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In light of the growing number of surviving children born very preterm, there is an increasing focus on their long-term outcomes in terms of growth, metabolic status, and neurocognitive development. Therefore, it is of importance to follow such children from birth onwards with the aim of identifying the causes of atypical development, developing preventative measures, and improving outcomes. Since such long-term follow-up needs to be conducted with the least possible burden, clinical investigations such as anthropometry and neurocognitive tests, if conducted rigorously, will continue to have a predominant role. The aim of this review is to discuss the complexity of longitudinal anthropometry in children born very preterm and to provide an overview of the main studies that have examined associations between growth, in particular head growth, and neurocognitive outcomes at around school age.

Background: Head circumference (HC) trajectories are held to reflect neurological development and the acquirement of intelligence. It is important to assess HC growth accurately because atypical HC growth is an indicator of various developmental disorders. HC growth is determined by both familial and physical factors but, hitherto, no one has considered both factors together. The aim of the present study was thus to investigate the relationship between HC, physical growth, and parental HC. Methods: The study group in the Japan Children's Study consisted of 192 healthy full-term Japanese children. HC (maximum occiptofrontal circumference), height and bodyweight were measured at the ages of 4, 9 and 18 months. Multiple regression analysis were conducted predicting the HC from the body measurements and mid-parental HC (defined as the average of standardized paternal and maternal HC). Results: Adjusted multiple R2 were 0.336, 0.307 and 0.259, measured at the aforementioned three stages. Bodyweight and mid-parental HC predicted the HC on each occasion (P < 0.01). Bodyweight was more relevant than mid-parental HC. Conclusions: HC growth is influenced by physical growth and parental HC; therefore, it is important to consider both physical and familial factors. A formula is herein proposed to assess HC using bodyweight and mid-parental HC.

The evolutionary origin of the long neck of giraffes is enigmatic. One theory (the ‘sexual selection’ theory) is that their shape evolved because males use their necks and heads to achieve sexual dominance. Support for this theory would be that males invest more in neck and head growth than do females. We have investigated this hypothesis in 17 male and 21 female giraffes with body masses ranging from juvenile to mature animals, by measuring head mass, neck mass, neck and leg length and the neck length to leg length ratio. We found no significant differences in any of these dimensions between males and females of the same mass, although mature males, whose body mass is significantly (50%) greater than that of mature females, do have significantly heavier (but not longer) necks and heavier heads than mature females. We conclude that morphological differences between males and females are minimal, that differences that do exist can be accounted for by the larger final mass of males and that sexual selection is not the origin of a long neck in giraffes.

The introduction of effective ventriculo-vascular shunt procedures has caused an undoubted improvement in the prognosis of infantile hydrocephalus (Rickham and Roberts 1960, Lorber 1961, Lawrence 1963, Yashon 1963, 1964, Macnab 1964). Nevertheless comparison between various published results is often impossible, and in this review of the experience of one surgical unit using only the Holter valve, we have tried to evaluate the control of head growth and the liability to revision operations in ways that are objective and could perhaps be used by others when reporting their results. SUMMARY 152 consecutive cases of infantile hydrocephalus treated in one unit with Holter valve ventriculo-atrial shunt are reviewed. 109 were associated with spina bifida or cranium bifidum. 73-5 per cent of those associated with spina bifida were alive at final follow-up (minimum period 1 year). 81-5 per cent of the others were alive at final follow-up. 15-8 per cent of all cases and 7 per cent of all operations became infected. The frequency of revision operations is discussed, and an attempt made to present the incidence and the resulting control of head circumference in relation to the length of follow-up. Head circumference is divided into normal, acceptable and unsatisfactory. 95 per cent of cases which were associated with spina bifida in this review were controlled in the normal or acceptable range. 76 per cent of the other cases were in this range at final follow up. RESUME Traitement de l'hydrocephalic infantile par la valve cle Holler: une analyse cie 152 cas conseculifs Les auteurs revisent 152 cas consecutifs d'hydrocephalie infantile traites dans le meme service avec la valve de Holter comme derivation ventriculo-atriale. 109 etaient associes de spina bifida ou cranium bifidum. 73,5 pour cent des cas associes avec spina bifida etaient encore en vie a la derniere visite (periode minimum un an). 81,5 pour cent des autres cas etaient vivants a la derniere visite. 15,8 pour cent de tous les cas et 7 pour cent de toutes les operations se sont infectes. La frequence des operations de revision est discutee et on essaie de presenter l'incidence et le controle en resultant de la circonference de la tete en relation a la longueur de la periode d'observation. La circonference de la tete est classee comme normale, acceptable, non-satisfaisante. 95 pour cent des cas associes avec spina bifida dans cette etude ont ete classifyes dans la serie normale ou acceptable. 76 pour cent des autres cas se rangeaient dans cette classe a la derniere visite. ZUSAMMENFASSUNG Behanclhtng von infaiuilem hydrocephalus mit der Anwendimg der Holter-Klappe: eine Analyse von 152 aufe inanderfolgenden Fallen Es werden 152 aufeinanderfolgende Falle von infantilem hydrocephalus besprochen, die in einer Einheit mit ventriculoatrialer Abzweigungsoperation mit der Holter-Klappe behandelt wurden. 109 traten im Zusammenhang mit spina bifida oder cranium bifidum auf. 73,5 Prozent der mit spina bifida assoziierten waren am Leben bei der endgultigen Nachprufung (Minimum-Periode 1 Jahr). 81,5 Prozent der anderen waren bei der endgultigen Nachprufung am Leben. 15,8 Prozent aller Falle und 7 Prozent alter Operationen bekamen eine Infektion. Die Haufigkeit der Revisions-Operationen wird erortert, und es wird versucht, die HAufigkeit und die resultierende Kontrolle des Kopfumfanges in ein Verhaltnis zu der Dauer der Nachprufung zu stellen. SUMMARY Evidence of pulmonary embolisation was observed in 17 of 31 cases of ventriculo-atrial shunt coming to autopsy. Eleven had bland, and 5 septic emboli, while in one case both were present. These findings in a larger series than any previously published fully confirm earlier observations. The surgical implications of these results are discussed. RESUME Embolisation pulmonaire survenue a la suite de derivations atrio-ventriculaires pratiquEes dans le traitement de l'hydrocephalie Des preuves d'embolisation pulmonaire ont ete observees dans 17 des 31 cas de derivation atrio-ventriculaire parvenus a l'autopsie; 11 avaient des embolies non-septiques et 5 des embolies septiques, tandis que dans un cas les deux types etaient presents. Les resultats de cette etude, plus etendue que toutes celles publicees jusqu'a present, confirment pleinement les observations precedentes. Les implications chirurgicales de ces resultats sont discutees. ZUSAMMENFASSUNG Pulmonare Embolisierung als Komplikation ventriculo-atrialer Seitenanknupfungen bei Wasserkopfoperationen Anzeichen von pulmonarer Embolisierung wurden bei der Autopsie von 17 aus 31 Fallen ventriculo-atrialer Seitenanknupfung festgestellt; 11 hatten milde und 5 septische Embolie, wahrend bei 1 Fall beide auftraten. Diese Befunde, in einer grosseren Serie als irgendeine bisher veroffentlichte, bestatigen vollauf die fruheren Beobachtungen. Die chirurgischen Implikationen dieser Ergebnisse werden erortert. RESUMEN Embolia pulmonar coma complication deuna derivation venlriculo-auricular realizada a causa de hidrocefalia Entre 31 casos de derivacion ventriculo-cardiaca en los cuales se hicieron autopsias, sc encontraron en 17 evidencia de embolia pulmonar. 11 tenian embolos blandos, 5 tenian embolos septicos, y en un caso se encontraron ambos. Estos resultados en una serie mas grande que todas las que se han publicado hasta ahora confirman completamente las observaciones que ya se han hecho. Se discute la importancia quirurgica de los resultados.

SUMMARY The early detection of progressive hydrocephalus in pre-term infants often is a difficult diagnostic problem. In this study, 43 pre-term infants with birthweights between 1000 and 2000g were examined shortly after birth and an estimate of gestational age was made. Individual measurements of head circumference were made weekly for the first month of life and regularly thereafter up to 16 weeks of age. The results indicate that healthy pre-term infants regularly cross percentiles for head circumference in the first months of life, the most rapid rates of growth being observed in the smallest babies. Only infants with significant medical problems in the neonatal period conform to or fall below the expected patterns of growth. A velocity curve for pre-term infants, according to health and gestation, is presented. RESUME Etude de la croissance cephalique longitudinale chez le nourrisson premature. lere partie: taux normaux de la croissance de la tete La detection precoce d'une hydrocephalic progressive chez le nourrisson premature est souvent un probleme diagnostic difficile. Dans cette etude 43 nourrissons prematures (poids de naissance entre 1000 et 2000g) ont ete examines peu apres la naissance et il a ete fait une estimation de 1'âge de gestation. Des mesures individuelles du tour de tete ont ete faites, hebdomadaires durant le premier mois de la vie et mensuelles ensuite. Les donnees recueillies indiquent que les nourrissons prematures ‘en bonne sante’ croisent les lignes de percentile du tour de tete dans les premiers mois de la vie, les taux de croissance les plus rapides s'observant chez les nourrissons les plus petits; seuls les nourrissons ayant presente des problemes medicaux durant la periode neo-natale se conforment a l'allure prevue de croissance ou tombent en dessous. Une courbe de vitesse pour nourrissons prematures selon leur etat de sante et l'âge de gestation est presentee. ZUSAMMENFASSUNG Langzeit-Kopfwachstumsstudien bei Fruhgeborenen I. Teil: Normalwerte fur das Sehadelwaehstum Die fruhzeitige Entdeckung des wachsenden Hydrocephalus beim Fruhgeborenen ist oft ein schwieriges diagnostisches Problem. Bei dieser Untersuchung wurden 43 Fruhgeborene (Geburtsgewicht 1000 bis 2000g) kurz nach der Geburt untersucht und eine Beurteilung des Gestationsalters vorgenommen. Einzelne Kopfumfangsmessungen wurden wahrend des ersten Monats wochentlich und sparer monatlich vorgenommen. Die Daten zeigen, das das ‘gesunde’ Fruhgeborene regular die Percentilen des Kopfum-fanges im ersten Monat kreuzt—die schnellsten Wachstumsraten wurden bei den kleinsten Kindern beobachtet—und nur die Kinder, die wahrend der Neonatal-periode schwer-wiegende medizinische Probleme hatten, folgen den erwarteten Wachstumsmustern oder bleiben hinter ihnen zuruck. Eine Geschwindigkeitskurve (des Wachstums) fur Fruhgebprene, bezogen auf Gesund-heitszustand und Gestationsalter, wird angegeben. RESUMEN Estudios longitudinales del crecimiento de la cabeza en el lactante de pre-termino. la parte: grados normales de crecimiento craneano. La detection precoz de una hidrocefalia progresiva en un lactante de pre-termino es a menudo un problema de diagnostico dificil. En este estudio 43 lactantes pre-teminor (peso del nacimiento de 1000 a 2000g) fueron examinados poco despues del nacimiento y se hizo una estimation de la edad gestacional. Se realizaron mediciones individuales de la circunferencia craneana cada semana durante el primer mes de vida y luego cada mes. Los datos indican que los lactantes pre-termino ‘sanos’ cruzan regularmente los percentiles para la circunferencia de la cabeza en el primer mes de vida (habiendose observado las velocidades de crecimiento mas rapidas en los ninos mas pequenos) y que solamente aquellos ninos que han tenido problemas medicos significativos en el periodo neonatal son los que siguen o quedan por debajo de Ies esquemas esperados de crecimiento. Se presenta una curva de velocidad para lactantes pretermino de acuerdo con la salud y la edad gestacional.

SUMMARY The authors examined the relationship between disproportionate intra-uterine head growth, or a relatively small head, and later development, using data collected as part of the Collaborative Perinatal Project of the National Institute of Neurological and Communicative Disorders and Stroke. Within the population of infants who are normal by conventional criteria (term infants with appropriate weight and head-circumference for gestational age), a group with relatively small heads was defined by each of four different methods. Developmental outcome measures included the Bayley Mental and Motor Scales at eight months, Stanford-Binet IQ at four years and Wechsler Intelligence Scales IQ at seven years. No clinically meaningful differences in developmental outcome were found between the infants with relatively small heads and the remainder of the infants. RESUME Croissance intra-uterine disproportionnee de la tete el devenir du developpement Les auteurs ont examine la relation existant entre une croissance intra-uterine disproportionnee de la tete ou une tete relativement petite, et le developpement ulterieur, utilisant les donnees recueillies au sein du Collaborative Perinatal Project du National Institute of Neurological and Communicative Disorders and Stroke. Dans la population de nourrissons normaux selon les criteres conventionnels (nourrissons nes a terme avec poids en rapport et tour de tete correspondant a l'âge conceptuel), un groupe ayant de relativement petites tetes a ete defini par quatre differentes methodes. Les mesures du devenir du developpement ont inclus les echelles mentales et motrices de Bayley a huit mois, le QI au Stanford-Binet a quatre ans et le QI a l'echelle d'intelligence de Wechsler a sept ans. Aucune difference cliniquement significative dans le devenir developmental n'a ete trouvee entre les nourrissons avec relativement petite tete et les autres nourrissons. ZUSAMMENFASSUNG Unproportionierles Kopfwachstum in utero und spatere Entwicklung Anhand von Daten, die als Teil einer Collaborativen Perinatalen Studie des National Institute of Neurological and Communicative Disorders and Stroke gesammelt worden waren, haben die Autoren die Beziehung zwischen unproportioniertem Kopfwachstum in utero, oder einem relativ kleinen Kopf, und der spateren Entwicklung untersucht. Aus der Population von Sauglingen, die nach den ublichen Kriterien als normal gelten (zum Termin geboren, Gewicht und Kopfumfang dem Gestationsalter entsprechend), wurde durch vier verschiedene Methoden eine Gruppe von Kindern herausgefunden, die einen relativ kleinen Kopf hatten. Als Parameter fur die spatere Entwicklung wurden die Bayley Mental and Motor Scales im Alter von acht Monaten, der Stanford-Binet IQ mit vier Jahren und der Wechsler Intelligence Scales IQ mit sieben Jahren zugrunde gelegt. In der spateren Entwicklung fand sich zwischen den Sauglingen mit relativ kleinem Kopf und den ubrigen Sauglingen kein klinisch relevanter Unterschied. RESUMEN Crecimiento intrauterino desproporcionado de la cabeza y curso posterior del desarrollo Los autores examinan la relacion entre el crecimiemto desproporcionado intrauterino de la cabeza o una cabeza relativamente pequena y el desarrollo posterior como parte del Proyecto Colaborativo Perinatal del Instituto Nacional de Alteraciones de la Comunicacion y el Ictus. Dentro de la poblacion de lactantes que son normales segun los criterios convencionales (lactantes a termino con peso apropiado y perimetro craneal de acuerdo con la edad gestacional) un grupo con cabezas relativamente pequenas fue definido segun cuatro diferentes metodos. El curso posterior del desarrollo incluye las mediciones con las Escalas Bayley Mental y Motora a los seis meses, el CI con el Stanford-Binet a los cuatro anos y el CI con las escalas de inteligencia de Wechsler a los siete anos. No se hallaron diferencias clinicas significativas en el curso del desarrollo entre los lactantes con cabezas relativamente pequefias y el resto de los ninos.

It is estimated that 20 to 35 per cent of idiopathic microcephaly is hereditary. Common practice dictates that after the birth of such a case, subsequent pregnancies should be monitored ultrasonographically in order to facilitate early antenatal diagnosis, and thus genetic counselling. Two cases are reported to indicate the difficulties encountered. In case 1 head growth appeared to be normal until the 20th week of gestation and then slowed down to 31 cm at birth. In case 2 head growth proceeded normally until the 28th week of gestation; at birth it was 32 cm and over the succeeding months became markedly microcephalic. The accuracy of various ultrasonographic techniques is reviewed, and the limitations are discussed. In the light of these findings it is concluded that reliable prenatal diagnosis of hereditary microcephaly is not available as yet.

The objective of this investigation was the development of a modified Neonatal Growth Assessment Score (mNGAS) for use in the evaluation of neonatal growth status. The growth of 74 fetuses at risk for intrauterine growth retardation (IUGR) was followed longitudinally, beginning in the first or early second trimester. Rossavik growth models derived from data obtained in the second trimester were used to predict the weight (WT), crown-heel length (CHL) and head (HC), abdominal (AC) and thigh circumferences (ThC) at birth, which were then actually measured within 24 h after delivery. These measurements were compared to age-specific size curves and used to calculate sets of five growth potential index (GPRIi) values, which in turn were used to calculate five-variable Neonatal Growth Assessment Scores (NGAS5). Neonates were initially classified as normal or IUGR on the basis of NGAS5, GPRIi, and anatomic measurements. A final classification based on principal component analysis and linear discriminant analysis was carried out. The score obtained using the first principal component function was defined to be mNGAS51. The effectiveness of mNGASij values, determined from 1-4 GPRIi values, in separating normal and IUGR neonates was also evaluated. Neonates initially considered to be normal had very few abnormal GPRI values or anatomic measurements, whereas the frequency of these abnormalities in IUGR neonates was significantly increased. However, no single anatomic variable was 100% normal in the normal neonates and 100% abnormal in IUGR neonates. Only 40% of IUGR neonates were small for gestational age. Classification of these neonates using principal component analysis and linear discriminant analysis was essentially the same (98.6%) as that made initially after reclassification of two IUGR neonates as normal. The characteristics of the initial and final normal and IUGR groups were very similar and the mNGAS51 was strongly correlated with the NGAS5 in the IUGR group. The effectiveness of mNGASij in separating normal and IUGR neonates increased with the number of GPRIi values included and the types used. GPRIThC and GPRIWT were the most important, followed by GPRIAC. GPRICHL and GPRIHC were much less important and in some cases detrimental. These findings support the concepts of a decrease in soft tissue mass as the initial step in the development of IUGR and the protection of head growth (brain-sparing). The characteristics of mNGAS51, particularly its comprehensiveness, its independence of differences in growth potential, its weighting of GPRIi values according to their importance in the detection of IUGR and its ability to detect different manifestations of IUGR in different individuals, indicate that this should be a most effective parameter for separating normal and IUGR neonates.

Head circumference is considered an important parameter of brain growth and development. Syndrome-specific standards for head circumference in Williams-Beuren syndrome (WBS) are not available to date, although mental retardation is a leading manifestation in the syndrome. Therefore, we investigated head growth in 63 girls (251 measurements) and 88 boys (298 measurements) with WBS between birth and adulthood. Most measurements in both sexes were from the first 4 years of life (n = 162 in girls and n = 189 in boys). Mean (+/- SD) head circumference at birth was 33.39 +/- 1.38 cm and 34.02 +/- 1.44 cm for term girls and boys, respectively. Although head growth in WBS girls and boys was at a slower velocity, the pattern of head circumference was similar to that in the normal population. After the age of 3 months, head circumference started to fall below the normal mean in girls (0.5-2 cm). In boys, mean head circumference was below the normal mean already at 1 month of age (2 cm). The deficit increased to 3 cm from 6 months to 4 years. Adult OFC was 52.85 +/- 1.75 cm (n = 16) compared to 55.70 +/- 1.83 cm (n = 46; P0.00001) in WBS women and 55.51 +/- 1.68 cm (n = 30) compared to 57.87 +/- 1.29 cm (n = 31; P0.00001) in WBS men. During development, microcephaly is only seen in about one third of WBS patients.

The auditory space map in the superior colliculus of the guinea-pig requires for its normal emergence at 32 days after birth (DAB) bimodal, auditory and visual experience during a 4-day crucial period (26 - 30 DAB) (Withington-Wray et al., Neurosci. Lett., 116, 280 - 286, 1990d). The need for auditory experience has been proposed to be linked to the rapid changes in the cues for auditory localization brought about by head growth. Head growth is particularly rapid during the first postnatal month but continues more slowly until approximately 70 DAB. This suggests that sensory experience may be required beyond the initial crucial period to accommodate later growth. In this paper the role of auditory experience beyond the crucial period has been investigated. Deprivation of ordinary auditory experience was effected by placement of the animals in an environment in which continuous omnidirectional noise obscured the cues of sound direction. Animals deprived of auditory experience during the crucial period and then allowed normal experience showed limited ability to construct an auditory space map and the resulting map was less accurate than that found in normally reared animals. Auditory deprivation following normal experience during the crucial period caused a profound degradation, of both spatial tuning and topography, of auditory multi-unit receptive fields in the superior colliculus. The spatial tuning and topography of auditory fields from older animals (100 DAB) deprived of ordinary auditory cues for a 4-week period were normal. Thus, in the guinea-pig, susceptibility of the superior collicular space map to deprivation of auditory cues is limited by the age of the animal. The timing of the cessation of vulnerability may, in part, be due to the stabilization of directional cues affected by head growth.

Pregnant women who have phenylketonuria (PKU; McKusick 261600) are at risk of having children with microcephaly, cardiac defects and mental retardation (Dent 1957; Mabry et al 1963; Lenke and Levy 1980). The results of a 7-year longitudinal, prospective, observational investigation on maternal PKU in the United States and Canada are reported. The goal of the study is to enroll all women of child-bearing age whose blood phenylalanine level on an unrestricted diet is greater than 240 μmol/L (4 mg/dl). Those planning pregnancy are treated with a phenylalanine-restricted diet and adequate nutrition is maintained while keeping the blood phenylalanine level between 120 and 360 μmol/L (2 and 6 mg/dl). The outcome of 318 pregnancies is presented and compared to 52 control pregnancies

Growth of the fetal head has been reexamined in a longitudinal study of 32 normally growing fetuses using the head profile area (HA). The Rossavik growth model (characterized by the coefficients c, k, and s) fit these data very well (R2 [+/- SD] 99.6 [+/- 0.3]%), and regression analysis indicated that optimum results are obtained when the coefficient k is kept constant. Head area growth before 28 weeks was found to be related to the coefficient c, which was strongly correlated (R2 69.2%) with the time of HA growth initiation. These results suggest that c reflects the genetic factors controlling growth in the early part of pregnancy. The coefficient s was negatively correlated with c (R2 82.1%) and had two components, one associated with c (pred. s) and one not associated with c (s-residual). This linkage between c and s, together with the parallel head growth seen after 30 weeks, suggests the possibility of a maternal system in which genetically determined head size (c) can be sensed and modified by other factors (s) to maximize compatibility between the fetal head and maternal pelvis.

Two children from a small Amerindian community presented with profound retardation, initial hypotonia progressing to hypertonia, scaphocephaly, a prominent occiput, poor postnatal head growth, and additional minor anomalies. Abuse of gasoline by inhalation was a widespread problem in the community, and gasoline inhalation during the pregnancy could be documented in both of the pregnancies. We are raising the question as to whether inhalation of gasoline during pregnancy may be teratogenic in humans.