Sterling K. Clarren

Incidence of fetal alcohol syndrome and prevalence of alcohol-related neurodevelopmental disorder.

We critique published incidences for fetal alcohol syndrome (FAS) and present new estimates of the incidence of FAS and the prevalence of alcohol-related neurodevelopmental disorder (ARND). We first review criteria necessary for valid estimation of FAS incidence. Estimates for three population-based studies that best meet these criteria are reported with adjustment for underascertainment of highly exposed cases. As a result, in 1975 in Seattle, the incidence of FAS can be estimated as at least 2.8/1000 live births, and for 1979-81 in Cleveland, approximately 4.6/1,000. In Roubaix, France (for data covering periods from 1977-1990), the rate is between 1.3 and 4.8/1,000, depending on the severity of effects used as diagnostic criteria. Utilizing the longitudinal neurobehavioral database of the Seattle study, we propose an operationalization of the Institute of Medicines recent definition of ARND and estimate its prevalence in Seattle for the period 1975-1981. The combined rate of FAS and ARND is thus estimated to be at least 9.1/1,000. This conservative rate--nearly one in every 100 live births--confirms the perception of many health professionals that fetal alcohol exposure is a serious problem.

Brain malformations related to prenatal exposure to ethanol.

Microcephaly and mental retardation have been principal features of the fetal alcohol syndrome. This article describes the neuropathologic findings in four human neonates who were exposed to large quantities of ethanol at frequent intervals during gestation. The findings suggest that intrauterine exposure to ethanol can result in structural abnormalities of the brain. All four brains displayed similar malformations stemming from errors in migration of neuronal and glial elements. Hydrocephalus was one consequence of the malformations in two of the infants. Futhermore, the brain alterations may be the only distinct abnormality produced by in utero ethanol exposure. Only two of the four subjects were diagnosed as having the fetal alcohol syndrome from external criteria.

Maternal Alcohol Use during Breast-Feeding and Infant Mental and Motor Development at One Year

Abstract The detrimental effects of maternal drinking during pregnancy on fetal health have been documented. The consequences for infants of maternal drinking during breast-feeding are unknown, but research in animals suggests that the infant could be affected by exposure to alcohol through the mothers milk. In a study of 400 infants born to members of a health maintenance organization, we investigated the relation of the mothers use of alcohol during breast-feeding to the infants development at one year of age. Mental development, as measured by the Bayley Mental Development Index (MDI), was unrelated to maternal drinking during breast-feeding. However, motor development, as measured by the Psychomotor Development Index (PDI), was significantly lower in infants exposed regularly to alcohol in breast milk (after alcohol exposure during gestation was controlled for), with a dose–response relation (P for linear trend, 0.006). The infants of breast-feeding mothers who had at least one drink daily had a me...

The differential diagnosis of posterior plagiocephaly : true lambdoid synostosis versus positional molding

&NA; The diagnosis and treatment of posterior plagiocephaly is one of the most controversial aspects of craniofacial surgery. The features of true lamibdoid synostosis versus those of deformational plagiocephaly secondary to positional molding are inadequately described in the literature and poorly understood. This has resulted in many infants in several craniofacial centers across the United States undergoing major intracranial procedures for nonsynostotic plagiocephaly. The purpose of this study was to describe the detailed clinical, imaging, and operative features of true lamhdoid svnostosis and contrast them with the features of positional plagiocephaly. During a 4‐year period from 1991 to 1994, 102 patients with posterior plagiocephaly were assessed in a large multidisciplinary craniofacial program. During the same period, 130 patients with craniosynostosis received surgical treatment. All patients were examined by a pediatric dysmorphologist, craniofacial surgeon, and pediatric neurosurgeon. Diagnostic imaging was performed where indicated. Patients diagnosed with lambdoid synostosis and severe and progressive positional molding underwent surgical correction using standard craniofacial techniques. Only 4 patients manifested the clinical, imaging, and operative features of unilambdoid synostosis, giving an incidence among all cases of craniosynostosis of 3.1 percent. Only 3 among the 98 patients with positional molding required surgical intervention. All the patients with unilambdoid synostosis had a thick ridge over the fused suture, identical to that found in other forms of craniosynostosis, with compensatory contralateral parietal and frontal bossing and an ipsilateral occipitomastoid bulge. The skull base had an ipsilateral inferior tilt, with a corresponding inferior and posterior displacement of the ipsilateral ear. These characteristics were completely opposite to the findings in the 98 patients who had positional molding with open lambdoid sutures and prove conclusively that true unilambdoid synostosis exists as a specific but rare entity. Awareness of the features of unilambdoid synostosis will allow more accurate diagnosis and appropriate treatment of posterior plagiocephaly in general and in particular will avoid unnecessary surgical intervention in patients with positional molding. (Plast. Reconstr. Surg. 98: 765, 1996.)

Nasal dermoid sinus cysts: association with intracranial extension and multiple malformations.

Nasal dermoid and sinus cysts (NDSC) are uncommon congenital anomalies that may have intracranial extension and can be associated with other anomalies. We identified 22 patients in a retrospective review of cases diagnosed with NDSC at our institution over the past 10 years. Nine (41 percent) had associated anomalies and ten (45 percent) had intracranial extension of the sinus. In half of the patients with intracranial extension, the sinus transversed either the cribriform plate or foramen cecum and attached to the dura; in the other half, the sinus extended to cysts within the falx or other brain structures. Of the patients with multiple anomalies, six (67 percent) had intracranial extension. Presurgical complications occurred in a total of eight patients (36 percent): two had meningitis, two had osteomyelitis, four had periorbital-nasal cellulitis, three had nasal abscess, and four had nose anomalies requiring rhinoplasty.

Fetal alcohol syndrome: A new primate model for binge drinking and its relevance to human ethanol teratogenesis

Ethanol was administered nasogastrically to four gravid pigtailed macaques (Macaca nemestrina) weekly from 40 days after conception to term. Three animals received 2.5 gm/kg and one received 4.1 gm/kg per dose. One animal aborted after the first dose of 2.5 gm/kg ethanol. Serum ethanol and acetaldehyde were measured after each dose in the other three animals, who carried to term. After delivery the infants were assessed for growth, dysmorphic features, and neurologic and psychological development over six months and were compared with 10 age- and sex-matched controls. Complete autopsies with neuropathologic examinations were performed. The animal exposed to the high dose had neurologic, developmental, and facial anomalies similar to those seen in human fetal alcohol syndrome. One of the animals exposed to the more moderate dosage was similarly but less severely affected. The study demonstrates that a model for binge drinking and fetal alcohol syndrome can be developed in a primate. The model should be useful in exploring the mechanisms of teratogenesis and in determining the median effective dose for the production of the various anomalies seen in fetal alcohol syndrome.

Cognitive and behavioral deficits in nonhuman primates associated with very early embryonic binge exposures to ethanol

This study was undertaken to evaluate teratogenesis associated with early weekly ethanol exposure followed by later gestational abstinence. Ethanol, 1.8 gm/kg, was orally administered weekly to gravid nonhuman primates (Macaca nemestrina) for the first 3, 6, or the entire 24 weeks of pregnancy. Control animals received weekly sucrose solution as did the 3- and 6-week cohort animals in subsequent weeks. Thirty-five viable infants were assessed for growth, malformations, and behavioral and cognitive dysfunction. Animals in the 6-week and 24-week cohorts were uniformly abnormal in behavior and inconsistently abnormal in physical development relative to the control animals. Animals in the 3-week cohort were equivocally normal. These results demonstrate ethanols capacity to produce behavioral teratogenesis (brain dysfunction) in isolation from physical anomalies in the rest of the body. The results strongly suggest that binge drinking in the first 6 to 8 weeks of pregnancy (a period when women may not know that they are pregnant), followed by later gestational abstinence, is as dangerous to the fetus as exposure throughout gestation.

Fetal alcohol syndrome : Changes in craniofacial form with age, cognition, and timing of ethanol exposure in the macaque

One component of the fetal alcohol syndrome (FAS) facial phenotype is a frontonasal anomaly characterized by a thin upper lip and a smooth philtrum. The expression of this anomaly can diminish with age and occurs infrequently in prenatal alcohol-exposed individuals. This study sought to explain these observations. Standardized craniofacial cephalograms of 18 nonhuman primates exposed weekly to ethanol or sucrose solution in utero were measured at ages 1, 6, 12, and 24 months to assess skeletal changes in craniofacial form with age, cognition, and timing of ethanol exposure. The data suggest that there may be a critical period for induction of alcohol-induced craniofacial alterations that occurs very early in gestation and is very short in duration (gestational days 19 or 20). The alterations were scarcely detectable at age 1 month, were most prominent at 6 months, and diminished progressively at 12 and 24 months in the macaque. The appearance and disappearance of the thin upper lip and smooth philtrum may be explained by underlying changes in skeletal structure with age. The infrequent occurrence of the FAS frontonasal anomaly may be explained, in part, by its short critical period of induction.

Ankylosed teeth as abutments for maxillary protraction: A case report

It has been recognized that using the maxillary teeth to deliver extraoral force to the maxilla not only results in sutural remodeling but also periodontal remodeling and tooth movement. In patients with severe maxillomandibular malrelationships, the potential for tooth movement often limits the amount and duration of extraoral force and, consequently, affects the success of treatment. This case report describes a technique to intentionally ankylose deciduous teeth in a patient with severe maxillary retrusion. The ankylosed teeth were used as abutments to deliver an anteriorly directed intermittent extraoral force. After 12 months of treatment, the anterior crossbite was nearly corrected. At that point the ankylosed teeth loosened because of root resorption and the treatment was terminated. Cephalometric superimposition demonstrated that the occlusal correction was the result of anterior maxillary movement with little mandibular growth and no movement of the ankylosed teeth. The results suggest that intentionally ankylosed teeth may be used as abutments for extraoral traction in patients with a severe disturbance in maxillary growth.

FIT OF BICYCLE SAFETY HELMETS AND RISK OF HEAD INJURIES IN CHILDREN

Background—Although bicycle helmets are effective in preventing head and brain injury, some helmeted individuals nevertheless sustain head injury. One of the possible reasons may be poor fit of the helmet on the head. This study was undertaken to examine the relationship between helmet fit and risk of injury. Methods—1718 individuals who were helmeted riders in a crash were queried on helmet fit and position. A sample of 28 children 2–14 years of age who sustained a head injury while wearing a bicycle helmet and 98 helmeted individuals of the same age treated in the same hospital emergency departments for injuries other than to the head, underwent anthropometric measurements of helmet fit. Measurements were made of the childs head, the helmet, and on a cast made of the childs head. Results—Individuals whose helmets were reported to fit poorly had a 1.96-fold increased risk of head injury compared with those whose helmets fit well. Children with head injuries had helmets which were significantly wider than their heads compared with children without head injuries. Helmet fit was poorer among males and among younger children. Conclusions—Poor fit of helmets may be associated with an increased risk of head injury in children, especially in males. Helmets may not be designed to provide optimal protection.