Surgical Treatment of Lip Pits in Van der Woude Syndrome: A Preliminary Retrospective Study of 24 Patients.

BACKGROUND: Van der Woude syndrome (VWS) is a rare congenital malformation characterized by lower lip pits among patients with a lip and/or palate cleft. It is transmitted by an autosomal dominant inheritance with variable expressivity. METHODS: The study group consisted of 24 consecutive patients (13 males and 11 females) with VWS operated on at a single center between 2009 and 2022. They suffered from: bilateral cleft lip and palate - 6 patients; unilateral cleft lip and palate - 9 patients; cleft lip - 1 patient; and isolated cleft palate - 8 patients. RESULTS: In 16 (66%) cases pits of lower lip occurred on both side of midline, while in 8 (34%) the pits were detected unilaterally. The primary cleft repairs were performed according to one-stage principle at the mean age of 8.6 months (SD 1.4, range 6-12). In all patients lower lip pits repairs were performed after the primary cleft repairs as a separate procedure at the mean age of 37 months (SD 11.3 range 14-85). The mean number of all primary repairs of the syndrome-both cleft defect and lower lip pits repairs-was 2.46. Nine patients (37.5%) required additional secondary corrections of the lower lip due to the poor aesthetic post-operative outcome. CONCLUSIONS: The frequent need for secondary corrections of residual lower lip deformities indicates the considerable difficulties in obtaining a satisfactory outcome of the repairs to lip pits caused by VWS. The average number of the primary surgical interventions in evaluated material remained low.

Comparative study on cleft palate morphology after passive presurgical plate therapy in unilateral cleft lip and palate.

BACKGROUND: Presurgical plate therapy has been widely accepted as a treatment prior to palatal cleft closure. The effects of passive presurgical plate therapy on cleft morphology prior to single-stage unilateral cleft lip and palate (UCLP) repair were quantified. PATIENTS AND METHODS: We compared the dimensions of cleft width and cleft area (true cleft and palatal cleft) measured preoperatively at 2 European cleft centers. Center A performed single-stage UCLP repair in 8-month-old infants without any presurgical orthopedic treatment. Center B initiated passive presurgical plate therapy immediately after the birth of the neonates, followed by single-stage UCLP repair at 8 months of age. RESULTS: We included 28 patients with complete UCLP from Center A and 12 patients from Center B. The average anterior width of the true cleft before surgery was significantly smaller in infants at Center B than that in Center A (p = 0.001) with 95% confidence interval of (1.8, 5.7) mm, but the average posterior width was similar in the 2 groups. The mean presurgical true cleft area amounted to 106.8 mm2 (SD = 42.4 mm2) at Center A and 71.9 mm2 (SD = 32.2 mm2) at Center B, with a confidence interval for the difference being (9.8, 60.1) mm2. This corresponded to a 32.7% reduction of the true cleft area when passive presurgical plate therapy was used for the first 8 months of the infants' life. CONCLUSION: Passive presurgical plate therapy in UCLP significantly reduced the cleft area. Implications for the subsequent surgical outcome might depend on the surgical technique used.

Digital impressions from newborns to preschoolers with cleft lip and palate: A two-centers experience.

BACKGROUND: Documenting cleft lip and palate morphology prior to surgery is standard care. Presurgical orthopedic treatment also requires a 3D cleft model. Endangering the airway, conventional impressions require additional safety measures and resources. We investigate the implementation and risks of digital impressions for the youngest patients with orofacial clefts. METHODS: We report a retrospective cohort study of patients with cleft lip and palate, aged up to 6 years, treated at two cleft centers in Europe (Basel (A), Warsaw (B)). We scanned with the Medit i500 (Medit Corp, Seoul, South Korea). Center A for presurgical orthopedics and prior surgery from June 2020 to March 2022. Center B prior surgery from December 2020 to May 2021. Scanning data were analyzed for adverse events and adverse device effects, scanning duration, and number of images according to cleft type and age. RESULTS: We analyzed 342 digital impressions in 190 patients (center A: 71, B: 119). The median age was 8.7 months with a range from the first day of birth (presurgical orthopedics) to six years of life (early alveolar bone grafting). No adverse events or adverse device effects were observed. The median scan duration was 85.5 s for cleft palate and 50 s for cleft lip and nose (IQR 56 s and 39 s, respectively). CONCLUSION: Digital impressions with intraoral scanners are safe in patients with cleft lip and palate from newborn to preschool age. Given the funding to purchase an intraoral scanner, interfaces to electronic patient records, and point-of-care 3D printing, cleft centers can successfully implement this technology.

Bone Defect Repair Using a Bone Substitute Supported by Mesenchymal Stem Cells Derived from the Umbilical Cord.

OBJECTIVE: Bone defects or atrophy may arise as a consequence of injury, inflammation of various etiologies, and neoplastic or traumatic processes or as a result of surgical procedures. Sometimes the regeneration process of bone loss is impaired, significantly slowed down, or does not occur, e.g., in congenital defects. For the bone defect reconstruction, a piece of the removed bone from ala of ilium or bone transplantation from a decedent is used. Replacement of the autologous or allogenic source of the bone-by-bone substitute could reduce the number of surgeries and time in the pharmacological coma during the reconstruction of the bone defect. Application of mesenchymal stem cells in the reconstruction surgery may have positive influence on tissue regeneration by secretion of angiogenic factors, recruitment of other MSCs, or differentiation into osteoblasts. Materials and Methods. Mesenchymal stem cells derived from the umbilical cord (Wharton's jelly (WJ-MSC)) were cultured in GMP-grade DMEM low glucose supplemented with heparin, 10% platelet lysate, glucose, and antibiotics. In vitro WJ-MSCs were seeded on the bone substitute Bio-Oss Collagen® and cultured in the StemPro® Osteogenesis Differentiation Kit. During the culture on the 1st, 7th, 14th, and 21st day (day in vitro (DIV)), we analyzed viability (confocal microscopy) and adhesion capability (electron microscopy) of WJ-MSC on Bio-Oss scaffolds, gene expression (qPCR), and secretion of proteins (Luminex). In vivo Bio-Oss® scaffolds with WJ-MSC were transplanted to trepanation holes in the cranium to obtain their overgrowth. The computed tomography was performed 7, 14, and 21 days after surgery to assess the regeneration. RESULTS: The Bio-Oss® scaffold provides a favourable environment for WJ-MSC survival. WJ-MSCs in osteodifferentiation medium are able to attach and proliferate on Bio-Oss® scaffolds. Results obtained from qPCR and Luminex® indicate that WJ-MSCs possess the ability to differentiate into osteoblast-like cells and may induce osteoclastogenesis, angiogenesis, and mobilization of host MSCs. In animal studies, WJ-MSCs seeded on Bio-Oss® increased the scaffold integration with host bone and changed their morphology to osteoblast-like cells. CONCLUSIONS: The presented construct consisted of Bio-Oss®, the scaffold with high flexibility and plasticity, approved for clinical use with seeded immunologically privileged WJ-MSC which may be considered reconstructive therapy in bone defects.

[Influence of selected factors on the treatment and prognosis in newborns with gastroschisis on the basis of own experience]. / Wplyw wybranych czynników na przebiegleczenia i rokowanie u noworodkówz wrodzonym wytrzewieniemna podstawie doswiadczen wlasnych.

AIM: The purpose of the study was to evaluate the influence of selected prognostic factors for postoperative course and prognosis in newborns with gastroschisis. MATERIAL AND METHODS: A study of all newborns with gastroschisis treated between 2000-2010 in the Clinic of Surgery of Children and Adolescents, Institute of Mother and Child was performed. Data collected from medical documentation included the following: presence or lack of prenatal diagnosis, mode of delivery, gestational age (below or above 37 weeks), birth weight, necessity on transportation from provincial hospitals or transfer within Institute, condition of the bowel (good - little fibrinous inflammation or bad - massive inflammatory peel, necrosis, perforation, atresia), interval between delivery and operation, kind of surgery (primary repair, silo closure), complication requiring secondary operation, period of ventilatory support (PVS), time needed to achieve full enteral feeding (FEF), total length of hospital stay (TH), number and cause of death. Selected information obtained from the data of the patients were separated into two periods of time: 2000-2005 and 2006-2010 for better evaluation of the influence of individual factors on the efficiency of treatment and prognosis. Multivariate logistic regression was used to investigate the association between selected risk factors and end points (PVS,FEF,TH). Statistical analyses were performed using Stata v.10 (College Station, TX, Stata Corporation LP 2007). RESULTS: During the study period 32 newborns with gastroschisis were treated. Prenatal diagnosis was made in 22 patients (69%) and the mean age of diagnosis was 30.7 weeks. Cesarean section was performed in 25 cases and vaginal delivery occurred in 7 cases. The mean gestational age during delivery was 35.7 weeks, mean weight was 2430 g. Twenty one newborns were delivered before 37 week of gestation, eleven after 37 week. Fifteen patients were transported from provincial obstetrics hospitals, seventeen were transferred within the Institute (from the Obstetrics Clinic to Clinic of Pediatric Surgery). A good condition of the externalized bowel was found in 18, a bad condition in 14 patients (therein necrosis with perforation in 2, atresia in 2). Mean delivery - operation interval was 6.3 hours. The operation was performed till 3rd hour after birth in 12, over 3rd hour in 20 newborns. During the first surgical intervention primary closure was possible in 29 cases, silo was used in 3 patients. Five patients required more than one surgical intervention (2 patients after silo closure and 3 patients after primary repair). For patients who survived mean PVS was 4.6, mean time FEF was 24.7 days, TH was 34.5 days. Five patients died. The reasons for death were heart tamponade in 2 and complications in the course of sepsis in 3 patients. In the period 2006-2010 versus 2000-2005 number of prenatal diagnosis significantly increased (46% and 84% respectively), mean age at delivery decreased (38.6 and 35.3 respectively), period between delivery and operation shortened from 8.8 to 3.8 hours, more patients were operated on during first three hours after birth (7.6% and 58% respectively). The condition of the bowel was assessed similarly in both periods (bad condition 38% and 47% respectively). All deaths occurred in newborns treated in the years 2000-2005. Multivariate logistic regression showed there was one independent risk factor that influenced the two end points: the period of respiratory support and the length of hospital stay, i. e. the delivery - operation interval. Patients with delivery - operation interval over 3 hours after birth had a significantly higher risk of long-standing ventilatory support or death (OR=12.4, 95%CI {1.7, 89.3}, p=0.013) and a significantly higher risk of longer total hospital stay or death (OR=12.7, 95%CI {1.7, 97.0}, p=0.014). None of the factors analyzed had statistical significance with respect to the length of time needed to achieve full enteral feeding. CONCLUSION: The main independent risk factor having influence on the course of treatment and prognosis was the delivery - operation interval. Early repair of gastroschisis makes primary closure easier and shortens the post-operative course. Newborns with gastroschisis despite progress in prenatal diagnostics, neonatal intensive care and surgical methods remains a serious therapeutic problem requiring multidisciplinary care and long-standing hospital stay.

Castleman's disease - an unusual indication for laparoscopic adrenalectomy.

Castleman's disease is one of the rare entities which cause lymph node hyperplasia with no characteristic clinical symptoms. Pathomorphological examination usually enables diagnosis, especially in the case of a localized form. Its uncommon location in the retroperitoneal cavity is estimated at 12% in the literature. Asymptomatic nature of the disease and its untypical location in the adrenal field imitated incidentaloma of this gland in the imaging examinations. Surgical treatment, laparoscopic excision of the tumour (lymph node) and right adrenal gland from the lateral, transperitoneal approach allowed final diagnosis and offered effective therapy.