| 1. |
- Ahlman, Håkan, 1947, et al.
(författare)
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Clinical management of gastric carcinoid tumors.
- 1994
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Ingår i: Digestion. - 0012-2823. ; 55 Suppl 3, s. 77-85
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Tidskriftsartikel (refereegranskat)abstract
- Four types of gastric carcinoids have been identified: (1) multiple small body-fundus carcinoids associated with chronic atrophic gastritis type A (A-CAG); (2) sporadic solitary lesions without specific pathogenetic background (non-A-CAG); (3) carcinoidosis associated with Zollinger-Ellison/MEN 1 syndrome, and (4) rare tumors, e.g. gastrin cell tumors, neuroendocrine carcinomas and mixed endocrine-exocrine tumors. In a retrospective study of 15 patients with gastric carcinoids (11 A-CAG, 3 non-A-CAG and 1 gastrin cell tumor) over a 10-year period, the histopathological and clinical features were assessed. The A-CAG-type carcinoids were clinically silent with lymph node metastases in 2/11 cases but no hepatic metastases. The non-A-CAG-type carcinoids were malignant with disseminated disease, hormonal symptoms and increased urinary excretion of the main histamine metabolite, MeImAA. Five patients with A-CAG tumors were subjected to antrectomy to remove hypergastrinemia, which is thought to be of pathogenetic importance for these tumors. During the observation period (1.5-8 years) 1 patient developed recurrent tumors, while the other 4 showed persistent argyrophil cell hyperplasia. A prospective treatment protocol of these tumors is suggested with endoscopic removal of less numerous, small lesions as first-step therapy, followed by antrectomy at recurrence. Larger lesions should be excised in combination with antrectomy. Gastrectomy is reserved for the rare cases of invasive tumors with lymph node metastases. As evident from the outcome of patients with non-A-CAG tumors radical surgery should be performed whenever practicable.
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| 2. |
- Kölby, David, 1993, et al.
(författare)
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Craniotomy of the Fused Sagittal Suture Over the Superior Sagittal Sinus Is a Safe Procedure
- 2017
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Ingår i: Journal of Craniofacial Surgery. - : Ovid Technologies (Wolters Kluwer Health). - 1049-2275. ; 28:3, s. 666-669
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Tidskriftsartikel (refereegranskat)abstract
- Introduction: Spring-assisted cranioplasty to correct sagittal synostosis is based on midline craniotomy through the closed sagittal suture, over the superior sagittal sinus (SSS). The aim of the present study was to evaluate the perioperative safety of this technique. Materials and Methods: This is a retrospective study of all patients operated with median craniotomy and springs from 1998 to the end of 2015. For comparison, all Pi-plasties performed during the same time interval were also evaluated. The safety measures were evaluated based on incidence of damage to SSS, incidence of dural tears, perioperative blood loss, operative time, and hospital stay. Results: In the group that had undergone midline craniotomy combined with springs (n = 225), 4 perioperative damages to SSS and 1 dural tear were seen. The perioperative blood loss was 62.8 +/- 65.3mL (mean +/- standard deviation). The operative time was 67.9 +/- 21.5 minutes and the hospital stay was 4.8 +/- 1.1 days. In the group that had undergone pi-plasty (n = 105), no damages to SSS but 3 dural tears were seen. The perioperative blood loss was 352.8 +/- 174.4 mL. The operative time was 126.0 +/- 31.7 minutes and the hospital stay was 7.1 +/- 1.4 days. Conclusion: Craniotomy SSS in sagittal synostosis is a safe procedure with low morbidity in terms of damage to the SSS. Midline craniotomy combined with springs has significantly lower preoperative blood loss, operative time, and hospital stay (P < 0.001 for all) compared to pi-plasty.
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| 3. |
- Paganini, Anna, 1979, et al.
(författare)
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In search of a single standardised system for reporting complications in craniofacial surgery: a comparison of three different classifications.
- 2019
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Ingår i: Journal of plastic surgery and hand surgery. - 2000-6764. ; 59:6, s. 321-7
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Tidskriftsartikel (refereegranskat)abstract
- Comparing complication rates between centres is difficult due to the lack of unanimous criteria regarding what adverse events should be defined as complications and how these events should be compiled. This study analysed all adverse events in a cohort of craniofacial (CF) operations over a 10-year period and applied three different scales (Clavien-Dindo, Leeds and Oxford) for systematic comparison. A total of 1023 consecutive CF procedures in 641 patients was identified. The Clavien-Dindo scale captured 74 complications in 74 procedures (7.2%), whereas the Leeds and Oxford scales captured 163 complications in 134 procedures (13.1%) and 85 complications in 83 procedures (8.1%), respectively. The Clavien-Dindo scale appeared less suitable for CF surgery, because it is predominantly adapted to severe complications and also regards blood transfusion as a complication. The Leeds scale provided a detailed picture of all complications, as well as minor events, whereas the Oxford scale captured all major complications well but applied less accurate definitions for the minor events. Our findings contribute to the benchmarking of complications between CF centres and suggest that both the Leeds and the Oxford scale appear relevant, depending on the emphasis required for major and minor complications and inter-centre audits, respectively.
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| 4. |
- Ahlman, Håkan, 1947, et al.
(författare)
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The relevance of somatostatin receptors in thyroid neoplasia.
- 1997
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Ingår i: The Yale journal of biology and medicine. - 0044-0086. ; 70:5-6, s. 523-33
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)abstract
- 111In-octreotide scintigraphy in patients with persistent medullary thyroid carcinoma (MTC) visualized tumors in about half of the surgically explored sites. Tumor visualization correlated with rapid tumor growth and large tumor volume as judged from calcitonin levels. The 111In concentration ratio between tumor (T) and blood (B) in surgically excised lymph node metastases of MTC showed a large variation, with low values for microscopic and high values for macroscopic metastases in individual patients. Three cases of MTC, Hürthle cell adenoma and papillary thyroid cancer are reported with preoperative scintigraphy, T/B ratios and Northern analyses of the surgical biopsies. Visualization of tumors was possible in the absence of sstr2 (the high affinity receptor for octreotide) with the exception of microscopic tumor growth. T/B values in the patient with Hürthle cell adenoma were similar to those found in the contralateral thyroid lobe with goitre. The relatively high uptake of 111In in benign thyroid conditions probably limits the use of octreotide scintigraphy in the diagnosis of primary tumors. The technique has certain advantages over radioiodine scintigraphy after the surgical treatment of thyroid tumors: no need for withdrawal of thyroxin substitution; a possibility to diagnose metastases of tumors that do not concentrate radioiodine (MTC, Hürthle cell cancer); and complementary information about metastatic sites of non-medullary thyroid cancer (papillary and follicular tumors).
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| 5. |
- Amiri-Mosavi, A, et al.
(författare)
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Expression of cholecystokinin-B/gastrin receptors in medullary thyroid cancer.
- 1999
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Ingår i: The European journal of surgery = Acta chirurgica. - : Oxford University Press (OUP). - 1102-4151. ; 165:7, s. 628-31
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Tidskriftsartikel (refereegranskat)abstract
- To characterise the cholecystokinin (CCK) receptor subtypes in medullary thyroid cancer by measuring the expression of CCK-A and CCK-B/gastrin receptor mRNA.
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| 6. |
- Bernhardt, Peter, 1966, et al.
(författare)
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A novel quantitative image-based method for evaluating cranial symmetry and its usefulness in patients undergoing surgery for unicoronal synostosis.
- 2013
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Ingår i: The Journal of craniofacial surgery. - 1536-3732. ; 24:1, s. 166-9
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Tidskriftsartikel (refereegranskat)abstract
- Background: Unicoronal synostosis presents with cranial asymmetry. Fixed points are difficult to identify; surgical results are therefore difficult to evaluate. The aim of this study was to develop a computer-based method for evaluation of forehead symmetry to enable evaluation of surgical results in unicoronal synostosis. Methods: The MATLAB tool was programmed to segment computed tomographic images, leaving the outermost contour. Cephalometric images were segmented manually due to lower contrast. A center-point (O) and an end-point were manually defined in the midline of the forehead and at the nonfused coronal suture, respectively. The program then found a point (p) on the fused side, at the same distance from the O as the end-point. The contours of the left and right side of the forehead were thereafter superimposed, and the position of minimal area mismatch of the sides was identified. To correct for growth between preoperative images and follow-up, the number of mismatching pixels was related to the area outlined by the contour of the forehead, the end-point and p. Two quantities, the relative symmetry change and the absolute symmetry change, were defined and evaluated by repeated measurements on spherical and elliptical phantoms and 15 patients. Results: Measurements with the MATLAB program were reliable with an SD of 0.26% to 5.39% for the expected range of differences. The SD was lower for measurements on computed tomographic images than for measurements on cephalometric images. The SD was also lower in patients with large surgical improvement than in patients with little improvement. The results support the use of relative symmetry change to evaluate surgical results. Conclusions: Our new computer-based method is capable of measuring forehead symmetry with good precision. This method can be used for systematic evaluation of surgical outcome for unicoronal synostosis and other asymmetric skull deformities.
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| 7. |
- Bevilacqua, Ruggero, et al.
(författare)
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Spring-Assisted Surgery in the Treatment of Complex Craniosynostosis
- 2018
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Ingår i: Journal of Craniofacial Surgery. - 1049-2275. ; 29:4, s. 920-924
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Tidskriftsartikel (refereegranskat)abstract
- Multisutural nonsyndromic craniosynostosis is a rare group of malformations, whose frequency has been reported between 3% and 7% of all craniosynostosis. The clinical diagnosis can be difficult and computed tomography is usually required. Surgical treatment is challenging and staged procedures are performed in up to 80% of patients. The aim of the present study was to determine the reoperation rate and to evaluate the surgical outcomes by measuring intracranial volume (ICV) preoperatively and at follow-up, and comparing it to a control group. Perioperative variables and reoperation rate were recorded. Fifty-one patients presented with a complex pattern of synostosis without a recognizable syndrome (5% of cases of total patients evaluated). Fifteen patients have been treated with spring-assisted surgery, either alone or in combination with a foreheadplasty. The mean follow-up was 6.2 years. The mean preoperative ICV of the patients was smaller, but not significantly, than in the normal population (P = 0.13). Postoperatively, the mean ICV was similar to that of the control group at 1 year (P = 0.92), while at 3 years it was appreciably smaller, although not significantly different (P = 0.06). Five patients (33%) went through a secondary skull expansion for either raised intracranial pressure or cosmetic reasons. Spring-assisted surgery seems to temporarily expand ICV in children with complex synostosis and lower the reoperation rate, thus reducing the need for a second procedure. A longer follow-up would be necessary to further investigate the effects of springs over time.
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| 8. |
- Cornelissen, M. J., et al.
(författare)
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Perinatal complications in patients with unisutural craniosynostosis: An international multicentre retrospective cohort study
- 2017
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Ingår i: Journal of Cranio-Maxillofacial Surgery. - : Elsevier BV. - 1010-5182. ; 45:11, s. 1809-1814
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Tidskriftsartikel (refereegranskat)abstract
- Purpose: Craniosynostosis may lead to hampered fetal head molding and birth complications. To study the interaction between single suture craniosynostosis and delivery complications, an international, multicentre, retrospective cohort study was performed. Materials and methods: All infants born between 2006 and 2012 in the Netherlands and Sweden with sagittal or metopic suture synostosis were included. All births were included as a reference population. The primary outcome measure was rate of medically assisted labor. The secondary outcomes included method of conception, term of birth and fetal position. Results: We included 152 trigonocephaly patients, 272 scaphocephaly patients and 1.954.141 controls. A higher rate of assisted reproductive technology (ART) was found in patients with trigonocephaly (13%) and scaphocephaly (7%) compared to controls (3%, p < 0.001). Scaphocephaly resulted in more postterm births (8% vs 4%, p < 0.001). Trigonocephaly patients showed more preterm births (11% vs 6%, p < 0.001), breech position was more frequent (10% vs 4%, p = 0.003) and labor was more often induced. Rate of assisted delivery, including cesarean section, was significantly higher in both patient groups. Conclusions: Scaphocephaly leads to more postterm births and an increased rate of cesarean sections. Trigonocephaly is related to ART, and in addition higher rates of breech position and cesarean section are found. Prenatal detection of single suture craniosynostosis could improve perinatal care. (C) 2017 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
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| 9. |
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| 10. |
- Fischer, Sara, et al.
(författare)
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Comparisons of Intracranial Volume and Cephalic Index After Correction of Sagittal Craniosynostosis With Either Two or Three Springs
- 2021
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Ingår i: The Journal of craniofacial surgery. - 1536-3732. ; 32:8, s. 2636-2640
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Tidskriftsartikel (refereegranskat)abstract
- ABSTRACT: In this retrospective study, the authors determined changes in intracranial volume (ICV) and cephalic index (CI) in patients with sagittal craniosynostosis and operated with craniotomy combined with either 2 or 3 springs. The authors included patients (n=112) with complete follow-up that had undergone surgical correction for isolated sagittal craniosynostosis with craniotomy combined with springs between 2008 and 2017. All patients underwent computed tomography examination preoperative, at the time of spring extraction, and at 3years of age. Intracranial volume was measured using a semiautomatic MATLAB program, and CI was calculated as the width/length of the skull. The authors found that craniotomy combined with 2 springs increased the ICV from a preoperative value of 792±113mL (mean±standard deviation) to 1298±181mL at 3years of age and increased the CI from 72.1±4.1 to 74.6±4.3, whereas craniotomy combined with 3 springs increased the ICV from 779±128mL to 1283±136mL and the CI from 70.7±4.3 to 74.8±3.7. The relative increase in ICV was 65±21% in the two-spring group and 68±34% in the three-spring group (P value=0.559), and the relative increase in CI was 3.6±3.3% in the two-spring group as compared with 6.0±5.0% in the three-spring group (P=0.004). These findings demonstrated that use of 3 springs resulted in additional absolute and relative CI-specific effects as compared with 2 springs during the time when the springs were in place, with this effect maintained at 3years of age.
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| 11. |
- Fischer, Sara, et al.
(författare)
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Intracranial volume is normal in infants with sagittal synostosis.
- 2015
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Ingår i: Journal of plastic surgery and hand surgery. - 2000-6764. ; 49:1, s. 62-64
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Tidskriftsartikel (refereegranskat)abstract
- Abstract Premature sagittal synostosis results in an elongated, narrow skull shape, scaphocephaly. It has been unclear whether the intracranial volume (ICV) of these children is different from that of normal children. The aim of the present study was to precisely determine the ICV in a large cohort of children with premature sagittal synostosis and to compare it to the ICV of a sex- and age-matched control group. All patients (n = 143) with isolated sagittal synostosis registered in the Göteborg Craniofacial Registry until the end of 2012 with a preoperative CT examination were identified. For each case, a sex- and age- (±30 days) matched control was identified from children who had undergone CT for post-traumatic or neurological reasons. The ICV was measured in a semi-automatic MATLAB program with functions such as region growing, watershed, and thresholding in axial CT slices. The ICV was calculated using the Cavalieri principle. The mean (± SEM) values of ICV for children with sagittal synostosis and for corresponding controls were 866 ± 13 ml and 870 ± 15 ml, respectively. The mean ages of these groups were 173 ± 8 days and 172 ± 8 days, respectively. Subgroup analysis of sex and age at CT (≤180 days and >180 days) did not reveal any differences in ICV between cases and controls. Precise determination of ICV in addition to the use of adequate controls has made it possible to conclude that children with premature isolated sagittal synostosis have a normal ICV.
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| 12. |
- Fischer, Sara, et al.
(författare)
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Psychosocial conditions in adults with Crouzon syndrome: a follow-up study of 31 Swedish patients.
- 2014
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Ingår i: Journal of plastic surgery and hand surgery. - 2000-6764. ; 48:4, s. 244-247
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Tidskriftsartikel (refereegranskat)abstract
- Abstract Crouzon syndrome presents with craniosynostosis, maxillary hypoplasia, exophtalmus, and sometimes hampered neuropsychological development. The aim of the present study was to evaluate the quality of life for adult patients with Crouzon syndrome. Forty patients with Crouzon syndrome born before 1990 could be identified. A questionnaire addressing education, employment, social relations, and quality-of-life was used. A matched control group was created for comparison. Logistic regression, correcting for the influence of age and sex, was used to compare patients and controls. Thirty-one patients and 285 controls answered the questionnaire. The level of education was lower in patients than in controls (p < 0.015). Patients were less often married or had a partner (p = 0.059), had fewer children of their own (p = 0.004), and had less experience of a sexual relationship (p < 0.001). The difference in housing was not significant, and only one patient lived in a care centre and three patients required a personal assistant to manage activities of daily living. The patients' estimation of their somatic health was equal to that of the controls, but the patients more often used anti-epileptic medication (p = 0.003). Periods of depressive mood were more common in patients (p = 0.001), but there was no difference between the groups regarding a general positive attitude to life. In conclusion, patients with Crouzon syndrome often have intellectual and social shortcomings that negatively affect their lives. However, the range of abilities is wide in this group.
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| 13. |
- Fischer, Sara, et al.
(författare)
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Springs Produce Favorable Morphological Outcomes Relative to H-craniectomy According to a Two-center Comparison of Matched Cases.
- 2024
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Ingår i: Plastic and Reconstructive Surgery. - 0032-1052 .- 1529-4242. ; 154:2
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Tidskriftsartikel (refereegranskat)abstract
- Sagittal synostosis is the most common type of premature suture closure, and many surgical techniques are used to correct scaphocephalic skull shape. Given the rarity of direct comparisons of different surgical techniques for correcting craniosynostosis, this study compared outcomes of craniotomy combined with springs and H-craniectomy for non-syndromic sagittal synostosis.Comparisons were performed using available pre- and postoperative imaging and follow-up data from the two craniofacial national referral centers in Sweden, which perform two different surgical techniques: craniotomy combined with springs (Gothenburg) and H-craniectomy (Renier's technique; Uppsala). The study included 23 pairs of patients matched for sex, preoperative cephalic index (CI), and age. CI, total intracranial volume (ICV), and partial ICV were measured before surgery and at 3 years of age, with volume measurements compared against those of pre- and postoperative controls. Perioperative data included operation time, blood loss, volume of transfused blood, and length of hospital stay.Craniotomy combined with springs resulted in less bleeding and lower transfusion rates than H-craniectomy. Although the spring technique requires two operations, the mean total operation time was similar for both methods. Of the three complications that occurred in the group treated with springs, two were spring-related. Importantly, the compiled analysis of changes in CI and partial volume distribution revealed that craniotomy combined with springs resulted in superior morphological correction.The findings showed that craniotomy combined with springs normalized cranial morphology to a greater extent than H-craniectomy based on changes in CI and total and partial ICVs over time.
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| 14. |
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| 15. |
- Fröjd, Victoria, 1986, et al.
(författare)
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Conservative Healing of an 11×9-cm Aplasia Cutis Congenita of the Scalp with Bone Defect.
- 2014
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Ingår i: Journal of neurological surgery reports. - : Georg Thieme Verlag KG. - 2193-6358 .- 2193-6366. ; 75:2
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Tidskriftsartikel (refereegranskat)abstract
- ObjectivesAplasia cutis congenita is a rare congenital condition, and it is difficult to find scientific support for optimal treatment strategies. In addition, these may vary due to defect size, tissue layers involved, contemporary malformations, and the physiologic status of the affected child. Clinical PresentationThis case report describes complete skin coverage in 20 weeks and uneventful healing of a large 11×9-cm defect of the vertex, involving both skin and skull bone, using conservative treatment. To prevent infection and promote healing, the defect was kept moist and covered at all times, and it was treated with surgical debridement when necessary. For infection control, ionized silver-coated dressings were used in addition to prophylactic antibiotics over the first 3.5 weeks. Follow-up was 2 years. ConclusionSurgical treatment is usually preferred for larger aplasia cutis congenita defects, but it is accompanied with potential risks and will exacerbate secondary reconstruction of alopecia or skull bone defects. This case shows that even very complex defects may be treated conservatively.
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| 16. |
- Hagmarker, Linn, et al.
(författare)
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A new quantitative image-based method for evaluation of bony temporal hollowing in metopic synostosis
- 2016
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Ingår i: Journal of Plastic Surgery and Hand Surgery. - : Medical Journals Sweden AB. - 2000-656X .- 2000-6764. ; 50:6, s. 343-348
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Premature craniosynostosis is a congenital disorder causing a skull deformity. For both functional and cosmetic reasons, the deformity is surgically treated with a cranioplasty before the age of 1 year. Temporal hollowing is a common and undesirable remaining deformity after cranioplasty for metopic synostosis. The most common method to determine the degree of temporal hollowing is subjective judgement of the temporal region. The aim of the present project was to develop a quantitative semi-automatic computer tool for objective measurement of bony temporal hollowing. Methods: Using MATLAB, a tool was developed to segment computed tomography images, defining the outermost contour. The images were dorsally limited to the widest point of the head. In each case, a sex-and age-matched control was identified and the contours compared. The bony temporal hollowing of the cases was calculated. Results: The intra-user coefficient of variation (CV) was 5.0% (95% CI = 4.2%-6.2%) and the inter-user CV was 3.0% (95% CI = 2.1%-8.6%). For clinical testing purposes, the tool was used in 14 patients, seven of whom had been operated on with a spring-assisted cranioplasty and seven with a cranioplasty using a bone graft. Conclusions: In summary, this study presents a new tool for objective measurement of the surgical result after cranioplasty for metopic synostosis.
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| 17. |
- Hallen, T., et al.
(författare)
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Cranioplasty without Periosteal Dissection Reduces Blood Loss in Pi-Plasty Surgery for Sagittal Synostosis
- 2017
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Ingår i: Pediatric Neurosurgery. - : S. Karger AG. - 1016-2291 .- 1423-0305. ; 52:4, s. 284-287
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Tidskriftsartikel (refereegranskat)abstract
- Background/Aims: Cranioplasty is often accompanied by a substantial oozing bleeding from the bone surface and bone edges. Our aim was to measure if strict subgaleal dissection without any periosteal release reduces blood loss during pi-plasty surgery for sagittal synostosis. Method: A group of 32 children who underwent pi-plasty surgery at the Sahlgrenska University Hospital between 2010 and 2014 for premature sagittal synostosis with traditional subgaleal dissection combined with incision and release of the periosteum adjacent to the osteotomy lines was compared to a group of 7 children who underwent pi-plasty with strict subgaleal dissection and osteotomy through the bone with the periosteum attached. Information about blood loss and body weight was extracted from medical records. Results: The blood loss in the group of 7 children with strict subgaleal dissection was 102 +/- 86 mL (mean +/- SD) (10 +/- 7 mL/kg) compared to 320 +/- 119 mL (32 +/- 12 mL/kg) in the control group with traditional periosteal release (p < 0.001). Conclusion: Intact periosteum at the osteotomy lines significantly reduces blood loss in pi-plasty surgery for sagittal synostosis. The mechanism is likely because of preserved veins between the bone surface and periosteum.
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| 18. |
- Jansson, Svante, 1948, et al.
(författare)
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Treatment of bilateral pheochromocytoma and adrenal medullary hyperplasia.
- 2006
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Ingår i: Annals of the New York Academy of Sciences. - : Wiley. - 0077-8923. ; 1073, s. 429-35
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Tidskriftsartikel (refereegranskat)abstract
- The risk for bilateral tumors and long-term outcome after conservative cortical-sparing adrenal surgery was studied in a consecutive single-center series. One hundred fifty-four patients were operated on (1950-2004) for pheochromocytoma (PC=137), or abdominal paraganglioma (PG=17). Twenty had MEN 2 (16 MEN 2A; 4 MEN 2B), 15 von Recklinghausen's disease (VRD), and 1 von Hippel-Lindau (VHL) disease. Twelve patients had, or developed, bilateral adrenal medullary tumors; four with MEN 2A, four with MEN 2B, three with VRD, and one with probably hereditary PC associated with brain tumors/meningioma. Two patients with MEN 2B and one with MEN 2A with had bilateral adrenalectomy (adx). Three VRD patients, two MEN 2B and one MEN 2A patients had cortical-sparing surgery. Two patients were operated on unilaterally, but developed small contralateral tumors; one of these (MEN 2A) had a second asymptomatic PC diagnosed at an older age, so surgery was withheld; the other patient (hereditary PC syndrome) had a small contralateral PC diagnosed at autopsy 9 years later. Only three of nine patients with bilateral operations needed corticosteroid replacement after surgery. Four of six patients died of associated tumors (MTC and meningioma). The mean follow-up was 13 (1-25) years. Twelve MEN 2A patients with unilateral adx have been followed up for 20 (4-36) years without developing a second PC. Cortical-sparing adrenal surgery can safely be performed in the majority of patients with bilateral PC. On the basis of our long-term experience of MEN 2A we perform contralateral adrenal resection only if a second PC is confirmed. Five patients underwent adrenal exploration because of clinical and biochemical findings compatible with PC. Four had asymmetrical positive MIBG scans. They all underwent unilateral adx and diffuse medullary hyperplasia was confirmed (medullary weight estimated morphometrically to 1.0-3.4 g vs. normal weight 0.3-0.5 g in matched controls). These patients have been followed for 19 (5-27) years with normal clinical and biochemical findings. In this rare condition removal of the largest adrenal seems adequate.
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| 19. |
- Kljajić, Marizela, et al.
(författare)
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Children Treated for Nonsyndromic Craniosynostosis Exhibit Average Adaptive Behavior Skills with Only Minor Shortcomings
- 2021
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Ingår i: Plastic and reconstructive surgery. - 1529-4242. ; 147:2, s. 453-464
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Tidskriftsartikel (refereegranskat)abstract
- Copyright © 2021 by the American Society of Plastic Surgeons. BACKGROUND: Adaptive behavior skills are important when assessing cognitive functions related to daily life; however, few studies have assessed these skills in patients treated for nonsyndromic craniosynostosis. In this study, the authors assessed the adaptive behavior skills of children treated for craniosynostosis and examined whether their outcomes are related to surgical technique. METHODS: The Adaptive Behavior Assessment System, 2nd Edition, parent report was used for children (age, 7 to 16 years) treated for sagittal (n = 41), metopic (n = 24), and other rare synostoses (n = 8). Background data, including intelligence quotient, were controlled for confounders. RESULTS: All evaluated children treated for craniosynostosis were estimated as lower in all aspects of adaptive behavior skills (full-scale, conceptual, social, and practical composites; effect size, 0.36 to 0.44) as compared with norms. The sagittal group showed shortcomings in social composite (effect size, 0.48) and subscales measuring self-care and self-direction, although no difference was observed between spring-assisted surgery and pi-plasty regarding outcomes of adaptive behavior skills. In addition, children treated for metopic synostosis showed results indicating shortcomings with adaptive behavior according to the full-scale, conceptual, and social composites (effect size, 0.53 to 0.61) relative to norms. Furthermore, attrition analysis revealed no significant differences between responders (rate, 80.2 percent) and nonresponders. CONCLUSION: These results found that children treated for craniosynostosis display average adaptive behavior skills, and that the two surgical techniques used to treat sagittal synostosis did not differ in their behavioral outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.
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| 20. |
- Kljajić, Marizela, et al.
(författare)
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Sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis.
- 2020
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Ingår i: Child neuropsychology : a journal on normal and abnormal development in childhood and adolescence. - : Informa UK Limited. - 1744-4136. ; 26:4, s. 475-488
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Tidskriftsartikel (refereegranskat)abstract
- Attention problems are common in patients with craniosynostosis. Craniosynostosis is a rare condition, studies face challenges of selection bias, small sample sizes, and wide age ranges. The aim of the study was to assess the sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis. To reduce selection bias, we included children that had previously undergone surgery for craniosynostosis, were between 8 and 16years, and lived close to the craniofacial centre. The Connors Continuous Performance Test (3rd edition) was used to measure sustained attention and vigilance (n =61; response rate: 76.3%). Attrition analysis revealed no differences between responding and non-responding groups regarding background variables. One identified difference between the SS (n =28) and MS (n =23) groups involved significantly better performance by the SS group in the hit-reaction time (HRT) test relative to the MS group (p<0.05). Compared with the norms, the SS group showed significantly worse response style, detectability, omissions, commissions, perseverations, HRT response speed (HRT-SD), HRT inter-stimulus interval change (HRT-iC) (p<0.01 for all), and variability (p<0.05). The MS group showed significantly worse detectability, HRT-SD, variability (p<0.01 for all), commissions, perseverations and HRT-iC (p<0.05 for all) as compared with norms. No differences regarding attention was detected for the two different surgical techniques used for correction of sagittal synostosis. There were shortcomings in sustained attention and vigilance as compared with the norms in the SS and MS groups, although the deviations were small.
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| 21. |
- Kljajić, Marizela, et al.
(författare)
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The Cognitive Profile of Children with Non-syndromic Craniosynostosis
- 2019
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Ingår i: Plastic and reconstructive surgery. - 1529-4242. ; 143:5
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Tidskriftsartikel (refereegranskat)abstract
- Long-term neuropsychological and cognitive outcomes in patients with non-syndromic craniosynostosis have proven difficult to evaluate objectively due to methodological problems with published studies based on their small and biased samples of patients, wide age ranges, and testing with unacceptable psychometric properties. This study evaluated full-scale intelligence quotient (FSIQ) and its subscales in a cohort with a small selection bias.Patients (aged 7-16 years) born with non-syndromic craniosynostosis and surgically treated were tested using the Wechsler Intelligence Scale for Children, Fourth Edition (WISC-IV). Ninety-one patients were invited, and 73 patients were tested.There was no difference in FSIQ between patients having undergone operations for sagittal synostosis or metopic synostosis and norms provided by the test. Patients operated on for sagittal synostosis showed a significantly higher perceptual reasoning IQ, but also significantly lower working-memory IQ and processing-speed IQ as compared with the norms. Patients operated on for metopic synostosis showed no differences in any IQ index as compared with the norm. Additionally, attrition analysis showed no differences in background factors between responders and non-responders.These results derived from a group of patients with uniform age range, and tested using an established tool revealed that non-syndromic children having undergone surgery for craniosynostosis exhibited average intellectual ability. However, the analysis indicated possible issues with working memory and processing speed in patients operated on for sagittal synostosis, highlighting impairments potentially associated with neuropsychological problems and that might contribute to learning disabilities.
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| 22. |
- Knudsen, Elisabet, 1944, et al.
(författare)
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Parental estimation of early psychological development in children operated on for single suture synostosis.
- 2012
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Ingår i: Journal of plastic surgery and hand surgery. - 2000-6764. ; 46:3-4, s. 152-154
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Tidskriftsartikel (refereegranskat)abstract
- Abstract Impaired psychological development in children with single suture craniosynostosis is often described in the literature. The authors' own experience was that these children appeared to have normal psychological development during their first years of life. The aim of the present study was, therefore, to evaluate if parental estimation would reveal any shortcomings in psychological development. All consecutive patients operated on for non-syndromal single suture synostosis between October 2002 and June 2006 were included (n = 66). A questionnaire was filled out by the parents when the child was 3 years old. The questions concerned development of speech, motor control, personal abilities, and emotional development. The results were compared with randomly selected controls of the same age (n = 180) whose parents answered the same questionnaire. The results showed that children operated on for single suture synostosis did not show any signs of lasting disadvantages due to craniofacial surgery. The children did not in any respect suffer from a hampered psychological development up to 3 years of age. In summary the psychological development of patients operated on for single suture synostosis seems to be normal up to 3 years of age as far as parental estimation can reveal.
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| 23. |
- Kölby, Lars, 1963, et al.
(författare)
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Somatostatin receptor subtypes, octreotide scintigraphy, and clinical response to octreotide treatment in patients with neuroendocrine tumors.
- 1998
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Ingår i: World journal of surgery. - 0364-2313. ; 22:7, s. 679-83
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Tidskriftsartikel (refereegranskat)abstract
- Several types of neuroendocrine tumor express high numbers of somatostatin receptors (sstr). We have compared the expression of sstr subtypes with the outcome of octreotide scintigraphy in patients with carcinoids and medullary thyroid carcinoma (MTC) in comparison with Hürthle cell tumors. The effect of sstr activation (octreotide treatment) on tumor markers was also studied in patients with disseminated carcinoid tumors. Six patients with carcinoid tumors (four midgut and two foregut), and three patients with thyroid tumors (one MTC, one Hürthle cell carcinoma, and one Hürthle cell adenoma) were studied. Octreotide scintigraphy visualized tumor sites in all nine patients. Macroscopic tumor was verified at these sites at subsequent surgical exploration. Using Northern blotting and subtype-specific riboprobes, sstr could be detected in all tumors examined. All five sstr subtypes were detected in most of the carcinoid tumors. All six carcinoids expressed sstr2. This was in contrast to the findings for the thyroid tumors analyzed, which also expressed several sstr subtypes but in some cases lacked expression of sstr2. This was also the case for normal thyroid tissue. Clinically, octreotide treatment of the patients with midgut carcinoid tumors resulted in palliation of hormonal symptoms accompanied by a significant reduction of urinary 5-HIAA levels (28-71%). These results indicate that carcinoid tumors frequently express all five sstr subtypes. The thyroid tumors also expressed multiple sstr but could lack expression of sstr2. Nevertheless, these tumors were visualized by octreotide scintigraphy, indicating that sstr2 expression is not a prerequisite for tumor imaging.
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| 24. |
- Malmqvist, Carina, et al.
(författare)
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Simplified Postoperative Care After Spring-assisted Strip Craniotomy for Sagittal Synostosis: A Prospective Before-and-After Study.
- 2021
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Ingår i: The Journal of craniofacial surgery. - 1536-3732. ; 32:4, s. 1507-1510
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Tidskriftsartikel (refereegranskat)abstract
- Since the development of spring-assisted techniques for corrective craniofacial surgery, routine postoperative admission to intensive care units (ICUs) has been questioned. However, close monitoring is necessary if the continuous infusion of morphine is used as recommended for better pain relief. In this study, the authors evaluated a simplified postoperative protocol without continuous morphine infusion and no indwelling urinary catheter following spring-assisted surgery (SAS) for sagittal synostosis. Ten children were cared for according to a standard protocol with postoperative intravenous (i.v.) infusion of morphine and an indwelling urinary catheter, and 11 consecutive children were treated according to a simplified protocol with pain relief based on intermittent injections of morphine and clonidine [according to Face, Legs, Activity, Cry, Consolability (FLACC) scores >4] without the indwelling catheter. A Mann-Whitney U test was used for comparison of distributions between the two groups. The results revealed no differences between groups regarding the proportion of FLACC scores >4, total amount of administered i.v. morphine and clonidine, total volume of buffered glucose infused, time to first feeding on breast milk or substitute, or the length of stay. Despite the inherent limitations of our small observational study, the authors concluded that at our institution, it was possible to exclude a standard continuous i.v. infusion of morphine and an indwelling urinary catheter from our postoperative care protocol without decreasing the quality of pain relief in children submitted to SAS for sagittal synostosis. This finding supports downgrading the level of care from the ICU to a regular ward after limited immediate postoperative observation.
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| 25. |
- Maltese, Giovanni, 1974, et al.
(författare)
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Correction of hypotelorism in isolated metopic synostosis.
- 2014
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Ingår i: Journal of plastic surgery and hand surgery. - 2000-6764. ; 48:1, s. 63-66
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Tidskriftsartikel (refereegranskat)abstract
- Abstract Surgical correction of trigonocephaly is performed by fronto-orbital reshaping and advancement. Some authors supplement the fronto-orbital remodelling with direct surgical correction of the hypotelorism, but the role of this step of the procedure has been questioned. At Sahlgrenska University Hospital, hypotelorism in metopic synostosis is treated with spring-assisted surgery (S) in children below 6 months of age and with fronto-orbital advancement with the interposition of a bone graft (BG) in the fronto-orbital region at higher age. The aim of the present study was to evaluate the anterior bony interorbital distance (BIOD) preoperatively and at follow-up in patients operated on with the two techniques and to compare the results with adequate control groups. Preoperatively, the patients affected by metopic synostosis had a significantly reduced BIOD compared to their respective controls (S group: 13.8 ± 1.6 (mean ± SD) mm vs 18.6 ± 1.4 mm, p < 0.001, BG group: 14.7 ± 1.0 mm vs 18.8 ± 1.4 mm, p < 0.001). At 3 years follow-up, BIOD was improved in both groups, but only in the S group the mean BIOD did not differ from the control group (19.7 ± 2.9 mm vs 20.2 mm ± 1.3 mm, p = 0.3). In the BG group the BIOD was still significantly different between patients and controls (17.6 ± 1.8 vs 20.0 ± 1.1 mm, p < 0.001). Spring-assisted surgery performed before 6 months of age can normalise hypotelorism in metopic synostosis. Full correction in a large series of patients has now been achieved for the first time.
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