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1981;67;753PediatricsLouis I. Sobel, Jeffrey G. Odel and Alan H. Friedman

On Angiomas of Retina, Brain, and Skin

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ISSN: 0031-4005. Online ISSN: 1098-4275.

PrintIllinois, 60007. Copyright 1981 by the American Academy of Pediatrics. All rights reserved.by the American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village,it has been published continuously since 1948. PEDIATRICS is owned, published, and trademarkedPEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication,

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LETTERS TO THE ED ITOR 753

th e fir st var iab le in th at the contro l gro up rec eiv ed nu rs-er y care before nurses w ere aw are of the trea tm ent tech -n iques . T he au thors p o in t ou t tha t paren ts m ay havein trodu ced som e v ar ia tion in tha t the experim enta l fam -lies , hav ing observed th e in te rv en tio n prog ram in thenurse ry , m ay have m odeled the ir sub sequen t behav iora fte r the care tak ing ob served in the nursery and influ -en ced short-term resu lts. A ctu ally th is w ould have li ttlepractical im portance in that it w ould not m atter w heth erim p roved deve lopm en t w as due to n urse in tervention ,paren ta l ob servation of the in terven tio n , o r bo th . A po-ten tia l va riab le n ot m entioned by the authors co nce rnsin fo rm ed consen t. They ind ica te th is w a s ob tained , b utnot w heth er con tro l paren ts knew th eir ch ild w as no trec eiv ing sp ecia l care or w heth er ex per im enta l paren tsknew the ir in fan ts w ere be ing en riched . I f th is w ere so ,paren ts exp ec tations and p erc eption s could have alteredth eir re la tio nsh ip w ith th e ir in fan ts and th is m ig h t havein fluen ced th e outcom e in th e dire ction of th e resu lts .This w ould h ave m o re prac tica l im por tance becau se itw ou ld pro bab ly no t pe rsist a fter the en richm ent p rog rambecam e standard .

O f g rea tes t im po rtance is th e n ece ssi ty fo r abso lu teobjec tiv ity in m easuring ou tcom e fo r b eh av io ra l research .The B ay ley m o tor an d d eve lopm enta l testing w as doneapprox imate ly six m on ths fo llow ing nu rsery disch arg e byone o f the authors, and s inc e con tro l subjects w e re testedbefore the exp erim en tal su bje cts, th is au tho r w ould h aveb een aw are of a ssig nm en t in ea ch ca se. The B ayley tes tfo r a 6-m onth -o ld in fan t ha s a d efin ite sub ject ive com -pon ent , a nd it is do ub tfu l that anyon e w ho had in ve stedten m onth s cou ld rem a in object ive , irre sp ective o f in ten-tions or in te llec tua l h onesty . H opefu lly the teste r w asb lin d , an d if n ot, subsequ ent s tud ies o f th is ty pe shou ldutilize da ta co llec to rs unaw are o f experim enta l v s con tro lstatus.

RE FER ENCE

W ILL IAM A . ALTEM E IER III , M DDepartm ent of Ped ia tricsV and erb ilt U nivers ity M edica l C en te rN ashv ille, TN 37232

1. L eib SA , B enf ield G , G u idubald i J: E ffec ts o f ea rly in te rven-tion and s tim ula tio n on the p reterm infan t . Ped ia trics 66:83 ,1980

I n R ep ly .-W e apprec ia te D r A ltem eie rs in te res t in our paper,

and w ou ld like to re spond to th e issue s he ha s raised .A s sta ted in the paper, d uring the tim e period of the

con tro l g roup s tudy , stand ard nurse ry care w as g iven toa ll in fan ts in the n eona tal in ten sive care u n it. T here w ereno infan ts rec eiv ing the p rescrib ed enrichm en t pro ce-dures at tha t tim e. P aren ts of the con tro l in fan ts g aveth eir in form ed w ritte n consent for their in fan ts to p art ic-ipa te in a research pro jec t to m easure the ir in fan ts g row th and d eve lopm ent. P aren ts of the infan ts in th eexperim enta l g roup gave in form ed w ritten con sen t fo rth eir in fan ts to p art ic ip ate in a spec ial en ric hm ent pro -gram in th e nurse ry , w hich w as to be adm in is tered by thenu rses . B o th groups of paren ts w e re inform ed th at w ewou ld use the fo llow in g evaluat ive m ea su res: adm inis tra -tion of the B ra zelton N eona tal B ehavio r A ssessm ent

Sca le while th eir in fan ts w ere rece iv ing in te rm edia te andconva lescen t care in the nu rse ry and adm in istra tion ofthe B ay ley S ca les o f In fan t D eve lopm ent, g row th m ea-su rem ents, and phys ica l exam in atio ns a t s ix m onths pas texpec ted da te o f con fin em en t.

The pre- and postassessm ents w ere adm in iste red by al icensed ch ild c lin ical psycho log ist, w ho w as no t b lind .W e agree tha t subsequen t s tu d ies u sing in dependen t ev al-ua to rs who are un aw are of ex perim en tal vs con tro l sta tuswould be h ig h ly d es irab le . H owever, w e offe r the fo llow -ing .

A t the tim e of the stu dy , the au tho r who adm in is te redth e B razelton Sca les and B ayley S ca les w as a PhD grad -ua te stud en t w ith exp er ience in adm in iste ring n ew bornand infan t assessm ent m easu res. To m in im ize sub jec tiv -ity , a re liab ility check w as ob ta ined by tw o observers, oneof whom was a licensed c lin ica l psy cho log is t w ith expe-r ience w ith the B ay ley S ca les, and the o the r , a g rad ua tes tuden t w ith tra in ing in th e adm in istra tion o f the B ay leySca les . In add itio n , the au th or w as tra ined to reliab ilityin th e ad min is tra tio n of the B raze lto n S ca les a t theU niversity of K an sas In fan t L abora to ry . H er re liab ilityw as aga in checked by T . B erry B raze lton , M D , a t theCh ild ren s Hosp ita l M edica l C en te r, B o ston . F urther-m o re , a 93% agreem en t w as fo und b e tw een ped iatric iansand psycho log is ts independen t ra tings of th e in fan ts curren t leve l o f deve lopm ent. W e hope th ese com m entshe lp clarify the issues ra ised by D r A ltem e ie r .

D . G AR Y BEN FIELD , MDSU SAN LEIB , PHDD epartm en t o f P ed iatr ic M edic ineChi ld ren s H osp ita l M edical C en te r o f A kron281 Locu st S tAkron , OH 44308

On Ang iom as o f R etina , B ra in , an d Sk inTo the Ed itor.-

Recen t ly Russe ll et a l (Ped ia tr ic s 66:306 , 1980) de-sc ribed a ch ild w ith a h em ang iom a of th e righ t side of thefo rehead and righ t upper eye lid , an ar te riov enous m alfo r-m ation of the basa l gang lia and suprase lla r reg ion , an dan ips ila te ra l ang iom atou s ocu la r lesion , w ho had aniso la ted grow th horm one d ef ic iency . T hey conc lud ed tha tth e vascu la r m alfo rm ations w ere consis ten t w ith a varian tof th e Sturge-W eber syndrom e. It is our op in ion tha tthe ir fundus and ce reb ra l ang iogram illustratio ns and theclin ica l descrip tio n of th e cutaneous va scu lar n evi repre-sen t a case of W yburn -M ason syndnom e.24

T he W ybu rn -M ason syndrom e in clu des a rte r iovenousan eu rysm s of the bra in , racem ose ang iom as of the ips i-la te ral ret ina , and assoc ia ted cu taneous v ascu la r n ev i.

T he pho to grap h of the righ t fu ndu s in the ir a r ticle ,alth ough ro tated 90 degrees coun te rc lockw ise , rep re sen tsthe sig na l fm din g of the W ybu rn -M ason syndrom e, aracem o se ang iom a of th e re tina . T h is les ion show s aninc reased num ber and size of seem ing ly fu lly dev elo ped

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7 5 4 P E D IA T R IC S Vo l. 67 No . 5 M ay 1981

tortuous and w idened retinal ves se ls w ith probable ate-riovenous comm unications. In co ntrast the S tu rg e-W ebersyndrom e dem onstrates cavernous hem angiom as of thec h o r o i d w ithout retinovascular involvem ent w ith the ex-c e p t i o n of m ildly dilated otherw ise norm al retinal veins.5

The angiogram illustrated in their article show s ap r o b a b l y unilateral arteriovenous aneurysm of the cere-bral vessels, consistent w ith the W yburn-M ason syn-drom e. Intracranially the Sturge-W eber syndrom e in-c ludes leptom enigeal angiom atosis usually covering theoccipital lobe and plane x-ray show s a gyriform patterno f c ortica l c alc ifica tio ns deep to the vascular m alform a-tion of the m e ninges.6

C utaneous vascular nevi are seen in both the Sturge-W eber and the W yburn-M ason syndrom es. W e w ouldlike to em phasize that the association of cutaneous vas-c u l a r nevi, ocular vascular abnorm alities, and cerebralvascular m alform ations are seen in clinical syndrom eso t h e r than Sturge-W eber. These syndrom es include W y-burn-M ason,2 V on H ippel-L andau,7 G ass,8 and O sler-W e-ber-R endu.9 C areful attention to the clinical presentationand fundoscopic findings perm it accurate differentiationof these entities. The variable prognosis, therapy, andgenetic counseling associated w ith these syndrom es m an-date accurate differentiation.

REFERENCES

LOUI S I. SOBEL , M SJEFFREY G . ODEL , M DALAN H . FRI EDMAN , M DD e partm ent of O p hthalm ologyM ount Sinai M edical C enter100th St and 5th A veN ew Y ork, N Y 10029

1. R ussell JD , W ise PH , R ischbieth H G: V ascular m alform a-tion of hypothalm us: A cause of isolated grow th horm onedeficiency. Ped ia trics 66:306, 1980

2. W yburn-M ason R : A rteriovenous aneurysm of m id-brainand retinal, facial naem i and m ental changes. Bra in 66:163,1943

3. W alsh FB , H oyt W F: C lin ic al N eu ro -o ph th alm olo gy , ed 3.B altim ore, W illiam s & W ilkins C o, 1969, pp 1693-1694

4. L ecuire J, D echaum e JP, B ret P: B onnet-D echaum e-B lancsyndrom e, in V inken PJ, B ruyn G W (eds): H andbo ok o fC lin ica l N eu ro logy : T he P ha kom ato se s. N ew Y ork, A m er-ican E lsevier Publishing C o, 1972, vol 14, pp 260-266

5. Susac JO , Sm ith JL, Scelfo R J: The tom ato catsup fundusin Sturge W eber syndrom e. Arch O ph tha lm ol 92:69, 1974

6. D e R econdo J, H aguenau M : N europathologic survey of thephacom atoses and allied disorders, in V inken PJ, B ruyn G W(eds): Handboo k o fC lin ica lN euro lo gy: Th e P hakom ato ses .N ew Y ork, A m erican Elsevier Publishing C o, 1972, vol 14,pp 63-65

7. G rossm an M , M elm on K L: V on H ippel-Landau disease, inVinken PJ, B ruyn G W (eds): H andbo ok o fC lin ic a l N eu ro l .og y : T he P hakom ato ses . N ew Y ork, A m erican E lsevier Pub-lishing C o, vol 14, pp 241-259

8. G aas JD : C avernous hem angiom a of the retina, a neuro-oculo-cutaneous syndrom e. Am J O phth a lm ol 71:799, 1971

9. Lieboldt G P, Leiber B : C utaneous dysplasias associated w ithneurological disorders synopsis and differential diagnosis, inV inken PJ, B ruyn G W (eds): H andbo ok o fC lin ic a l N euro l-og y: Th e Ph akom atoses. N ew Y ork, A m erican E lsevier Pub-lishing C o, 1972, vol 14, p 120

D ie t a r y C h lo r id e D e fic ie n c yTo the E d ito r.-

W ith regard to the article entitled The D ietary C hlo-ride D eficiency Syndrom e (Ped ia trics 66:366, 1980), ifthe infants m entioned had been cared for by pediatriciansw ho believe in early feeding of infants, the problem ofdietary ch lo r ide deficiency w ould not have occurred. Inaddition to their form ula the infants w ould be gettingother foods and wou ld be unlikely to develop such ad e f i c i e n c y .

H aving lived through the problem s of diarrhea due toovers ter i iza t ion and breaking dow n of sugars in the for-m ula, seizures in infants due to pynidoxine deficiency inthe form ula, and now chloride deficiency, it still seem s tom e that w hen the child is finishing off about 4 oz perfeeding, solid food can be introduced. This assures thechild of adequate caloric intake, helps him s l e e p throughthe night, and ensures that the child w ill get an adequatediet in spite of any goof that m ight occur as a result ofm anufacturing deficiencies in the form ula. Furtherm ore,w hen the infant is young, it is easier to approach m om toput daddy on a decent diet too.

H aving fought m ilk anem ia for 30 years, it is reallydisturbing for an old-tim er like m e to hear those young-sters in pediatrics insist the babies m ust have only for-mu la for six m o n t h s . I think here is a good argum ent fore a r l y feeding of other foods.

In R ep ly .-

PATRIC IA BLACKM AN , M DD ivision of M aternal and C hild H ealthTexas D epartm ent of H ealth1100 W 49th StA u s t i n , T X 78756

W e w ant to respond to tw o of the issues raised in D rB lackm a ns letter. T he first is that the early in tro ductionof solid foods w ou ld have p reven ted the occurrence ofd ie ta ry c h lo rid e d e fic ie n c y in the infants ingesting chlo-ride-deficient N eo-M ull-Soy. The Table show s the age atw hich solid foods w ere introduced into the diet of 1 1 ofthe 12 infants described in our paper. Solid foods w ereintroduced into th e d ie ts o f se ven o f 1 1 of th e in fa n tsprior to 5 m onths of age. In eight instances solid foods

T A B L E . D ie t a r y Ch lo r ide D eficiency and Introductionof Solid Foods to Infant D ietPatient

N o.Age Neo-M ull-

S oy S tarted(m o)

Age SolidsIntroduced

(m o)

A ge at D iagnosis ofM etabolic A lkalo sis

(m o)1 1#{189}

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1981;67;753PediatricsLouis I. Sobel, Jeffrey G. Odel and Alan H. Friedman

On Angiomas of Retina, Brain, and Skin

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Online ISSN: 1098-4275.Copyright 1981 by the American Academy of Pediatrics. All rights reserved. Print ISSN: 0031-4005.American Academy of Pediatrics, 141 Northwest Point Boulevard, Elk Grove Village, Illinois, 60007.has been published continuously since 1948. PEDIATRICS is owned, published, and trademarked by thePEDIATRICS is the official journal of the American Academy of Pediatrics. A monthly publication, it

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