Archives of Disease in Childhood 1994; 70: 515-519

Turned head adducted hip truncal curvature

syndrome

Chiaki Hamanishi, Seisuke Tanaka

AbstractOne hundred and eight neonates andinfants who showed the clinical triad of ahead turned to one side, adduction con-tracture of the hip joint on the occipitalside of the turned head, and truncal cur-vature, which we named TAC syndrome,were studied. These cases included sevenwith congenital and five with late infantiledislocations of the hip joint and 14 whodeveloped muscular torticollis. Forty onewere among 7103 neonates examined byone of the authors. An epidemiologicalanalysis confirmed the aetiology of thesyndrome to be environmental. The sideto which the head was turned and that ofthe adducted hip contracture showed ahigh correlation with the side of thematernal spine on which the fetus hadbeen lying. TAC syndrome is an import-ant asymmetrical deformity that shouldbe kept in mind during neonatal examina-tion, and may be aetiologically related tothe unilateral dislocation of the hip joint,torticollis, and infintile scoliosis whichdevelop after a vertex presentation.(Arch Dis Child 1994; 70: 515-519)

Department ofOrthopaedics, KinkiUniversity School ofMedicine, Osaka,JapanChiaki HamanishiSeisuke Tanaka

Correspondence to:Dr Chiaki Hamanishi,Department of Orthopaedics,Kinki University School ofMedicine, Ohno-Higashi,Osaka-Sayama, Osaka 589,Japan.Accepted 6 January 1994

It has been postulated that abnormal intra-uterine pressure produces many congenitalpostural deformities. 1-5 Mau reported a

clinical combination of congenital deformitiesthat he referred to as the 'seven signssyndrome',6 and Lloyd-Roberts and Pilcherdescribed the 'moulded baby syndrome' con-

sisting of a tetrad of postural scoliosis, skullmoulding, rib cage moulding, and pelvicobliquity.7 The ipsilateral coexistence of dis-location of the hip joint with a palpable sterno-mastoid mass810 or with plagiocephaly'1 12

suggests a specific intrauterine asymmetricalposture of the fetus. Most of these reportedcases, however, were not in neonates but ratherin older infants, and several postnatal factors,such as the development of a sternomastoidtumour or persistently turned head, trunk andpelvis to one side, especially in the supine posi-tion could clearly have modified the clinicalsigns. We observed a group of neonates whoshowed a characteristic set of asymmetricalpostural deformities identified during theroutine newborn examination. Their threecommon signs were a strong tendency to turnthe head to one side, adduction contracture ofthe hip joint or restriction of hip abduction inflexion correlating with the occipital side of theturned head, and curvature of the trunk. Wetermed this combination of deformities theTAC syndrome, for turning, adduction, and

curvature. An epidemiological analysis wascarried out to determine whether the intra-uterine environment of these asymmetricallydeformed babies had been restricted. Theclinical course of each feature of the clinicaltriad was also analysed to determine whetherTAC syndrome is aetiologically related to anysubsequent paediatric disorders.

Patients and methodsWe studied a total of 108 cases with TACsyndrome. Of them, 41 were among a totalnumber of 7103 neonates personally examinedby one of the authors (CH) at newborn exami-nations conducted at hospitals in four citiessince 1981. Thirteen were referred neonates.The remaining 54 were among infants agedfrom 10 days to 3 months who were referred toone of the authors (CH) with suspected torti-collis, congenital dislocation of the hip joint,truncal curvature or leg length discrepancy.The mothers of the infants in the latter groupstated that they showed a strong tendency toturn the head to one side and that thistendency was apparent immediately afterbirth. The epidemiological survey for the 54neonates was conducted separately from thatfor the 54 infants. Control data were obtainedfrom the birth records of 518 normal livebabies examined by one of the authors (CH) in1982, in Moriyama City, Japan. The clinicalcourse of each ofthe triad of signs was followedup until the contractures and asymmetry dis-appeared, or for up to seven years in thosepatients with dislocation of the hip joint andscoliosis.

ResultsAll 108 patients showed the clinical triaddescribed above (fig 1). Statistical data for theneonates, infants, and all patients are shownin table 1. The neonatal incidence was5-8/1000 live births (41/7103). There was asignificant preponderance of girls, first bornchildren (neonates and total), those born inthe winter months (neonatal and total), thoseborn with lower birth weight (infants andtotal), those whose mothers had smallabdominal circumference (neonates andtotal), and those whose mothers had their firstchild at higher than the average age (neonatesand total). One hundred and two babies wereborn by vertex presentation, and the remain-ing six by breech presentation. In three of thelatter, the mother had a uterus bicornis.Changes in the presentation or position of thefetus in the uterus during the last 10 weeks ofgestation were noted by examining the weekly

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Figure I Clinical appearance of neonates with TAC syndrome. These three babies are turning their heads to the rightside, and showing adduction contracture of the left hip on the occipital side. The hip joint on the right (facial) side ismarkedly abducted.

Table I The epidemiologicalfindings for the patients with TAC syndrome

N'eonatal Infanitile Total Controls(ni = 54) (=54) (= 1 08) (n = 5318)

Female/male 41/13** 38/16** 79/29*** 267/251First born/others 36/9*** 25/25 61/34*** 201/317Winter/summer births 34/20* 30/24 64/44* 248/270Birth weight (g)t 3028 (409) 2897 (448)** 2953 (433)** 3121 (390)Mother's abdominal circumference (cm)t 88-1 (2-7)** 89-3 (4 5) 88-8 (4 0)* 90 3 (5-4)Maternal age of first born (years)t 28-2 (4U0)** 25 4 (2 4) 27-1 (3 7)* s5-8 (3 0)Change in fetal position (times/1O weeks)t# 1-04 (12)*** 0-69 (1 0)*** 0-78 (1-07)*** 2-33 (1 69)

*p<0.05, **p<0-01, ***p<0.001. tMean (SD); tDuring the last 10 weeks of pregnancv.

obstetric outpatient records. Tchange of position in utero xwlower than that in the normalthe fetus' back was on, for ex

side of the mother, that is, thefetus in vertex presentationmother's spine (fig 2), the addture of the hip joint and mus

were almost always on the patSimilarly, when the fetus' bamaternal right side, these sigialways on the right; those co

highly significant (table 2). TIprematurity, postmaturity, oland twin births did not difffrom that in the control grcsurvey was conducted on 62the 39 siblings, 17 (440 o) ha(

Abducted right hipAdducted left hip/ Pes ca

Front axis I.oof the fetus

he frequency of deformities, consisting of four with torticollis,{as significantly three with acetabular dysplasias, two withfetuses. When congenital dislocation of the hip, one with

cample, the left adduction contracture of the hip joint, oneleft side of the with scoliosis, and six with other asymmetricalrested on the postural deformities. The incidence of con-

uction contrac- genital dislocated hips in the siblings (5.3%)cular torticollis was greater than that of the general populationtient's left side. in Japan (02-030 %).13 Only four among,ck was on the 124 parents had asymmetrical deformities:ns were almost torticollis, congenital dislocation of the hip,)rrelations were acetabular dysplasia, and facial asymmetry.he incidence of There are obvious difficulties in ascertainingigohydramnios, whether these transient deformities were'er significantly present in older relatives (grandparents,)up. A familial aunts, and uncles), and no instances ofprobands. Of asymmetrical postural deformities were found

d asymmetrical in second degree relatives. Twelve (3-8%)of 318 first cousins had asymmetrical deform-ities (four with congenital dislocation ofthe hip, four with acetabular dysplasias,

Icaneovalgus two with torticollis, one each with scoliosisForced turning and cleft lip), but the incidence was not

of the head

to the right significantly different from that for the generalpopulation.

Left sidedplagiocephaly anddeformed face

Figure 2 Sketch from a perspective inferior to the mother'sspine of a child with TAC syndrome lying on the spine inleft occipital position. The head is forced to the right and theleft hip is adductedfronm the front axis line of the body.

Table 2 Relationship of the side of the torticollis andadducted hip contracture with fetal presentation

Muscular torticollis Adducted contracture

Presentation Left neck Right neck Left hip Right hip

Left occipital* 7 0 41 10Right occipital 0 7 6 29

p<0 005 p<0-001

*Left occipital position: the back of the fetus is located on theleft side of the mother and the left hip and left side of the neckof the fetus are situated on the mother's spine in vertexpresentation.

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Turned head - adducted hip - truncal curvature syndrome

Figure 3 Occipital curve. Left:- a view of the back showing thoracolumbar curvature convex to the occipital side. Centre:the black arrow indicates the facial side of the turned head. A spinal curvature convex to the occipital side is observed evenwhen the skull is held in neutral rotation by the radiologist. Facial deformity, flatness on the facial side, is seen. The whitearrow indicates the wider obturatorforamen on the occipital side due to pelvic tilt. Right: a magnified view ofpelvisindicating asymmetry of the obturator foramen.

CLINICAL COURSE FOR EACH SIGN IN THETRIADThe terms occipital side and facial side as usedherein refer to the side of the patient's bodycorresponding to, respectively, the occipitaland facial side of the head when in the rotatedposition.

1. Tendency to turn the head to one sideRotation of the head to the opposite side with-out any resistance could be performed in 85patients, although it seemed to cause discom-fort to the babies, who immediately revertedthe head to the original position on release. Inthe other 23 patients, contracture and someresistance were felt when the head waspassively turned to the opposite side. A palp-able mass in the sternocleidomastoid muscledeveloped in 14 of these 23 patients within thefirst few weeks on the expected side, that is, theleft if the fetus' left side rested on the mother'sspine (table 2). All were successfully treated bysingle gentle full rotation of the head to theoccipital side (manual myotomy) and byplacing them in prone position when theywere laid down. In one patient delivered bycaesarean section, the rotated head positionand contracture persisted for one month,although a palpable mass did not develop.

2. (A) Adduction of the hip joints on the occipitalsideAll of the 108 patients showed adduction con-tracture of the hip on the occipital side of theturned head, which was defined as restrictionof hip abduction in flexion compared withthe angle of abduction on the facial side.

Radiographs usually demonstrated pelvicrotation toward the facial side, which wasdetected by increased transverse diameter ofthe obturator foramen on the occipital side(fig 3, right). In 81 patients, this limitation inabduction disappeared either spontaneouslyor by placing the patients in the proneposition. Barlow's test was positive in twoneonates and in five referred infants. In fourneonates, Barlow's test was negative at thenewborn examination but became positivewithin one to three months. In 16 patientsunilateral acetabular dysplasia was demon-strated radiographically at 2 to 3 months ofage. In one referred infant, the hip was stablewas a nxegative Barlow's test at the first visitbut was dislocated with positive Barlow's testand Ortolani's sign two months later. All ofthese 12 patients (two with congenital, fivewith suspected congenital, and five with lateinfantile dislocation) were successfully treatedby placing the neonates in a prone position orby using the Pavlik harness for infants. At 4years of age, four patients with previouslydislocated hip showed unilateral acetabulardysplasia.

2. (B) Abduction contracture of the hip joint onthe facial side of the turned headIn 51 patients, the hip joint on the facial sidewas contracted in the abducted position,which was defined by tilting the gluteal fissureto the facial side in the prone position onbringing both legs together (fig 4, middle).Five required manual stretching of the con-tracture in order to correct persistent pelvictilt and resulting lumbar scoliosis as describedbelow.

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Hamanishi, Tanaka

Figure 4 Abduction contracture of the hip joint observed in the patients with TAC syndrome. Left: the range of abductionof both hip joints. The arrow indicates the facial side of the turned head. Centre: the pelvis and glutealfissure tilt to thefacial side when the knees are brought together, gzving the appearance of a leg length discrepancy. Right: the radiographshows the spinal curvature convex to the facial side (facial curve) due to abduction contracture of the right hip withresulting pelvic tilt.

3. Truncal curvatureWe observed three types of truncal c

(A) All 54 neonatal cases showedtruncal curve which was convex irto the occipital side of the turnThis type of curvature was alsoradiographically in 10 infantil(occipital curve) (fig 3, left, cenicurve in most cases disappeared q

ously within the first year, but in fivpersisted for 18 months and in oneyears.

(B) In 18 infantile cases, acontracture of the hip joint on the fcaused a pelvic tilt to the facial sboth legs were brought together, anpostural scoliosis with the conveto the facial side ensued (facial ci4, right). In four patients (22%)was still present when the child swalk, and in one it persisted untilage.

(C) A double curve (thoracic occlumbar facial) was observed radiograr18 infants, in two of whom it was stiat 3 years of age.

Figure 5 The arrows indicate the facial side of the turned head. Severe (left) a(right) pes calcaneovalgus of the right foot on the facial side is shown.

curvature.a natural

i relationred head.observedle casestre). Thespontane-'e (8%) itfor three

ibductionFacial side

OTHER CLINICAL MANIFESTATIONSWe observed pes calcaneovalgus exclusively onthe facial side in 12 babies; it was probably dueto moulding by the uterine wall (fig 5).Forefoot adduction or supination exclusivelyon the occipital side was seen in four patients.Skull deformities were slight in the newbornperiod but were progressively aggravated if thehead turning persisted and the patients wereplaced in the supine position. Facial asym-metry (straight on the facial side and round onthe occipital side) was seen in 36 patients.

ide when DiscussionId lumbar It has been reported that 15-20% of patientsxity also with torticollis also show dysplasia or disloca-urve) (fig tion of the hip joint,8 9 and that 8-10% of thosethe curve with congenital dislocation of the hip show,tarted to torticollis,8 10 mainly ipsilaterally. It has been3 years of speculated that the aetiology of this ipsilateral

coexistence and also those with plagio-ipital and cephaly1 is intrauterine or partly postnatal.phically in The subjects of these reports, however, wereill present infants, and neither analyses of their neonatal

condition or presentation of the fetus in uteronor comprehensive epidemiological surveyswere carried out. Although congenital disloca-tion of the hip and torticollis are highly associ-ated with breech presentation,3-5 14 1565-90% of the babies with these conditionswere delivered by a normal vertex presenta-tion.9 12 16 The aetiological factors andmechanisms of these deformations in patientsdelivered by a normal vertex presentation havenot yet been fully explained. Dunn demon-

q strated that the leg resting on the mother'sspine was more likely to be adducted and to bedislocated.4 Our present epidemiologicalresults suggest that TAC syndrome in a childdelivered by vertex presentation is almostinvariably associated with a tight uterus, andthat both unilateral congenital dislocation of

md milder the hip and torticollis develop on the side ofthe fetus lying on the mother's spine (fig 2).

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Turned head - adducted hip - truncal curvature syndrome

Conversely, the contralateral hip joint lyinganteriorly showed the abduction contracture.We usually advised parents of babies withTAC syndrome to place them in the prone

position, but some parents were reluctant to doso, and the asymmetrical deformities pro-

gressed. The five patients with late dislocationsof the hip joint were among the latter, whichsuggests that persistent adducted contracturemay inhibit the acetabular development,displace the femoral head laterally, and cause

late infantile dislocation. The combination ofadduction and abduction contractures ofthe hip joints in the same patient has beennoted.17 18 Postural scoliosis due to pelvic tiltcaused by the abducted hip joints has beendescribed by Tachdjian.19 The clinical courseof this type of curvature, however, has not beenfully delineated. Although the natural 'occi-pital' curves in most patients resolved within a

few weeks, as did the neck contracture, the'facial' lumbar curves and the double curves

associated with abduction contracture of thehip joint persisted longer, even until after thestart of walking. A persistently turned fetalhead towards the anterior wall of the uteruscould well cause shortening,1' disturbedvenous circulation,20 and contracture of thesternocleidomastoid muscle on the side ofmother's spine or on the fetal occipital side.We have also observed this contracture in a

baby delivered by caesarean section. A palp-able mass was found in 14 patients with TACsyndrome, eight of whom were among the7103 examined newborns (incidence, 0 1%).A shortened and degenerated sternal branch inthe sternocleidomastoid muscles would bevulnerable to stretching in the second internalrotation during vertex delivery, in which thehead is rotated forcibly towards the maternalsacrum. Aggressive repair of these damagedmuscles by granulomatous tissue would leadto the formation of a palpable mass. Theassociated findings of a high proportion offirst born children, a greater proportion ofbirths during the winter months, and highermaternal age suggest an increase in the tone ofboth the abdominal and uterine wall,35 whichwould restrict movement of the fetus. The

high incidence of several asymmetricaldeformities in the siblings of index patientsalso suggests the influence of commonintrauterine or intra-abdominal factors. Of the7103 newborns, two developed congenitaldislocation of the hip and four late infantiledislocation. Such late infantile dislocationswould have been considered previously to becongenital dislocation which had been

overlooked. Of the 7103 newborns, apart fromthe patients with TAC syndrome, six hadother congenital dislocation of the hip, whichwas mostly bilateral, and had a teratological,generally hypotonic or strong genetic back-ground. The incidence of TAC syndrome in7103 live births was 0-58%, and that ofunilateral dislocation of the hip joint as a con-genital postural deformity was 0-09°/O (twocongenital plus four late dislocations in 7103),which represents half of the total cases ofcongenital dislocation of the hip in ourneonates. This incidence is a third to half ofthat of congenital dislocation of the hip inJapan at present (0-2-03%). Improvement inmaternal welfare and prenatal and postnataleducation may lessen the incidence of TACsyndrome and resultant postural deformitiesstill further.

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2 Chapple CC, Davidson DT. Study of relationship betweenfetal position and certain congenital deformities. J Pediatr1941; 18: 493-93.

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13 Ishida K. Prevention of the development of the typicaldislocation of the hip. Clin Orthop 1977; 126: 167-9.

14 Wilkinson JA. A post-natal survey for congenitaldisplacement of the hip. J Bone Joint Surg Br 1972; 54B:40-9.

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