immediately after birth, quantity of blood in the swelling, the overriding of the sutures may be present turnout is soft and mushy or quite tense. and the fontanelles barely open. A few U.Jess the mass is very tense one can days later, however, some separatiml of feel the bony floor, a point of distinction the sutures occurs, and the anterior and from congenital foramina of the under- posterior fontanelle~ can be appreciated, lying bone. "]'he anterior fontanelle h~,s an Ctanfimm bifidum with encephalocoele. average diameter of about 2.5cm. It Cranium bifidum is the cranial analogue bulges somewhat when the infant cries, of spiaa bifida. There is incomplete Bulging ol the fontanelle in newborn closure of the bones of the skull, usually at some point in the rnidline. *Fron the Department of PaediaMrics, S.P Medical It is mostly accompanied by protrusion College, BikaLter. This is Part 1I of a three-part Of the meninges which contain el:her review, fluid alone or heterotopical nervous Received o- April 29, 1972: tissue.

For general practitioners examination of the newborn

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&~mt J. PMi,#. 4e: 114, 1975

F OR G E N E R A L P R A C T I T I O N E R S t

EXAMINA'I ' ION OF T H E N E W B O R N

Y ^ m P~UL Jodhpur

infants may be clue to inlraeranial H e a d haemorrhage, subdural baematoma or

The shape of the head at the time meninghis. I f bulging appears o:, the of birth is very variable due to moulding third or fourth day it is more l ike ly to

during the process of birth and so the be due to meningitis. A d,p, essed or diagnosis of any torm of c, anio+ya:~stosts sunke, l~man+lle o(:cu, s in det~yd~ation. should not be attempted at the time of Capwt succeda~:eum I , ceph;~lic p ' e se , t a -

birth, tiou especially in p:imiparity or when The size o [ ' t hehead also varies to the head is large and the outlet is smait,

some extent, due to mouldil~g during the the labour is p]olongvd and there i s a birth process. The average head sob tbsue swelling over the presen+irg circumference in the t~ewborn is 33 cm. part. the SUtUres override one another and is bigger by' 3cm. than the and the to-tanelles are tiny or circumference of the chest. [ f t h e h e a d obliterated. The swelling gradually is smaller titan the chest ~r if the head disappeart.

is bigger than the che.~t by more than Cepha.'haematoraa. Factors responsible 3 c m , one may think in terms of for cephJthaema,oma are almost the microcephaly or macrocephaly respec- same as for the caput, but the sw~lling tively. Absence of sutural separation appears generally around the fourth day (synostosis) or excessive separation are and is limited sharoly to one cranial both abnormal and rrquire re-assessment bone. Rarely the,e may be more than after a few days. one haematoma. Depending upon the Funlanelle. Immediately after birth, quantity of blood in the swelling, the overriding of the sutures may be present turnout is soft and mushy or quite tense.

and the fontanelles barely open. A few U.Jess the mass is very tense one can

days later, however, some separatiml of feel the bony floor, a point of distinction the sutures occurs, and the anterior and from congenital foramina of the under- posterior fontanelle~ can be appreciated, lying bone.

"]'he anterior fontanelle h~,s an Ctanfimm bifidum with encephalocoele. average diameter o f about 2 .5cm. I t Cranium bifidum is the cranial analogue bulges somewhat when the infant cries, of spiaa bifida. There is incomplete Bulging ol the fontanelle in newborn closure of the bones of the skull,

usually at some point in the rnidline.

*Fron the Department of PaediaMrics, S.P Medical It is mostly accompanied by protrusion College, BikaLter. This is Part 1I of a three-part Of the meninges which contain el:her review, fluid alone or heterotopical nervous Received o- April 29, 1972: tissue.

p/I, t l L ~ E X A b I I N A T I O N OF T H E NEA.~/I]ORN IL5

Cranium bifidum is recognized clinically by its encept:aloccele.

Usually the tumour is in the midline; only a few present in the parietal region through a defect in the suture line. In

the rnidline, most of them are occipital, a few are nasal, frontal or nasophatyn- geaI. Meningocoele o r encephal.o - meningocoele may be ,.mall or large and covered with zidn or a fine translucent

membrane , in which case the entire tumour is red. Cran ia lmeningocoe le is

pulsatile and the pressure increases when the infant cries.

Meningocoele of the anterior portion of the brain may preser~tin the forehead. I l i t is basal it may present at the root of the nose, or in the nasal

cavity on one side, or within theorb~t, cau.,ing proptosis. It may p~eset t t e v e n

within the mouth or 1he pharynx.

Occipital encephaloco,qes are usually large, cop~taln brain tissue and may be sessile or pedunculated.

Occipital encephalocoeles are usually large, contain brain tissue and may be sessile or pedunculated.

Macrocepha?us. I f the circumference of

the head at birth exceeds or,e-half of the total body leng,h in cm. plu- ten, it indicates macrocephalus. This condition

is very rare. The head is large at birth, the fontanelles are ~ ide but not tense. Transi l luminalion is negative, and vent, iculog,ams are normal. H3drocephalt~s. The t ram hydlocephalus is limited for the cases in which there is not only an exce.~s of fluid within the ventricular system, but the fluid is or has been under (xccssi~e p~essure. Ventri- cuh,grams are abl ,ormal. Hp'dranenceph,,f_i,. This te;m is used to

describe the condition in which the

cerebral hemispheres are completely or

almost completely lacking, their normal site being occupied by fluid. Transillu-

ruination is highly positive, ventriculo- grams are abnormal arid pneumoence-

pha]ography clinches the diagnosis; the air bubble moves fi'eely to the top in all

positions showing that no cerebral mass imoedes its free movement . Pseud~.h.)d~ocet,halus. (Russell dwarf) . In pseudoI-Lydrocephalus the head size is normal, but it appears to be very large

in comparison to the tiny face and dwat f~d body. The characteristic features are dwarfism, crat:iofacial dysostosis, disproportionately short at'ms, hemia- tropily a , d a variety of othe[ anomalies.

There is slight macrocephaly in achondroplasia. The head is tr iangular

ante1 opos tes iolly, with the broad

~pacmu: calva~ium ta|~ering down to a

n~,ttow base. The ,~.thet characteristic fea tmts ate sh,nt length and the ratio between the upper and lower segmea:ts (top of head to symphysis pubis: sym-

ph~sls pubis to heel) exceeds 17:t0. The

finger tips of the fully extended arm hardly reach the hip joint. The face is

triangular and narrow. Subdnrai h a e m a t o m a atso causes

macrocephaly. There may be in addition

vomiting or convulsions. (Sauiotabe.~. Denting as in a ping-pong ball may be ploduced by pressing the

cranial b,mes in case of prematu, ity, osteogej~.csis impetfecta, ctcido-crarAal dvsostosis, cretinism and mongolism. Congenital parietal foramina. On deep palpation, the bony floor is missing.

They are always symntetz ically bilateral. The co:,dit ioni31amilial . Herniation of cranial cow, tents through, these holes is

extremely rare.

I16 INDIAN JOURNAL OY PEDIATRICS VOL. 40, No. 302

Depretse4 flacture of Jktdt bones. This is caused by trauma particularly by appll. cation of the forceps. It can be seer, and felt as a small depression in the smooth contour of the skull.

Face

The faccls not only an i n d e x u f the m;nd but is also an index of intra- uterine stress and strain, congenital malformations and illness.

Fac ies

Broad facies arc seen in the basal cell r, aex'us sytldrotne. Tile associat,.d features are mental deficiency and rib

anomalies.

Triangular facies are seen in Silver's syndrome. There is a down Utrned rr~.outh, ahort s t a tu re and clinr,- dactyly of the little finger.

Hear t shaped facies are seen ~n Tmner ' s s.vndrome.

Round facies are seen in Albright 's hereditary osteod~,strophy ((pseudohy- popar~tby~oidlsm, sho, t stature, mental deficiency and obesity) and c'i 4zl ~It~l syndrome.

FI~,t facies are seen in Dotvrt's s~ ndroroe, Larsen's syndtome (sho~t metacarpals and multiple joint disloca. tions) and cerebro-ltepatorena] syt.drome (high forehead, short statute, hypotoni~, hepatomegaly and death in ea:ly infancy).

Coarse facies are seen in ctefiDism, generalized gangliosidosis (familial lieU-

tovisceral lipldosi~, p:eudo-l-lu~ler's syn- drome), Hu t l e t ' s synd romea t ld Hunlet 's syndrome.

Mildly coarse facies ale seen in Maroteaux-Lamy syndrome (mucopoly- sacchmidosis (MPS) t)pe VI), MolquiCs

disease (MPS type IV) and Sanfilippo

syndrome (MPS type l I I ) ']'he face is ex3ressionless in bila-

teral l~[oebius's syndrome, mental deficiency, scleroderma of the face and collodion face.

The moon face is seen in Cushing's syndrnme, nephrotic syndrome, acute nephritis and superior vena cava obstruction.

!n the Hippocratic facies, the eyes

are sunken and dull, the nose is pinched, and the skin is parched apd dark. This occurs in very serious toxic and pretermi- hal ( 'otidit ions.

In rlsus sardonicus (in tetanus) the spasm of tile facial muscles causes re-

tractitm of the angles of the mouth and prosing of the lips.

Pinched facies are found in marasmus and dehydration.

Fac ia l n e r v e p a l s y Facial nerve palsy is due to t rauma

and rarely due to agen~sis of tile facial nerve nucleus, q-he t rauma may occur in uteJo, during labour (including forceps application) or in later life.

ht the nuclear and infranuclear type of paralysls, tl,e upper arm lower areas of distriLution of Ille nerve are affected. The eye canr,ot be closed firmly, the forehead rdoes not ~rit,kle, the side of the face appea~t smooth, the nasolabial

fold ~s obliterated a~d the corner of the mouth droops. Supranuclear palsies -'.ffect only the lower part of tl~e face, leavi.",g movements of the eye and fore- head ul:disturbtd. This type of nerve paralysis is seldom an isolated sign but rep~eset, ts intracrani~l damage of some

kind. When facial nerve paralysis is bilateral , or is unilateral but associated

with other cranial tter~,e palsies

]pAI.yL~IXAMINA'rlON OF THI:'. NE%VBORN I17

especially VI, and other eonger, ital defects, guclear agenesis (MovlJius's syndrome) must be suspected.

Complete paralysis is evident in repose, while signs of partial paralysis or weakness may have to be elicited by making the infant cry.

Port wine marks , Alarge telar,giectasis in the t~i~e-

mlnal area is seen in the Sturge-Weber syndrome.

Facial a s y m m e t r y . The face may be asymmetrlcal

because of herniatroI~hy or due to an abnormal postllre dut in~ is;t0 aute ' ine life. If the jaw has been Iwld against a shoulder or ar~ extremity during the intrauterintr period the mandible may deviate fi'om the mldline. Fa~ ial asymmetry may result from ex,:essive moulding of she cranium or from dis- placement of the m,-,r, dibJe during breech or face presentations, because themandtble is tile otfly movable bone in the lace aJJd is attached to the cranium only at its cond,vlar ]wad by the muscles of mastication. Injury, infectjor0 or arthritis of the grov.'hlg cartilage of the condylar head dining intrauterine life or birth proce,s, may resuh in racial asymmetry. Congenital totticollis also causes asymmetr.~ ol the face.

L i p s The lips arc pale in anaemia, and

livid in cyanosis and cot~gestive cardiac failure. The) are thy and parched in dehydration,

"I he distance betweer, the columolla

of the nose and the upper lip is greater than normal in I-lurler's tyndrome.

There tuay be pits or fistulae on the lower lip in the popltteal web syn-

drome' and the lo~er lip fistula and cleft lip syndrome. '

In hare llp (cleft lip), Ihe cleft may be jusf a barely perceptible dent in the vermilinn bt,cder o, it may extend into tile no~t,'il; it may be unilateral, bilateral ,Jr midiinc. It may or may not he as,~,,ciatcd ~ith , leti parate.

1 he labbl . r sucking tubercle may be present jn suckli,g infan0s. Sucking plaques ,nay also be seen along the e d g e s o l t h e , p p v r and lower lips of the infa.e who sucks vigorously.

There re'q," I.,e fosion of the upper llp to the uuderlying gum in some childrH~ with ,:hou(lrocctode, nml dys- ptasia.

Chei l i t i s [)0yness ,ff the lips followed by

,,~,l,~klixlg. scaling ~lnd cracking and z~ct:otnpat~ied b y a chalacretistic burning ~ensation m,)y bc seen during .acute febrile staaes and these changes may also be due to sensitivity to contact substances (to.~ s and food) and photo~en~itivlty to s~n rays.

Fissming of the angles of the m,-,uth may be p, oduced by a number of causes. Ch~ilr~sis is a l'nrm of fissming caused by riboflavin deficiency. Rh~gadrs are moist, radiatilag lesions occurring about the corne~s of the rl3.out]'l in patterers with congenital syphilis. Rhogades ,nay also be seen in children with moz,goli:m aI~d meuta] deficiency w b.o drool excessively. Perliche is characterized by tissurlng, scaling, thinnit~g, maceration and crusting of the aogles of the mouth and is generally caused by C,:mlida afbicans and

occasionally by streptococci. Swelling , f the hpj. Swelling of one

or both lips m,'ty be due to angioneurotic

'1'18 l~l t~x jotJ~a~^~ ot~ pzD~,~'r~.ms

oedema, nel ~ritie, nephrosis, insect bites, t rauma, abscess, co,,osive poisons, carbuncle, cancrum otis, haemangloma

or lymphangioma.

Parled l ips (open mouth). Prated lips res~tlt flora obstruction in the nose

or ~aasopharyr~x due to obstruction of the posterior ehoanae, large adenoids, and is also seen in mongolism, cretinism

and mental deficiency.

N o s e

The nose must be examined for its patency. One sh,,uld hold ,he mouth fo[cibly closed and" watch for respiratory distress. Similarly the potency of both nares can be estabti~laed by closing the mouth and one naris by turn and watching for re.,piratory difficulty.

The nose shoul.t be examined Ibr snuffles, {a sign of co,,ge,,itai %vphilis), potency of lhe pos'etiot choa.t~ae and the presence of any turnout. Bridge of the nose. A low nasal bridge (saddle shaped) is seen in achondro- plasia, congenital syphilis, cretinism and following trauma to tt~e nose.

A flatt~.ed nasal bridge is seen in Conr adi's disease (chond,od~ soophia calc~ficar.s congeni'a), ctanio- metaphyseal dysplasia of Pyle and aminopteri .- indur sy,,dt nine (~he associated fe,ttmes are cranial dysFl.asia and low set ears).

Eyes

q-he gap betweerl the two eyes is increased (hypel telorism) in Ehler Danlos syt,d, nine, B,~n nevi. -UIh ich lyndtome, '~qaatder, bttlg'+ +y,tdrome and

Schmid Fraccaro syndrome. Hypotelo+i+m is seen it, arlfinoce-

phaly and ttigonocephaly.

Vot. 40, No. 302

The presence of epicanthus shc~uld be noted. Epicanthic folds are promir, ent in monogo!ism, tile X X X X Y syndrome (associated featllres are upslan, i lg of ,be p;,lpebral fisq,re,

limiled elbow ptonation, hypogenitalism, mental deficiencu and short stature), the Ehler-Daolos syndrome, colJgenitai ptosis of tt~e eyelids, the Bonnvie-Uih'ich syndrome, severe infantile hypercal- caemia, glycogen storage disease or'the muscles and bilateral renM agenesis, Upward slant of eyts. The pall,etbtal fissure slat~t5 upwards from wxtifin outward in Dow, ' s syndlome, pema-X syndrome (an additional tealure is patent ductus arterioms), the X N X N Y s'iI,dro,ne, ;1,e Rubinstein-Taybi syt,drome (a~ocia~cd features aie maxLda,y h} p(,pias,a, tmo ocephaly: })road thumbs a:,tt toes. mental deficiency a.ud ~ho, t s~alu, e), az.d the P{ adet- %~,'tlli ...y t~dt ctt:e (asso...;la too features ate tt],pu~t:ott..th~nb dt..tbet~s mellttus, tiypotoaia and r

D~,u,1~ward Slant , f E.yes. It is seen in the chromosome No. 21 longa, m deletion syndrome (a.,sociated features are

micrognathia and large malfo, reed exter,,at eats), the It reache,-Cullins s)'t,dL u.;.tle, the: fi,.:J,I.n/,d-F, a coal o syn- dxome ~,cat eye sy,,d~,ame; assot.tated le,ttu:es are hypettelo, i~m, co lobumaof tl~e tris itlld D. liai attenla)-

~t.lBk,..t't atb0. explt~SlOZ,[esn e]ces a,e seen in cratica,ly Ill a,tO severely debyd~ated lttfants. Strabismus. $u abimaus may be palalytic, cot,ge-i,al concotttttant or pseuclostra-

bi~tt,u~ due to ptolnitteat vpicanthic fu',ds If a f, ash-h~ht is il;ummated in,

front of the e)es. the btizht points of

tefltx in ease of pseud,-,st~ab~smus will be

pAI2L~EXAMINATION OF THE I~II~'I~Bf.)RN i19

seen to fall in correspoudir, g location in each eye all the positions, whereas in true strabismus the points of light will be asymmeterica] in some or all the

positions.

Ptosis (Drooping of the upper eyelid), Inability to raise one or both upper

eyelids may be encountered as a solitary congenital defect or in association with inability to move the eye ball upward.

It may be unilateral or bilateral. Isolated ptosis results when the superior rectus muscle forms and becomes functional, but its bud either fails to

form entirely or produces a defective levator. The combination results from total failure of the superior rectus muscle to develop along with the off.hoot, the levator palpebrae superioris. The other causes are paralysis or involvement of

the oculomotor t,erv% its r,ucleus or

corsica: centre (leading to paralysis of

the le,,ator palpebrae muscle); the ptosis is unilateral, complete altd azsuctated with a squint and a large pupil, it,volve- ment with paralysis of the cervical sympathetic nerve (Homer ' s syndrome),

the ptosis being uniltateral, partial and

~.ith a small pupil, oedema, tumour or trachoma of the eyelid causing increase

in the weight of the lid, tetanu~ and

cerebral ptosis (isolated ptosis). .N.yslogrnus. Nystagmoid movements are seen in subdural haematoma and other causes of intracranial damage, and in

central vision impairment due to opacification m the lens (cataract),

haziness of the cornea, opacification in the vitreous (tet~olentat fibroplasia) and

macular degeneration due to choriore-

tinitil.

Congenital idiopathic n3stagmut, The lens

should be examined for the presence of cataract. Small ooaelties can be detected if the lens is examined from a

distance of about 6 inches through plus 8.0 lens of tile ophthalmoscope.

Opacification of the corneas and

an ur,u.~ually large cornea (bigger than 11 ram) should make one ,uspee~ congenital glaucoma,

The conjunctivae should be

examined for inflammation and discharge.

Dacrostenosis rarely becomes

apparent during the neonatal period.

In this condition the eye remains constantly wet, and tears drop on the cheek from time to time.

Haemangiomas of the eye may be

ill the skin of the eyelids, conjunctivae or within the orbit.

Haema,giomas within the orbit

may produce prop'osis and disturbances of extraocular movements if it of

considerable size.

Dermoid cysts may be present. U~ually they arise from the limbus and o~,'edie part of the cornea and its adjacent conjunctiva.

Retinoblastoma is not uncommon during the neonatal period. O n e should he on watch for its presence because early and vigorous therapy may be life saving. The following signs should suggest the possibility of retinoblastoma: Grayish-yellow reflex from behind the pupil, strabismus, one dilated pupil, and phthislcal eyes.

Pupils. The size of the pupils on

both sides should be equal and reactive

to tight.

l ~ INDIAN JOUENAL OP PW-DIATIICt VOL. 40, No. 302

Dilated pup;Is occur in glaucoma, retlnoblastoma (additional findings are strabismus, grayish yellow reflex from behind the pupil, phthisical eyes), para- ly. is or involvement of the oculomotor nerve, its nucleus or cortical centre (additional findings are ..trahhmus and plosis), and in Adie's syndrome one pupil is larger arm myotonic, and the pa~ellar and Achdles reflexes are often absent.

A small pupil occurs in Hornet 's syndrome (due to involvement of the cervical sympathetic he, re; ptosis is also present).

L,~lcocotia (white pupil). A white pupiil,ry reflex is seen in cataract, retrolental fil)~opla~ia, retinol,lhstoma, persistent primaly h~pe~plastic vireous, severe intraocular inlection a,~d exuda- tire retinopathy.

E a r s

In malformations stemming from the firstbratach~al arch, the ears may be

malformfd. One m u t always look carehdly for abnotm.d skin tags. dimples and deep slr,uses, e~pecially in floats ot and below thettagus. The ears can be grossly malformed, u.usually large or small or unusually low set on the head.

Very low set ears of unusual size and floppiness should suggt.st renal agenesis.

l , trlsomy 16-18 (E) and trisomy 1:3-15 (D) the ears ate ab.otmal atJd low set.

In oculo-auriculovertebral dysplasia (Golder.haar synd r ( .me)one or both exter .a l ahd m,ddle ears ma'r be absevt, displaced dowr,ward or mahota ted, or thee e may be one or mot e tags.

M a n d i b l e

Mic,ognathia. Hypoplasia Of 11~e mandlble is enc~m.tered uncommo.ly

as an isolated f ind ing but more often as

a part of a group of signs,

Bilateral injnries to the growing cartilage of the condylar head or the mandible result in s tile mandible and chin to grow downward and Ibr- ward. causing the entire mandible to be

considerably smaller than normal and much ret,ud~d.

In the Pierre Robin syndrome mlcrognathla is a.~sociated with clelt or

high arched palate, glossoptosis, retrac- tion of the see, .urn .~nd cyanosis.

In hisomy 18 (tlisomy |6-18 or group E) micrognathia may be associated with any o1" all of the following: micro- stomia, low.set and abnormal ears, mental retardation, characres i, tlcally flexed fingers, with N~xion contraction of the two middle digits, whici~ are over- lapped by the flexed thumb and tndez and liltle fi-ger, congenital heart defect (almost always a ventticular septal defect, of, en coupled wlth patent duc,us arteriosus), rocker.bottom feet, syn- dactyly, an abnormally jut,it~g occluut, ptosis of or, eor bo~h ese]ids, genitouri- nary defects, hernias, simian palmar crease and pceponderance of arch patter ns on dermatoglyphtc studies.

In ie rri du chol (cat's cry) syttdrome mictognathia is a.,sr, ciated with mic, o- c. phalv, eplcanthus, oblique palpebral fissures, lt)pertelotism, low set eats, | everement . , l te la rda t ion and a peculiar cry resembling that of a cat.

I . matMtbul.facta| dy~oslosis (the Trencher-Colliers syndrome) microg-

na~hla is associated with ocular fissures slanting dowt~wards from within out. Other associated defects itJciude highly

arched palate, glossopt0sis and large,

PAU'Ib~lgXAII,~INAT"ION O! r "I'VE I'r | ~ !

floppy low set ear.,. In the Cornelia de Lange syndrome,

the mandible is small and associated with synophrys (cont inuou~ eye brows) and thin downturned upper" lip.

In the chromosome No. ~1 long arm deletion syndrome, the associated

findlr~gs are t,h~nlit,g palpebral fissures, large malfolmed external ears, mental deficiency and shots stature.

In the Ballerman Streiffsyndrome, mlcrognathia is associated with micro- ph tha lmiaand cataract, small pinched nose, h~,,potrichosis and d~ort stature.

Palate

Imperfect c]osure of the palate may vary from just a cleavage of the uvula at;d .,oft p,,late, single arm mid- line cleft of the palate with or witl',out hare lip, to doubleclefls tunr,ing from the toil palate forward to e;ther side of the yore, cor,tir~uous with a widely spread cleft Iip on each side, leaving the isolated imermaxillary process and the nose p, ojec,ing up~ard between gaping slits in the face.

In the Oral faciM.digital s)ndrome (OFD ~)ndrome), cleft palate is associ- ated w;th ,hic k muct~buccal fibrous bands bir~dir.g the tongue to the cheeks and gums, )obulations and bifid~.ty or multi- fitlity of the tongue, which also may be incompletely separated from the floor of the mouti,. Other associated deformities are shortness of Mar cartilages and columt-]la, median cleft of the lip and hypertelorlsrn. Occasi~1~ally there are alopecla, coarse hair and dry skin, brachydact)ly, clinodactyly and syn..

dactyly.

Cleft palate is found in the Pierre- Robin s)ndrome and ank~,loglossia

superior. H;gh arched palate is found in mandibu]ofa, ial dysostosls, Pierre- Robin syndrome, and anky]oglossia superior

Tongue The tongue should be examined

for colou% size, mobility and any abnor-

mal growth. It is pale in anaemia snd shock, blue in cyanosis, and yellow in deep j~undice.

Since the tongue follows the neural growth pattern it .may appear compara-

tively large at birth. 'Ihere is macro-

glossia in cretinism, :habdomyoma, lympha~gioma or cystic hygroma, haemavgioma, Hurler's syndrome, glyco- gen storage disease of skeletal muscleJ, and neur ofibromatosis.

The tongue may be adherent in the oral-facial dlgi,al syndrome (OFD syndrome) and aDkyloglossla superior.

Glossoptosis occu, s in mar,dibulo- facial d)sostosis and Pierre Robin syndrome.

A bifid tongue is seen in the oral- l'ac~a]-digital syndrome.

ganu la is a translucent, bluish retention cyst of the subli ,gual glands. This usually appears to one side of the fienutum underneath the tongue.

Frequent protrusion of the tongue

occurs in infants wi, h mental retardation, mongolism, cretinism and Niemann- Pick disease.

Snake like flicking in and out of the tongue--Foote's sign occurs in intracrat~ial haemor, hage or oedema.

Teeth

There may be one or more teeth present at the time of birth, in normal babies or tho.,e with congenital syphilis.

| ~ INDIAN JOUINAL OY PRDtATRIO8

T h e a lveolsr r idses must be exa- mined for t u m o u r s (el~gnathus, el:mils), and retention cysts.

T h r o a t

Examination of the throat of a new born is a difficult job because of the tongue which is difficult to depress. At

times one may have to make the infant cry by depressing the lower jaw forcibly. One should look for an aberrant thyroid, thyroglossal duct cyst and nasopharyn- geal teratoma.

Neck

The neck should be examined for sternomastoid tumour, hygroma, thyro- glossal duct c)st and an enlarged thyroid gland.

In the Klippel-Feil syndrome, the neck is unusually short with poor mobi- lity. Webbing of the neck is present in Turner's syndrome Webbing is caused by abnormal folds of skin running from

the mastoid to the acromial area. One must run the finger along the

clavicles to detect its absence or fracture.

E z t r e m l t i e s

The extremities should be exa- mined for abnormal length, absence of a part, presence of an extra finger, toe or swelling, abnormality in shape or size of hands, finger, foot or toe, absence of or abnormality of joint and range of movements.

The extremities are short in achondroplasla, dyschondroplasias osteo. genesis imperfecta (due to multiple fractures) and the Cornelia deLange syndrome (typus degenerativus ams~elo- damensis). The extremities are longer in arachnodactyly.

VOL 40, No. 50~

The fingers and toes are long in arachnodactyly.

In case of congenital dislocation of the hip there may be an apparent shortening ot the affected limb and the affected thigh cannot be abcLucted fully.

The movements of the joints should be examined. In arthrogryposis, the movements are extremely restric:ed. There may be an extraordinary laxity and abnormally large range of move- ment in congenital laxity of the ligments, mongolism, the Ehlet s-Danlo= syndrome, atoni.c diplegia, amyotonia congenita (Oppenheim's disease), myasthenia gravis and glycogen storage di-~seae of the cardiomuscular type.

The foot should be carefully examined for presence of pes calcane- ovalgus, pes metatarsova, us or pes equinovarus. The space between the great and second toe is increased and there is an ass ,ciated partial synddctyly and prominent skin crease in Dowa's syndr,,me.

The position and movements of the upper limbs should be given due consi- deration. In E, rb's palsy the limo lies adducted close to the chest, it is inter- nally rotated, held extended at the elbow with the forearm pronatecL On applying painful stimuli one can notice the obvious ahsence of abduction and

external rotation at the shoulder and flexion of the elbow and supinatiun. Grasp 1eflex remains strong and there is dors,flexion of the wrist. In Klumpke's paralysis the grasp reflex is abolished, the hand is held limply flexed, and

voluntary movements of the wrist are lost. There may be concomitant paralysis of the cervical sympathetic

PAX~T.-- ZX^MXN^~Om OF THF NEWgORN 12~

nerve (Homer's syndrome), in which

there is miosis, narrow palpebra] fissure

a~d absence of facial sweating on the

affected side.

The hands are sholt in Down's syndrome ar.d cretinism. In Down's

syndrome the fiflh finger is comparatively small and tends to curve inward, and

the space between first and second toes

i.~ increased.

References after the 3rd part wb~-.h will appear in the April 1973 issue of the ~,~1u/.