Speech correcting surgery after primary palatoplasty: a systematic literature review and meta-analysis.

OBJECTIVES: In cleft palate patients, the soft palate is commonly closed using straight-line palatoplasty, Z-palatoplasty, or palatoplasty with buccal flaps. Currently, it is unknown which surgical technique is superior regarding speech outcomes. The aim of this review is to study the incidence of speech correcting surgery (SCS) per soft palatoplasty technique and to identify variables which are associated with this outcome. MATERIALS AND METHODS: A systematic literature search was carried out according to the PRISMA guidelines. Inclusion and exclusion criteria were applied to focus on the incidence of SCS after soft palatoplasty. Additional variables like surgical modification, cleft morphology, syndrome, age at palatoplasty, fistula and assessment of velopharyngeal function were reported. A modified New-Ottawa Scale (NOS) was used for quality appraisal. Pooled estimates from the meta-analysis were calculated using a random-effects model. RESULTS: One thousand twenty-nine studies were found of which 54 were included in the analysis. The pooled estimate proportion of SCS after straight-line palatoplasty was 19% (95% CI 15-24), after Z-palatoplasty 6% (95% CI 4-9), and after palatoplasty with buccal flaps 7% (95% CI 4-11). CONCLUSIONS: A lower SCS rate was found in patients receiving Z-palatoplasty when compared to straight-line palatoplasty. We propose a minimum set of outcome parameters which ideally should be included in future studies regarding speech outcomes after cleft palate repair. CLINICAL RELEVANCE: Current literature reports highly heterogenous data regarding cleft palate repair. Our recommended set of parameters may address this inconsistency and could make intercenter comparison possible and of better quality.

Bulging after DIEP Breast Reconstruction: New Insights Concerning Rectus Diastasis and Medial Perforator Harvest.

The aim of this study was to evaluate the association between flap harvest technique and occurrence of abdominal bulging. Methods: A retrospective analysis of 159 patients undergoing DIEP flap breast reconstruction between 2014 and 2021 in the University Medical Center Utrecht was conducted. Outcomes measured were preoperative rectus diastasis, flap weight, laterality of flap harvest (unilateral or bilateral), timing of the harvest (immediate or delayed), number of perforators harvested (single or multiple), and location of the harvested perforator (medial, lateral, or both). Results: In 159 patients, 244 DIEP flaps were performed, 16 of these donor-sites (6.6%) developed a clinically evident abdominal bulge. When preoperative rectus abdominis diastasis was found (n = 97), postoperative bulging occurred significantly more often (P < 0.01). Patients in whom the medial perforator artery was harvested for reconstruction (n = 114) showed less abdominal bulging than patients in whom the lateral (n = 92) was harvested (P = 0.02). Using single versus multiple perforators for the DIEP flap, bilateral versus unilateral reconstruction or timing of the operation showed no significant difference in outcome of bulging (P = 1.00, P = 0.78, P = 0.59, respectively). Conclusions: The incidence of bulging in our study cohort is comparable to the literature. Harvesting the medial perforator artery for the DIEP flap showed less abdominal bulging than using the lateral perforator artery in a DIEP flap breast reconstruction. Also, preoperative rectus diastasis was found to be an important risk factor for the occurrence of bulging.

Reconstructive Strategies in Pediatric Patients after Oncological Chest Wall Resection: A Systematic Review.

An appropriate reconstruction strategy after surgical resection of chest wall tumors in children is important to optimize outcomes, but there is no consensus on the ideal approach. The aim of this study was to provide an up-to-date systematic review of the literature for different reconstruction strategies for chest wall defects in patients less than 18 years old. A systematic literature search of the complete available literature was performed and results were analyzed. A total of 22 articles were included in the analysis, which described a total of 130 chest wall reconstructions. All were retrospective analyses, including eight case reports. Reconstructive options were divided into primary closure (n = 21 [16.2%]), use of nonautologous materials (n = 83 [63.8%]), autologous tissue repair (n = 2 [1.5%]), or a combination of the latter two (n = 24 [18.5%]). Quality of evidence was poor, and the results mostly heterogeneous. Reconstruction of chest wall defects can be divided into four major categories, with each category including its own advantages and disadvantages. There is a need for higher quality evidence and guidelines, to be able to report uniformly on treatment outcomes and assess the appropriate reconstruction strategy.

Assessment of Health-Related Quality of Life in Robin Sequence: A Comparison of Mandibular Distraction Osteogenesis and Tongue-Lip Adhesion.

BACKGROUND: Numerous studies have proven the efficacy of mandibular distraction osteogenesis or tongue-lip adhesion in Robin sequence infants with upper airway obstruction. However, none has compared health-related quality of life outcomes. METHODS: In the present retrospective study, Robin sequence infants younger than 1 year, who underwent mandibular distraction osteogenesis or tongue-lip adhesion, were included (2006 to 2016). The infants' caregivers were asked to complete a questionnaire based on the Glasgow Children's Benefit Inventory. RESULTS: The response rate was 71 percent (22 of the 31 questionnaires; mandibular distraction osteogenesis, 12 of 15; and tongue-lip adhesion, 10 of 16) and median age at surgery was 24 days (range, 5 to 131 days). Median total Glasgow Children's Benefit Inventory scores after mandibular distraction osteogenesis and after tongue-lip adhesion were 21.9 (interquartile range, 9.4) and 26.0 (interquartile range, 37.5), respectively (p = 0.716), indicating an overall benefit from both procedures. Positive changes were observed in all subgroups emotion, physical health, learning, and vitality. In syndromic Robin sequence, both procedures demonstrated a lower positive change in health-related quality of life compared with isolated Robin sequence (p = 0.303). CONCLUSIONS: Both surgical procedures demonstrated an overall benefit in health-related quality-of-life outcomes, with no significant differences. The authors' findings contribute to the debate regarding the use of mandibular distraction osteogenesis versus tongue-lip adhesion in the surgical treatment of Robin sequence; however, studies evaluating health-related quality of life in larger Robin sequence cohorts are necessary to identify which procedure is likely to be best in each individual Robin sequence infant. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

The ontogeny of Robin sequence.

The triad of micrognathia, glossoptosis, and concomitant airway obstruction defined as "Robin sequence" (RS) is caused by oropharyngeal developmental events constrained by a reduced stomadeal space. This sequence of abnormal embryonic development also results in an anatomical configuration that might predispose the fetus to a cleft palate. RS is heterogeneous and many different etiologies have been described including syndromic, RS-plus, and isolated forms. For an optimal diagnosis, subsequent treatment and prognosis, a thorough understanding of the embryology and pathogenesis is necessary. This manuscript provides an update about our current understanding of the development of the mandible, tongue, and palate and possible mechanisms involved in the development of RS. Additionally, we provide the reader with an up-to-date summary of the different etiologies of this phenotype and link this to the embryologic, developmental, and genetic mechanisms.

Growth and prevalence of feeding difficulties in children with Robin sequence: a retrospective cohort study.

OBJECTIVES: In addition to breathing problems, patients with Robin sequence (RS) often encounter feeding difficulties (FD). Data regarding the occurrence of FD and possible influencing factors are scarce. The study aim was to elucidate these factors to improve treatment strategies. MATERIAL AND METHODS: A retrospective comparative cohort study was conducted, consisting of 69 infants diagnosed with both RS and a cleft palate and 64 isolated cleft palate only (iCPO) infants. Data regarding FD, growth, and airway intervention were collected during the first 2 years of life. A systematic review of the literature was conducted to identify reported FD in RS patients. RESULTS: RS patients had more FD (91 %) than iCPO patients (72 %; p = 0.004). Also, nasogastric (NG)-tube feeding was necessary more frequently and for a longer period (both p < 0.001). Growth was lower in RS than iCPO infants (p = 0.008) and was not affected by the kind of airway management (conservative/surgical; p = 0.178), cleft palate grade (p = 0.308), or associated disorders (p = 0.785). By contrast, surgical intervention subtype did significantly affect growth. Mean reported FD for RS in the literature is 80 % (range = 47-100 %), and 55 % (range = 11-100 %) of infants need NG-tube feeding. CONCLUSIONS: FD is present in a large proportion of infants with RS, which indicates the need for early recognition and proper treatment to ensure optimal growth. Growth during the first 2 years of life is significantly lower in RS patients than iCPO patients, which indicates the need for careful attention and long-term follow-up. CLINICAL RELEVANCE: This study indicates the need for early recognition and proper treatment of FD in RS to ensure optimal growth. In addition, growth needs careful attention and long-term follow-up.

Health-related quality of life in children with Robin sequence.

This patient-reported outcome (PRO) study reports on 102 children with Robin sequence (RS) and their parents. There has been differentiated between those with isolated RS and those with RS as part of a syndrome, and take various treatments into account. All RS families from an earlier described cohort were invited to participate. Parents and RS children completed online questionnaires regarding health-related quality of life (HRQoL), satisfaction with appearance, parental distress, and RS specific topics. Results were compared with the Dutch norm population if available. There was no major difference in HRQoL in RS children and the Dutch norm population, nor between children with isolated RS and those with RS as part of a syndrome. The latter is likely due to the large percentage of children with Stickler syndrome, and small number of RS children with intellectual disability. Parental distress was higher in RS children with syndromes compared to parents of isolated RS children. When comparing various treatments, the subgroup treated by mandibular distraction showed a tendency of lower HRQoL scores, less satisfaction with appearance, and more parental distress. Also in the NPA group parents showed a tendency of more parental distress. Subgroups for each treatment were very small, however, and firm conclusions cannot be drawn. In this study, HRQoL in RS children is demonstrated comparable to the norm population, despite variations in treatment, possibly with less favorable outcome for children who received mandibular distraction. Markedly larger studies are needed to allow more reliable comparison of PROs in various treatments, and to incorporate PROs in management guidelines to obtain optimal patient care. © 2016 Wiley Periodicals, Inc.

Best Practices for the Diagnosis and Evaluation of Infants With Robin Sequence: A Clinical Consensus Report.

IMPORTANCE: Robin sequence (RS) is a congenital condition characterized by micrognathia, glossoptosis, and upper airway obstruction. Currently, no consensus exists regarding the diagnosis and evaluation of children with RS. An international, multidisciplinary consensus group was formed to begin to overcome this limitation. OBJECTIVE: To report a consensus-derived set of best practices for the diagnosis and evaluation of infants with RS as a starting point for defining standards and management. EVIDENCE REVIEW: Based on a literature review and expert opinion, a clinical consensus report was generated. FINDINGS: Because RS can occur as an isolated condition or as part of a syndrome or multiple-anomaly disorder, the diagnostic process for each newborn may differ. Micrognathia is hypothesized as the initiating event, but the diagnosis of micrognathia is subjective. Glossoptosis and upper airway compromise complete the primary characteristics of RS. It can be difficult to judge the severity of tongue base airway obstruction, and the possibility of multilevel obstruction exists. The initial assessment of the clinical features and severity of respiratory distress is important and has practical implications. Signs of upper airway obstruction can be intermittent and are more likely to be present when the infant is asleep. Therefore, sleep studies are recommended. Feeding problems are common and may be exacerbated by the presence of a cleft palate. The clinical features and their severity can vary widely and ultimately dictate the required investigations and treatments. CONCLUSIONS AND RELEVANCE: Agreed-on recommendations for the initial evaluation of RS and clinical descriptors are provided in this consensus report. Researchers and clinicians will ideally use uniform definitions and comparable assessments. Prospective studies and the standard application of validated assessments are needed to build an evidence base guiding standards of care for infants and children with RS.

Long-Term Results of Mandibular Distraction Osteogenesis with a Resorbable Device in Infants with Robin Sequence: Effects on Developing Molars and Mandibular Growth.

BACKGROUND: Mandibular distraction osteogenesis with a unidirectional resorbable device is an effective treatment option for severe upper airway obstruction in infants with Robin sequence. Long-term effects, especially with regard to tooth development and mandibular outgrowth, are not known. METHODS: Robin sequence infants with a follow-up of greater than or equal to 5 years were included. Baseline characteristics were extracted from medical records. Panoramic and lateral cephalometric radiographs were analyzed and patients were recalled for physical examination. RESULTS: Ten infants underwent mandibular distraction osteogenesis at a mean age of 3.7 months (median, 19 months; range, 11 days to 27 months). Mean length of follow-up was 6.8 years (range, 5.0 to 7.9 years). Ten Robin sequence infants without mandibular distraction osteogenesis (mean length of follow-up, 7.4 years; range, 6.7 to 8.9 years) were the controls. Shape anomalies, positional changes, and root malformations of molars were seen significantly more often than in the control group (p = 0.007, p = 0.009, and p = 0.043, respectively). Mandibular length was shorter (p = 0.030), but mandibular ramus height was comparable (p = 0.838) with that of the non-mandibular distraction osteogenesis group. Compared with healthy controls, all Robin sequence infants had a significantly shorter mandible. CONCLUSIONS: Mandibular distraction osteogenesis with a resorbable system reveals overall good short- and long-term results, but the effects on developing molars and mandibular outgrowth likely necessitate secondary procedures. This factor should be considered when deciding on treatment options and counseling of parents. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.

Etiology and pathogenesis of robin sequence in a large Dutch cohort.

Robin sequence (RS) can be defined as the combination of micrognathia and upper airway obstruction/glossoptosis causing neonatal respiratory problems, with or without a cleft palate and either isolated or non-isolated. Pathogenesis varies widely. We hypothesize that optimal treatment depends on pathogenesis and therefore patients should be stratified according to diagnosis. Here, we evaluate diagnoses and (presumed) pathogeneses in an RS cohort. Medical records of all RS patients presenting between 1995-2013 in three academic hospitals were evaluated. Four clinical geneticists re-evaluated all information, including initial diagnosis. Diagnoses were either confirmed, considered uncertain, or rejected. If uncertain or rejected, patients were re-evaluated. Subsequent results were re-discussed and a final conclusion was drawn. We included 191 RS patients. After re-evaluation and changing initial diagnoses in 48 of the 191 patients (25.1%), 37.7% of the cohort had isolated RS, 8.9% a chromosome anomaly, 29.3% a Mendelian disorder, and 24.1% no detectable cause. Twenty-two different Mendelian disorders were diagnosed, of which Stickler syndrome was most frequent. Stratification of diagnoses according to (presumed) pathogenic mechanism in 73 non-isolated patients with reliable diagnoses showed 43.9% to have a connective tissue dysplasia, 5.5% a neuromuscular disorder, 47.9% a multisystem disorder, and 2.7% an unknown mechanism. We diagnosed more non-isolated RS patients compared to other studies. Re-evaluation changed initial diagnosis in a quarter of patients. We suggest standardized re-evaluation of all RS patients. Despite the relatively high diagnostic yield pathogenesis could be determined in only 59.7% (71/119), due to limited insight in pathogenesis in diagnosed entities. Further studies into pathogenesis of entities causing RS are indicated.

Birth prevalence of Robin sequence in the Netherlands from 2000-2010: a retrospective population-based study in a large Dutch cohort and review of the literature.

The birth prevalence of Robin sequence (RS) is frequently cited to be 1 in 8,500 to 14,000 live births (range: 7,1-11,8 per 100.000), which is based on just a few epidemiological studies. The objective of this study is to contribute to the limited knowledge of the epidemiology of RS by determining the frequency of RS in a cleft palate (CP) population and the estimated birth prevalence in live births in the Netherlands, using distinct diagnostic criteria. A retrospective population-based analysis of the National Cleft Registry was performed in order to obtain all CP patients registered in the Netherlands from 2000-2010, in addition to a thorough review of the medical records in three Dutch Academic Pediatric Hospitals for the same period. Furthermore, a systematic search of the literature was conducted to allow for comparison of our findings. The Dutch birth prevalence of RS was estimated to be 1:5,600 live births (or 17.7 per 100,000), with a slight female predominance. RS was estimated to occur in a third of the CP population and patients with RS had a more severe cleft grade than the general CP population. The literature search yielded 42 studies reporting the birth prevalence for RS, which varied between 1:3,900 and 1:122,400 (0.8-32.0 per 100,000), with a mean prevalence of 1:24,500 (8.0 per 100,000). The birth prevalence of RS in the Netherlands was higher than reported for most other countries when similar diagnostic criteria were used, with a slight female predominance. A third of the general CP could be classified as RS.

A pragmatic approach to infants with Robin sequence: a retrospective cohort study and presence of a treatment algorithm.

OBJECTIVES: Initial approaches to and treatments of infants with Robin sequence (RS) is diverse and inconsistent. The care of these sometimes critically ill infants involves many different medical specialties, which can make the decision process complex and difficult. To optimize the care of infants with RS, we present our institution's approach and a review of the current literature. MATERIAL AND METHODS: A retrospective cohort study was conducted among 75 infants diagnosed with RS and managed at our institution in the 1996-2012 period. Additionally, the conducted treatment regimen in this paper was discussed with recent literature describing the approach of infants with RS. RESULTS: Forty-four infants (59%) were found to have been treated conservatively. A significant larger proportion of nonisolated RS infants than isolated RS infants needed surgical intervention (53 vs. 25%, p = .014). A mandibular distraction was conducted in 24% (n = 18) of cases, a tracheotomy in 9% (n = 7), and a tongue-lip adhesion in 8% (n = 6). Seventy-seven percent of all infants had received temporary nasogastric tube feeding. The literature review of 31 studies showed that initial examinations and the indications to perform a surgical intervention varied and were often not clearly described. CONCLUSIONS: RS is a heterogenic group with a wide spectrum of associated anomalies. As a result, the decisional process is challenging, and a multidisciplinary approach to treatment is desirable. Current treatment options in literature vary, and a more uniform approach is recommended. CLINICAL RELEVANCE: We provide a comprehensive and pragmatic approach to the analysis and treatment of infants with RS, which could serve as useful guidance in other clinics.

Tracheostomy versus mandibular distraction osteogenesis in infants with Robin sequence: a comparative cost analysis.

Many treatments have been described for infants with Robin sequence and severe respiratory distress, but there have not been many comparative studies of outcome and cost-effectiveness. The aim of this study was to compare the cost and complications of two common interventions - mandibular distraction osteogenesis and tracheostomy. Nine patients with isolated Robin sequence (mandibular distraction osteogenesis, n=5, and tracheostomy, n=4) were included in the analyses. Predetermined costs and complications were obtained retrospectively from medical records and by questionnaires to the parents over a 12-month period. Overall direct costs (admission to hospital, diagnostics, surgery, and homecare) were 3 times higher for tracheostomy (€105.523 compared with €33.482, p=0.02). Overall indirect costs (absence from work) were almost 5 times higher (€2.543 compared with €543, p=0.02). There was a threefold increase in overall total cost/patient (both direct and indirect) for tracheostomy (€108.057 compared with 34.016, p=0.02) and 4 times more complications were encountered. This study shows that mandibular distraction osteogenesis in infants diagnosed with Robin sequence costs significantly less and results in fewer complications than tracheostomy, and this contributes to our current knowledge about the ideal approach for infants with Robin sequence and might provide a basis for institutional protocols in the future.

A systematic review on the outcome of mandibular distraction osteogenesis in infants suffering Robin sequence.

OBJECTIVES: Mandibular distraction osteogenesis (MDO) has been successfully applied in infants suffering Robin sequence (RS) with severe upper airway obstruction, but no comparative studies for the different types of MDO exist to date. The objective of the current study was to systematically review the published data considering this matter, providing a fundament for protocols and a more conscious treatment strategy for infants with RS in the near future. MATERIAL AND METHODS: For the period from January 1966 to January 2012, the Pubmed, EMBASE, and Cochrane Library databases were searched. Abstracts were screened based on predetermined selection criteria. Relevant full-text articles were retrieved. The articles were analyzed on the type of MDO used, preoperative workup, patient characteristics, postoperative outcome, and complications. RESULTS: The search yielded 109 articles. After checking abstracts and full texts on predetermined inclusion and exclusion criteria, 12 studies (four describing external MDO, five internal MDO, and three both types) were extracted for further analyses. CONCLUSION: Internal MDO seems very feasible in infants suffering RS, minimizing side effects such as hypertrophic scarring, nerve damage, and extensive care needs, although the indications for usage are more limited compared to the external device. Corresponding protocols and long-term outcome studies are needed to make a better comparison and the use and indication of the different types of distraction even more distinct. CLINICAL RELEVANCE: A base for a guideline to support the choice of a designated operative management for neonates with RS is provided, hereby obviating possible complications of the different types of MDO in the future.

[The treatment of drug extravasation]. / De behandeling van extravasaties van geneesmiddelen.

An extravasation is the unintentional leakage of an intravenously-administered drug into the surrounding tissue. Extravasation can cause serious tissue injury. Risk factors for tissue injury can be divided in patient-related factors (age, site of extravasation, comorbidities, physical condition of the patient) and drug-related factors (mechanical injury, pharmacological properties, physicochemical properties). If the right treatment strategy (expectative policy, pharmacological or surgical intervention) is chosen at an early stage, tissue injury can largely be avoided. Comprehensive multidisciplinary approach and communication between nursing staff, the treating physician, the plastic surgeon and the hospital pharmacist is mandatory for the treatment of a patient with extravasation.

A cadaver study of the vascular territories of dominant and nondominant internal mammary artery perforators.

The perfusion territory of the pedicled internal mammary artery perforator flap has been described, but the number of perforators to be included in the flap's pedicle is controversial. We studied the vascular territory of the dominant perforator and the contribution of additional nondominant perforators to it. Therefore, the dominant perforators in 9 fresh cadavers and the nondominant perforators in 4 of these, were injected with water-based ink. The dominant perforator vascularized a territory extending from the superior border of the clavicle to the xiphoid, and from midsternal to the anterior axial fold, with a mean craniocaudal length of 19.4 cm (range, 17.0-24.0) and a mean mediolateral width of 18.6 cm (range, 16.0-22.5). Additional injection of nondominant perforators did not lead to any substantial enlargement of this territory. One single dominant perforator vascularizes a large part of the hemithorax, allowing for various flap designs. Nondominant perforators do not have to be included in the vascular pedicle of the internal mammary artery perforator flap, which leads to less donor-site morbidity.

Anatomy of the vascular pedicle of the internal mammary artery perforator (IMAP) flap as applied for head and neck reconstruction.

INTRODUCTION: The vascular pedicle of the internal mammary artery perforator (IMAP) flap and its enhancement by inclusion of the internal mammary vessels up to the level of the first rib have not been systematically assessed anatomically, to date. This study assesses these features of this pedicle in light of the flap's application in head and neck reconstruction. MATERIAL AND METHODS: The length of the dominant perforator and the enhanced vascular pedicle of the IMAP flap were measured in 27 fresh cadaveric hemi-thoraxes. RESULTS: In 18 out of 27 cadaveric cases (0.67) the second perforator was dominant. The mean length of the 27 dominant perforators was 47 mm (range: 30-66 mm). By enhancement up to the level of the first rib, the mean length of the vascular pedicle could be doubled to 92 mm when based on the second perforator (0.67). In the five cases (0.19), where the third perforator was dominant, the mean length of the enhanced pedicle was 104 mm, whereas it was 61 mm in the four cases (0.15) where the first perforator was dominant. CONCLUSION: This is the first study that assessed the enhanced length of the vascular pedicle of the IMAP flap. Our results show that the mean length of the dominant second perforator may be doubled by such enhancement and that the arc of rotation to the head and neck region may, then, be doubled again. Hereby, even high-located defects in the head and neck region can be reached. This provides a better understanding of the feasibility of the pedicled IMAP flap in head and neck reconstruction.

Clinical application and outcome of the internal mammary artery perforator (IMAP) free flap for soft tissue reconstructions of the upper head and neck region in three patients.

BACKGROUND: The fasciocutaneous internal mammary artery perforator (IMAP) island flap allows for superior esthetical and functional skin cover in the head and neck region in combination with limited donor site morbidity. Its modification as a free flap allows reconstruction of more cranial defects. PATIENTS AND METHODS: Three IMAP free flaps varying from 7 × 4 cm² to 10 × 6 cm² were transplanted in three patients with a mean age of 59 years (range, 54-69 years). Enhancement of the flap's vascular pedicle at least doubles the diameter of the internal mammary vessels to be anastomosed. RESULTS: Coverage with excellent texture and color match was uneventfully obtained and the flaps' donor sites were primarily closed in all three cases. CONCLUSIONS: Our experience proves the consistent feasibility of successful transplantation of the IMAP free flap. Because of its characteristics, we suggest contemplating the use of this flap in the upper head and neck region.