Extensive and complex defects of the scalp, middle third of the face, and palate: the role of microsurgical reconstruction

Twenty-one patients with gigantic defects of the scalp and middle third of the face and palate following excision of neglected or recurrent tumors, burns, and infections have undergone microsurgical reconstruction. Wide resection of the middle third of the face, orbit, and palate requires "complex" three-dimensional volume reconstruction, whereas extensive defects of the scalp and skull (exceeding 80 cm2) require coverage of the larger surface area soft-tissue defect and the exposed brain and dura. The latissimus dorsi free-muscle flap and split-thickness skin graft have become our methods of choice for extensive scalp and skull defects. The latissimus dorsi musculocutaneous free flap is preferable for reconstruction of complex palatal and external skin and orbital defects of the middle third of the face. Microsurgical free-tissue transfer reliably frees the oncologic surgeon from the constraints imposed by conventional reconstructive techniques and may therefore allow improved curative or at least palliative resection of these extensive tumors.     

Differences and similarities in the intra-uterine behaviour of monozygotic and dizygotic twins.

Diagnostic advances have made it possible to use ultrasonograph to assess placentation and therefore zygosity in utero in the case of monochorionic-monozygotic twins. Foetal behaviour of 15 monozygotic and 15 unlike-sexed dizygotic twin pairs was studied serially with ultrasounds from 10 to 22 weeks gestational age. Each twin, regardless of its zygosity, showed individualised behavioural styles. One twin was found to be 'dominant' in the sense of being more active, but less reactive, possibly due to the fewer stimuli being generated by its co-twin. Monozygotic twins, as opposed to dizygotic twins, showed greater similarities in activity and reactivity levels, but were never behaviourally identical and decreased in likeness with increasing age. Our data suggest that so-called identical twins are very similar, but not behaviourally identical, from very early in pregnancy. The unequally shared intrauterine environment contributes to putting each monozygotic twin on a progressively distinct behavioural path.     

Reconstruction of large cranial defects in the presence of heavy radiation damage and infection utilizing tissue transferred by microvascular anastomoses

Six cases of large defects of the scalp, skull, and dura following tumor ablation and radiation are presented. Each was accompanied by chronic infection in the irradiated defect. Efforts to reconstruct the resulting defects with local flaps were not successful. One-stage reconstruction was then accomplished in each case utilizing a latissimus dorsi musculocutaneous or myo-osteocutaneous free flap transferred by microvascular anastomoses. The versatility of the latissimus dorsi musculocutaneous and/or osseous flap allows single-stage reconstruction of these complex defects.     

Successful use of olanzapine in adolescent monozygotic twins with catatonic schizophrenia resistant to electroconvulsive therapy: case report

We describe a case of monozygotic (MZ) male twins (14.6 years old) who suffered from a severe form of catatonic schizophrenia. On admission, the principal symptoms of the brothers were stupor, mutism, catatonic posturing, rigidity, negativism, and refusal of food and liquids. They were treated with electroconvulsive therapy (ECT) with no effect (twin A) and almost no effect (twin B). Both twins improved with initiation of olanzapine therapy. Twin B showed a marked improvement by week 2 on a dose of 10 mg daily (qd). Improvement in twin A was seen by week 4 on a dose of 15 mg qd. Twin B was discharged after 8 weeks and twin A after 11 weeks of olanzapine treatment. This appears to be the first report on concordant positive responses to olanzapine in MZ twins with catatonic schizophrenia, as well as, the first report on concordant resistance to ECT.     

The osteogenic role of the dura mater in adult rabbits during regeneration of the skull]

It was shown in experiments with adult rabbits that the regeneration of skull vault bones after artificial trauma proceeds, mainly, at the expense of osteogenic activity of dura mater, rather than by means of outgrowth of bone from the defect margins. During regeneration, dura mater connects with the granulation tissue which fills the area of defect. The first bone islets are formed by the surface layer of dura mater near the defect margins and then all over the defect area. During regeneration bone islets merge with each other and with the old bone at the defect margins. In experiments with separation of the defect margins from dura mater by millipore filter, regeneration is insignificant over the filter near the old bone margins (bone trabeculae form which close destructed bone marrow cavities); the bone forms intensively under the filter on dura mater. In experiments with the removal of a piece of skull bone together with the adjacent region of dura mater, no bone regeneration occurs, the defect area is filled by the scar tissue.     

Aplasia cutis congenita of the scalp: delayed closure complicated by massive hemorrhage

A case of aplasia cutis congenita involving the scalp, cranium, and dura is presented to illustrate the complication of major hemorrhage from the sagittal sinus that may occur if closure is delayed. Subsequent problems with scalp-flap cover eventually required split-skin grafting of the defect. The case for surgery as soon after birth as possible is reinforced.     

The role of the placenta in variability of fetal exposure to cocaine and cannabinoids: a twin study.

There is wide variability in the reported adverse fetal effects of cocaine and cannabinoids. The causes of this variability are largely unknown. We hypothesized that variability in placental handling of drugs affect fetal exposure. We used twin pregnancies as a paradigm to address the role of the placenta in this variability. We analyzed hair or meconium samples taken from dizygotic and monozygotic twins exposed in utero to illicit drugs. Out of 12 pairs, 5 had negative levels in both twins, and seven pairs of twins had chemical evidence of fetal exposure to cocaine (n = 5) or cannabinoids (n = 2). The one known monozygotic pair of twins had almost identical levels of cocaine. In contrast, the six dizygotic pairs had large disparities in either cocaine or cannabinoid concentrations. In three of these six dizygotic pairs, levels of cocaine (n = 2) or canabinoids (n = 1) were undetectable in one twin while positive in the other. Given that twins are theoretically exposed to similar maternal drug levels, our findings suggest that the placenta may have a major role in modulating the amounts of drug reaching the fetus.     

Circumferential abdominal skin defect possibly due to umbilical cord encirclement.

We report on a newborn black male twin with a distinctive circumferential abdominal skin defect who was identified through the Active Malformation Surveillance Program at the Brigham and Women's Hospital. There were no other malformations, and amniotic disruption was not present. Although it cannot be proven, we believe that this skin defect may have been caused by in utero encirclement of the abdomen by an umbilical cord.     

Congenital scalp defect with thrombosis of the sagittal sinus. Case report.

A case report of a congenital scalp defect, associated with thrombosis of the superior sagittal sinus, is presented. A thrombectomy of the sinus, followed by skin grafting, was successful in achieving wound closure and a healthy, normal child. We propose that thrombectomy be considered for future similar situations.     

A comparison of the proliferative and replicative life span kinetics of cell cultures derived from monozygotic twins

Summary We have compared the growth rates, kinetics of cell aging, and replicative life spans of skin fibroblast cell cultures derived from three pairs of monozygotic twins of similar ages. The results of these studies indicated no significant differences in the cell densities 7 days after inoculation or replicative life spans within each twin pair but highly significant differences among each twin pair. The kinetics by which each culture aged ([³H]thymidine-labeled nuclei) were compared within and among the twin cell cultures. Although the slopes of each regression line were not significantly different, comparisons of the elevations of each line supported the conclusion that the aging of monozygotic twin cell cultures is similar within the twin pairs but differs among the twin pairs. This research was supported by IIT Research Institute.     

Treating Cutis Verticis Gyrata Using Skin Expansion Method

The objective of this study was to evaluate the feasibility and clinical effect of repairing scalp defect after the excision of cutis verticis gyrata using expanded scalp skin flaps. For this purpose, 8 patients with cutis verticis gyrata were subjected to scalp skin expander implantation under the skin. After saline injection and scalp expansion for 2–3 months, the cutis verticis gyrata was excised and the expanded scalp flaps were applied to recover the skin defect. As a result, the flaps and hair grew well without contractures and significant scarring, suggesting that this method is useful for surgical correction of cutis verticis gyrata.     

超早期三维钛网颅骨修补术的临床研究

目的:探讨重型颅脑损伤去骨瓣减压手术后颅骨缺损在超早期(4~6周内)行三维钛网颅骨修补的可行性和对患者长期预后的影响,探讨超早期颅骨修补术手术中是否较常规手术存在优势。方法:回顾性分析自2012年1月-2015年1月行颅脑损伤后颅骨缺损手术修补患者99例。将所有患者根据去骨瓣减压术后行颅骨修补的间隔时间分为两组,4-6周以内为超早期组,共52例,3-6个月为常规组,共47例。采用不同国际评分标准比较两组患者在颅骨修补术后1个月、3个月、12个月的生存质量;对比分析两组患者的术中头皮剥离时间及术中出血量;比较两组患者术后1个月、3个月、12个月相应并发症的差异。结果:超早期组患者术后1个月的格拉斯哥评分(Glasgow outcome scale,GOS)、美国国立卫生院神经功能缺损评分(NIHSS)和远期卡氏功能状态(Karnofsky performance status,KPS)评分较常规组比较无统计学意义(P0.05);超早期组患者术后3个月和12个月的GOS、NIHSS和KPS评分较常规组均有显著提高(P0.05)。超早期组患者头皮剥离时间较常规修补组明显缩短(P0.05),出血量明显减少(P0.05);两组颅骨修补术后硬膜下积液发生率明显降低,差异有统计学意义(P0.05),然而总体并发症发生率并无明显差异(P0.05)。结论:重度颅脑损伤去骨瓣减压术后患者在超早期(4~6周内)行颅骨修补在临床上是安全有效的,能够改善患者的预后和减少术后并发症的发生概率,并且能够减少术中出血,手术中头皮剥离时间也有缩短。     

New chromosome 11p15 epigenotypes identified in male monozygotic twins with Beckwith-Wiedemann syndrome

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome demonstrating heterogeneous molecular alterations of two imprinted domains on chromosome 11p15. The most common molecular alterations include loss of methylation at the proximal imprinting center, IC2, paternal uniparental disomy (UPD) of chromosome 11p15 and hypermethylation at the distal imprinting center, IC1. An increased incidence of female monozygotic twins discordant for BWS has been reported. The molecular basis for eleven such female twin pairs has been demonstrated to be a loss of methylation at IC2, whereas only one male monozygotic twin pair has been reported with this molecular defect. We report here two new pairs of male monozygotic twins. One pair is discordant for BWS; the affected twin exhibits paternal UPD for chromosome 11p15 whereas the unaffected twin does not. The second male twin pair is concordant for BWS and both twins of the pair demonstrate hypermethylation at IC1. Thus, this report expands the known molecular etiologies for BWS twins. Interestingly, these findings demonstrate a new epigenotype-phenotype correlation in BWS twins. That is, while female monozygotic twins with BWS are likely to show loss of imprinting at IC2, male monozygotic twins with BWS reflect the molecular heterogeneity seen in BWS singletons. These data underscore the need for molecular testing in BWS twins, especially in view of the known differences among 11p15 epigenotypes with respect to tumor risk.     

Single-stage approach to skin grafting the exposed skull

Four patients are presented who suffered full-thickness loss of the scalp, exposing the skull. Removal of the outer table, immediate application of a split-thickness skin graft, and treatment with the VAC for 3 to 4 days resulted in approximately 100 percent graft take in each case without complications. When compared with the usual two-stage approach to skin grafting the exposed skull, this method spares the patient a longer hospital stay and a second operative procedure, and it results in a significant cost savings.     

Diversity of the human immunodeficiency virus type 1 (HIV-1) env sequence after vertical transmission in mother-child pairs infected with HIV-1 subtype A

Although several virologic and immunologic factors associated with an increased risk of perinatal human immunodeficiency virus type 1 (HIV-1) transmission have been described, the mechanism of mother-to-child transmission is still unclear. More specifically, the question of whether selective pressures influence the transmission remains unanswered. The aim of this study was to assess the genetic diversity of the transmitted virus after in utero transmission and after peripartum transmission and to compare the viral heterogeneity in the child with the viral heterogeneity in the mother. To allow a very accurate characterization of the viral heterogeneity in a single sample, limiting-dilution sequencing of a 1016-bp fragment of the env gene was performed. Thirteen children were tested, including 6 with in utero infections and 7 with peripartum infections. Samples were taken the day after birth and at the ages of 6 and 14 weeks. A homogeneous virus population was seen in six (46.2%) infants, of whom two were infected in utero and four were infected peripartum. A more heterogeneous virus population was detected in seven infants (53.8%), four infected in utero and three infected peripartum. The phylogenetic trees of the mother-child pairs presented a whole range of different tree topologies and showed infection of the child by one or more maternal variants. In conclusion, after HIV-1 transmission from mother to child a heterogeneous virus population was detected in approximately one-half of the children examined. Heterogeneous virus populations were found after peripartum infection as well as after in utero infection. Phylogenetic tree topologies argue against selection processes as the major mechanism driving mother-to-child transmission but support the hypothesis that virus variability is mainly driven by the inoculum level and/or exposure time.     

Mortality among twins after age 6: fetal origins hypothesis versus twin method.

OBJECTIVE--To test the validity of the fetal origins hypothesis and the classic twin method. DESIGN--Follow up study of pairs of same sex twins in which both twins survived to age 6. SETTING--Denmark. SUBJECTS--8495 twin individuals born 1870-1900, followed through to 31 December 1991. MAIN OUTCOME MEASURES--Mortality calculated on a cohort basis. RESULTS--Mortality among twins and the general population was not significantly different except among females aged 60-89, in whom mortality among twins was 1.14 times (SE 0.03) higher than in the general population. Mortality among female dizygotic twins was 1.77 times (0.18) higher than among monozygotic twins at age 30-59. Otherwise, mortality for monozygotic and dizygotic twins did not consistently differ after age 6. CONCLUSION--According to the fetal origins hypothesis the risk of adult morbidity and mortality is heightened by retardation in intrauterine growth. Twins, and in particular monozygotic twins, experience growth retardation in utero. The findings in the present study suggest that the fetal origins hypothesis is not true for the retardation in intrauterine growth experienced by twins. Furthermore, the data are inconsistent with the underlying assumption of a recent claim that the classic twin method is invalid for studies of adult diseases. The present study is, however, based on the one third of all pairs of twins in which both twins survived to age 6. The possible impact of this selection can be evaluated in future studies of cohorts of younger twins with lower perinatal and infant mortality.     

The importance of nonlinear tissue modelling in finite element simulations of infant head impacts

Despite recent efforts on the development of finite element (FE) head models of infants, a model capable of capturing head responses under various impact scenarios has not been reported. This is hypothesized partially attributed to the use of simplified linear elastic models for soft tissues of suture, scalp and dura. Orthotropic elastic constants are yet to be determined to incorporate the direction-specific material properties of infant cranial bone due to grain fibres radiating from the ossification centres. We report here on our efforts in advancing the above-mentioned aspects in material modelling in infant head and further incorporate them into subject-specific FE head models of a newborn, 5- and 9-month-old infant. Each model is subjected to five impact tests (forehead, occiput, vertex, right and left parietal impacts) and two compression tests. The predicted global head impact responses of the acceleration–time impact curves and the force–deflection compression curves for different age groups agree well with the experimental data reported in the literature. In particular, the newly developed Ogden hyperelastic model for suture, together with the nonlinear modelling of scalp and dura mater, enables the models to achieve more realistic impact performance compared with linear elastic models. The proposed approach for obtaining age-dependent skull bone orthotropic material constants counts both an increase in stiffness and decrease in anisotropy in the skull bone—two essential biological growth parameters during early infancy. The profound deformation of infant head causes a large stretch at the interfaces between the skull bones and the suture, suggesting that infant skull fractures are likely to initiate from the interfaces; the impact angle has a profound influence on global head impact responses and the skull injury metrics for certain impact locations, especially true for a parietal impact.     

Repair of scalp defects using a tissue expander and Marlex mesh.

A simple technique using Marlex mesh and a tissue expander to cover scalp defects is described and two patients are presented. This technique is suitable for medium-sized defects that cannot be closed primarily. Marlex mesh is sutured to the wound edges in lieu of a temporary skin graft and to prevent enlargement of the defect during tissue expansion. The tissue expander is placed under adjacent normal scalp in a subgaleal pocket developed through the scalp defect. The scalp defect is closed secondarily using the expanded scalp flap. This technique was performed in two patients with satisfactory results. Marlex mesh obviates the need for a temporary skin graft to cover the scalp defect.     

The outcome of twin pregnancies complicated by single fetal death after 20 weeks of gestation.

A retrospective study involving 972 twin births was conducted to evaluate the maternal and fetal outcomes of twin pregnancies complicated by single fetal death. The incidence of single fetal death in twin pregnancies after 20 weeks was 3.3%. Preterm birth rates for 37 and 32 gestational weeks were 81.3% and 41.6% respectively. The median interval between the diagnosis of fetal death and the delivery was 11 days (range 1-27 days). Eighteen (56%) infants were delivered by cesarean and 14 (43%) vaginally. Twin-twin transfusion syndrome (TTTS) was the cause of single fetal death in 8 of 32 twin pregnancies (25%). Ten of the surviving co-twins were lost in the neonatal period (31.3%) and half of those neonatal deaths were due to TTTS. TTTS is the major contributor for perinatal mortality in same-sex twins complicated by single fetal death. The death of one twin in utero should not be the only indication for preterm delivery, and in case of severe prematurity with a stable intrauterine environment; expectant management may be advisable until fetal lung maturation ensues.