Leon N.A. van Adrichem

The increase of metopic synostosis: A pan-European observation

Metopic synostosis is thought to have an incidence of about 1 in 15,000 births. Traditionally, this makes it the third most frequent single-suture craniosynostosis, after scaphocephaly (1 in 4200-8500) and plagiocephaly (1 in 11,000). Our units have, independently from each other, noted a marked increase in the number of metopic synostosis over the recent years. This is a pan-European, retrospective epidemiological study on the number of cases with metopic synostosis born between January 1, 1997, and January 1, 2006. This number was compared to the prevalence of scaphocephaly, the most frequently seen craniosynostosis. In the 7 units, a total of 3240 craniosynostosis were seen from 1997 until 2006. Forty-one percent (n = 1344) of those were sagittal synostosis, and 23% (n = 756) were metopic synostosis. There was a significant increase of the absolute number as well as of the percentage of metopic synostosis over these years (regression analysis, P = 0.017, R2 = 0.578) as opposed to a nonsignificant increase in the percentage of sagittal synostosis (P > 0.05, R2 = 0.368). The most remarkable increase occurred around 2000-2001, with the average of metopics being 20.1% from 1997 to 2000 and 25.5% from 2001 to 2005 (independent t-test, P = 0.002). The sagittal synostosis showed a smaller and nonsignificant increase in the same years: from 39.9% in 1997-2000 leading up to 42.5% in 2001-2005 (independent t-test, P > 0.05). The number of metopic synostosis has significantly increased over the reviewed period in all of our units, both in absolute numbers as in comparison to the total number of craniosynostosis.

The acute effect of cigarette smoking on the microcirculation of a replanted digit

Thirty-one patients who had undergone digital replantation or revascularization volunteered to participate in a study of the acute effect of smoking on the microcirculation of the skin of replanted fingers. Fourteen were smokers and 17 were nonsmokers at the time of the study. Blood flow was assessed by means of the laser Doppler flowmeter under standard conditions. Each smoker inhaled 2 cigarettes. During smoking of the first and second cigarettes a mean decrease in laser Doppler flow of 8% and 19%, respectively, was found, whereas the nonsmokers showed a slight increase of 4% and 4%, respectively. Ten minutes after the last cigarette almost no recovery could be detected. The negative effect of smoking on the microcirculation in replanted digits proved to be more pronounced in the patients operated on more recently. This experiment confirms that smoking after replantation surgery should be prohibited to guarantee optimal circulation.

The effect of cigarette smoking on the survival of free vascularized and pedicled epigastric flaps in the rat

&NA; Microsurgeons suspect that cigarette smoking reduces the survival of free vascularized flaps and replantations, but this has never been proven. This experimental study investigates the effect of smoking on free‐flap survival. A fasciocutaneous epigastric flap was used in 30 rats as a free flap and in 30 rats as a pedicled flap. Of each group, 10 rats were smoked 6 weeks before and 2 weeks after surgery, 10 rats were smoked only 6 weeks before surgery, and 10 rats underwent the sham smoking procedure. Also, a distally based dorsal skin flap was cut in all rats, representing a random vascularized flap. Vitality and size of both flaps and patency of the vascular anastomoses were assessed 14 days after surgery. The epigastric flaps were monitored by laser Doppler flowmetry and thermometry during the experiment. Survival of the free vascularized epigastric flaps was significantly lower in smoking rats. All pedicled flaps except one survived. The epigastric flaps only necrosed or survived completely, exactly correlating to the patency of the vascular anastomoses. The mean surviving area of the dorsal flaps was best for nonsmoking rats, worse for only preoperatively smoking rats, and worst for preoperatively and postoperatively smoking rats. The differences were statistically significant. Postoperative laser Doppler flow differed significantly between surviving and dying flaps, affirming the value of laser Doppler flow monitoring in microvascular surgery. In conclusion, this study proves that smoking of cigarettes is detrimental to the survival of free vascularized flaps. (Plast. Reconstr. Surg. 97: 86, 1996.)

Diagnostic strategies for the evaluation of asymmetry in infancy - a review

Asymmetry in infancy is a diagnosis with a large spectrum of features, expressing an abnormal shape of parts of the body or unequal postures and movements. Symptoms may be structural and/or functional, with localised or generalised expression. Within the last decade, many professionals have focused on the adverse consequences on motor performance of infants, associated with the recommendations on the prevention of sudden infant death. The purpose of the present study was to highlight different aspects of asymmetry in infancy based on best available evidence in the current literature and to present a flow chart illustrating different diagnostic pathways of asymmetry in infancy. Conclusion:we conclude that asymmetry in infancy is a diagnosis with a large spectrum of features and a multifactorial aetiology without consensus in definition, nomenclature and classification. Systematic diagnostic management of asymmetry in infancy is indicated. The presented diagnostic flow chart might serve as a basis.

Imatinib mesylate for children with dermatofibrosarcoma protuberans (DFSP).

Dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor in children. DFSP is characterized by a specific fusion of the platelet‐derived growth factor β (PDGFβ) with the collagen type 1α1 (COL1α1) gene which renders these tumors responsive to targeted therapy with tyrosine kinase inhibitors, such as imatinib mesylate, as is reported in adults. In the current report, we describe the first small pediatric DFSP series, in which response to imatinib mesylate contributed to successful treatment outcome. Pediatr Blood Cancer. 2010;55:369–373.

Bitemporal depressions after cranioplasty for trigonocephaly: a long-term evaluation of (supra) orbital growth in 92 patients.

Long-term results after cranioplasty for trigonocephaly often show bitemporal depressions and a residual hypotelorism. Both findings fuel the perception that the growth of the periorbital region and the forehead as a whole continues to be restricted, even after correction. The aim of this study is to evaluate the growth process of the periorbital region after correction for trigonocephaly in the long term. From 1986 to 2004, 123 patients underwent a cranioplasty for the correction of trigonocephaly. Cephalometric analysis was performed on the radiographs taken at presentation and on the last available radiograph before the age of 6 years (92 posteroanterior and 93 lateral cephalograms). Cephalic landmarks were used to analyze the growth of the forehead: Mo (medial orbital wall), Lo (lateral orbital wall), Losp (crosspoint between lateral orbital wall and sphenoid), and Eu (most lateral point of the skull). As a result of the lack of standardized cephalograms, growth ratios were used instead of absolute numbers. The Eu-Eu growth rate was higher than the Lo-Lo rate, which in its turn surpassed the Losp-Losp rate. An initial undercorrection of the hypotelorism was noted followed by an increased limited autocorrection. A higher Mo-Mo growth rate was noted in the group operated after 1 year of age. Increased interorbital growth accounts for an autocorrection of the residual hypotelorism. The growth rate of the anterotemporal area (Losp) was shown to be the lowest, which could explain the bitemporal depressions so often seen after a frontosupraorbital cranioplasty.

Comparison of laser Doppler flowmetry and thermometry in the postoperative monitoring of replantations

Reliable postoperative monitoring in microvascular surgery is necessary to improve the success rate of reexplorations following vascular compromise. Surface thermometry is known as an easy and inexpensive objective postoperative monitor and therefore is used by many microsurgeons. Reliability, however, is not satisfactory, and therefore several other instrumental methods have been tested of which laser Doppler flowmetry shows the most promising results. This study compared laser Doppler flowmetry to thermometry in the postoperative monitoring after replantation surgery. In 34 patients, 45 replantations and revascularizations were monitored by laser Doppler flowmetry and thermometry. A reliable alarm value of 10 PU was defined for replantations and revascularizations, with a sensitivity of 93% and a specificity of 94%. Thermometry showed a sensitivity of 84% and a specificity of 86% at 29 degrees C.

3D surface imaging in medicine: A review of working principles and implications for imaging the unsedated child

This article provides an overview of the methods used for optical surface imaging during the last 30 years, with the primary focus on the imaging of the unsedated child. The goal is to provide the reader with an overview of the working methods behind the published articles. This will enable the reader to better interpret current data and decide if a certain approach is suitable for their particular research question.

Reducing glucose infusion safely prevents hyperglycemia in post-surgical children.

BACKGROUND & AIMS To investigate the effects of two different glucose infusions on glucose homeostasis and amino acid metabolism in post-surgical children. METHODS This randomized crossover study evaluated glucose and amino acid metabolism in eight children (age 9.8 ± 1.9 months, weight 9.5 ± 1.1 kg) admitted to a pediatric intensive care unit in a tertiary university hospital after surgical correction for non-syndromal craniosynostosis. Patients were randomized to receive low (LG; 2.5 mg kg(-1) min(-1)) and standard (SG; 5.0 mg kg(-1) min(-1)) glucose infusion in a crossover setting. After a bolus (4 g kg(-1)) of deuterium oxide, we conducted a primed, constant, 8 h tracer infusion with [6,6-²H₂]Glucose, [1-¹³C]Leucine, [ring-²H₅]Phenylalanine and [3,3-²H₂]Tyrosine. RESULTS SG resulted in hyperglycemia (defined as > 6.1 mmol L(-1)), while during LG plasma glucose levels were normoglycemic (5.9 ± 0.6 vs. 7.5 ± 1.7 mmol L(-1); LG vs. SG respectively, p = 0.02). Hypoglycemia did not occur during LG infusion. Endogenous glucose production was not fully suppressed during the hyperglycemic state under SG and increased with reduced glucose infusion (2.6 ± 1.5 vs. 1.1 ± 1.4 mg kg(-1) min(-1); LG vs. SG; p = 0.05). Whole body protein balance derived from leucine and phenylalanine kinetics was slightly negative but not further affected with a decrease in glucose infusion. CONCLUSIONS The current recommended glucose infusion induces hyperglycemia in post-surgical children. A reduced glucose infusion safely reduced high glucose levels, while children were capable to sustain normoglycemia with increased endogenous glucose production. The reduced glucose infusion did not exacerbate the mild catabolic state in which the patients were.

Craniofacial Stability in Patients With Crouzon or Apert Syndrome After Le Fort III Distraction Osteogenesis

Objective Le Fort III osteotomy with distraction osteogenesis (DO) is used to improve the retruded midface in patients with Crouzon or Apert syndrome. This study aimed to evaluate sagittal and vertical preoperative and postoperative cephalometric changes of DO of the midface in patients with Crouzon or Apert syndrome. Design Population-based case-control study. Patients and Methods Records of patients with the syndrome of Crouzon (N = 6) or Apert (N = 7) were compared, before and after Le Fort III DO, with a nonsyndromic untreated control group (N = 486). Main Outcome Measures Sagittal and vertical cephalometric maxillary landmarks and measurements were used to predict and measure midface advancement and rotation after Le Fort III DO. Cephalograms were taken before surgery (T0), 4 months after surgery at removal of the distraction device (T1), and 1 year after removal of the distraction device (T2). Analysis Z scores were performed to compare cephalometric measures of syndromic patients with control subjects. Results Cephalograms of 13 patients with Crouzon syndrome (N = 6) or Apert (N = 7) (age range 8.2 to 19.8 years) were evaluated. Treatment changes (T1-T2) showed statistically significant maxillary advancement, with no significant differences between the patients with the Crouzon or Apert syndrome. Conclusions DO of the midface in patients with Crouzon or Apert syndrome seems to be stable in the sagittal direction after follow-up. Although Crouzon and Apert differ after DO, anteroposterior craniofacial dimensions were significantly improved and were closer to patterns of normal subjects.