Sokolova, Rozeta

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Sokolova, Rozeta

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Sokolova, Rozeta

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BARDET-BIEDL SYNDROME
(Department of Anaesthesia and Reanimation, Faculty of Medicine, Ss. Cyril and Methodius University in Skopje, R.N. Macedonia, 2025-03-01)
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Ljumani Bakiji, Lj
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Abstract Bardet-Biedl syndrome (BBS) is a genetic and multisystem disease that affects the genitourinary tract, locomotory system, causes eye anomalies, cognitive disorders and characteristic truncal obesity. It is caused by mutations in certain genes, namely: BBS1 to BBS21 gene. The approach to this disease is multidisciplinary. Material and Methods: We present a 3-years-old female child who was referred to the Clinic for Pediatric Surgery due to supernumerary toes on both feet. This is postaxial polydactyly. Intrauterine lobulated kidney structure was observed. The child had problems with her peers and avoids socializing with them. The following examinations were performed: cardiological, ophthalmological and genetic. Genetic examinations confirmed the BBS syndrome. Results: The patient was operated at the clinic for pediatric surgery, the operative and postoperative period were without complications. Conclusion: BBS is a rare autosomal disease that requires timely detection and appropriate multidisciplinary treatment. This allows complications to be reduced and the child to be included in everyday activities.
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VERRUCOUS MALFORMATIONS AS A RARE CASE IN THE GROUP OF VASCULAR ANOMALIES WITH SLOW BLOOD FLOW IN CHILDREN
(Македонско лекарско друштво = Macedonian medical association/De Gruyter, 2025-04-04)
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Kamiloski, Marijan
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Lumani Bakiji, Njomza
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Racaj, Anila
Verrucous venous malformations belong to the group of vascular anomalies. The International Society for the Study of Vascular Anomalies (ISSVA) classifies these changes into two major groups: vascular tumors and vascular malformations. The vascular malformations group includes lymphatic malformations, venous malformations, and their combinations (malformations with slow blood flow), as well as arteriovenous malformations (malformations with fast blood flow). Verrucous venous malformations (VVM) are a rare congenital vascular anomaly first described by Helwig in 1967. They are characterized by varying degrees of hyperkeratosis of the skin and subcutaneous tissue. Clinically, they manifest as dark red solitary or multiple hyperkeratotic plaques, most commonly localized on the extremities. Treatment is combined and includes surgical intervention, electrocoagulation, laser therapy, topical agents, and systemic medications. Materials and Methods: Between 2019 and 2024, two children with suspected verrucous venous malformations were admitted to the Public Healthcare Institution – University Clinic for Pediatric Surgery. The lesions were unilaterally located on the lower extremities. The gender distribution was 1:1 (male to female), with an average age of 10 years. The changes had been present since birth. There was no positive family history in either patient. Clinically, one of the patients presented with inflammation of the lesion, accompanied by pain and serous secretion. Results: Following appropriate preoperative preparation, both patients underwent surgical excision and autologous skin transplantation. The postoperative course was uneventful. Histopathological examination confirmed the diagnosis. After excision, electrocoagulation was applied to residual lesions. A reduction of approximately 90% in lesion size was recorded. Conclusion: Verrucous venous malformations are a rare type of malformation within the group of vascular anomalies, requiring a multidisciplinary treatment approach.
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TREATMENT OF VENOUS MALFORMATIONS IN PEDIATRIC POPULATION – THREE- YEAR EXPERIENCE
(Институт за јавно здравје на Република Македонија = Institute of public health of Republic of Macedonia, 2022-12-30)
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Lumani-Bakiji, Njomza
Venous malformations (VMs) are a type of vascular malformations that result in abnormal development of veins that become extensible over time due to an error in vascular morphogenesis. They usually appear in newborns or in early adulthood as a bluish, soft, swollen and eventually painful skin formation. Treatment includes conservative therapy, sclerotherapy, and surgical excision. Aim of the paper is to evaluate the therapeutic effect of sclerotherapy in pediatric patients with venous malformations. Material and methods: In a three-year period, from 2019 to 2021, venous malformations were found in 33 patients aged 4 to 14 years (average age: 8 years). Pain as a symptom occurred in 8 patients. Two patients had lesions measuring up to 5 cm and 5 cm, respectively, while in the remaining subjects the lesion was over 5 cm. Ultrasound was performed routinely in all subjects, and MRI in two patients. Conservative treatment was instituted in 13 patients with venous malformations of the extremities; surgical excision with local reconstruction was performed in 11 patients, and sclerotherapy with bleomycin under general anesthesia was performed in 8 patients. Combined treatment was used in one patient that presented with venous malformation of the upper arm that underwent partial sclerotherapy with subsequent operative excision due to a phlebolith. Follow-up examinations revealed regression of the change not only from functional but from aesthetic aspect as well. Conclusion: Sclerotherapy is the established golden standard, first-line treatment for venous malformations. Excellent results were achieved as the reduction of the lesions was below 50% of the initial size. However, the modality of treatment should be individualized to each patient as it can sometimes require a combination of more than one treatment option. Venous malformations are best treated early, but they usually recur over time. Treatment helps relieve symptoms and control the growth of vascular malformations.
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TREATMENT OF LYMPHATIC MALFORMATIONS IN CHILDREN: 3 YEARS EXPERIENCE
(University of Tetovo, 2023-05)
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Njomza LUMANI-BAKIJI
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Lazo JOVCHESKI
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Aleksandar STEPANOVSKI
Introduction: Lymphatic malformations (LM) are congenital benign malformations from the group of slow-flow vascular anomalies consisting of pathological cystic dilatation of the lymphatic vessels. The incidence of LM ranges from 1.5 to 2.8% in 2000 to 4000 newborn children, and are relatively rare congenital vascular anomalies. They are characterized with equal representation between the genders. From the structural aspect, LM are divided into macrocystic, microcystic and mixed type, with its own therapeutic and prognostic implications. A small percentage of LM are combined with additional anomalies and are part of syndromes such as CLOVES, Klippel-Trenaunay, Proteus and others. In recent years, the method of choice in the treatments is sclerosing as a non-invasive method achieving almost excellent results.
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СУПЕРФИЦИЈАЛЕН АНГИОМИКСОМ КАЈ 3-ГОДИШНО ЖЕНСКО ДЕТЕ - ПРИКАЗ НА СЛУЧАЈ
(Association of Albanian Medical Doctors in Macedonia, 2022)
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Радоје Симиќ
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Лазар Тодоровиќ
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Њомза Љумани Бакији
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Вовед. Ангиомиксомот за првпат е опишан во 1948 година од Stout, а суперфицијалниот ангиомиксом за првпат е опишан од Allen во 1988 година како ретка бенигна туморозна формација составена од миксоидна матриксна формација и бројни крвни садови. Оваа лезија е генерално нодуларна со големина од 1 сантиметар (см) до 5 см, со локализација на глава, врат и предилекција за гениталната регија. После хирушкиот третман потребно е следење од генетичар педијатар за исклучување на Carney-евиот complex (мултипни кутани миксоми, мамарни миксоми, срцеви миксоми и ендокрини пореметувања). Потребно е додатно следење од детски хирург заради опасноста од локална рекуренција. Во светот се опишани рекуренции од 30% до 40% во првите две години по хирушката ексцизија. Не е објавено појава на малигнитет. Приказ на случај. девојче на возраст од 2 години хоспитализирана поради туморозна формација на десната голема усна на вулвата со големина од 3 см во должина и широчина од 1.2 см. Која се појавила во тек на првата година без дополнителни симптоми. Со оперативен зафат е направена хирушка ексцизија и локална пластика под општа анестезија. Патохистолошкиот наод е во прилог на суперфицијален ангиомиксом во кој се опишуваат: присуство на многуслоен плочест епител под кој се наоѓа мултинодуларна пролиферација на стеласти клетки и многу крвни садови“. Консултиран педијатар генетичар кој освен минимално зголемено ниво на естрогени хормони кои се следени и во опаѓање, се изјасни за уреден ендокринолошки наод. Детето е следено една година, без знаци за рецидив. Заклучок. Суперфицијалниот ангиомиксом најчесто се јавува во средна возраст и е сосема редок во педијатриската популација. Во диференцијална дијагноза се вбројуваат: фокални кутани муцинози, миксоидни перифоликуларни фиброми, миксоидни неурофиброми, дермални нервни миксоми, миксоидни липосаркоми и Carney-ев complex. Патохистолошката анализа вклучува специфични хистохемиски и имунохистохемиски иследувања.
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Topical treatment of minor facial burns in pediatric patients
(2017)
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Risto Simeonov
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Vladimir Chadikovski
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Introduction Inguinal hernias have been treated traditionally with conventional methods, but the trends have changed with the introduction of minimal access surgery.The study aims to compare the open technique versus laparoscopic assisted technique of percutaneous closure of the internal inguinal(PIRS).Materials and methods: The study is done at the University Clinic for Pediatric Surgery in Skopje in the period 2016/17. There are analyzed 30 female children aged 1-14 years with clinically diagnosed indirect inguinal hernia.Results: The average duration of the intervention in the experimental or control group was 25,1 ± 6,1 v.s 44,8 ± 6,3 minutes. The length of hospital stay in the two groups is consequently 10,7 ± 2,1 v.s 25,2 ± 3,3 hours. The shortest stay was 8 v.s 24 hours and the longest 17 v.s 26 hours. All children in the experimental group took up a normal position in bed for less than 3 hours compared to the control group in which it accounted for 5 hours. The average length of the mark is 1,9 ± 0,9mm in the test and 24,8 ± 17,4mm in the control group. Analgesia because of pain is given in 3 (20%) children in the test group and on 7 (46,6%) in control group. Easy impressive scar have 8 (53,3%) patients in the control group and no patient in the experimental group. The mark does not disturb the aesthetics in 9(60%) of patients in the experimental group and in 2 (13,3%) in the control group. Conclusion: Laparoscopic assisted technique of percutaneous closure of the internal inguinal ring is minimally invasive method and is totally safe for the treatment of inguinal hernias in children with special advantage for females.
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Correction of Congenital Ear Deformities - Our Experience
(2018-10)
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Jovceski, Lazo
Congenital ear deformities cover a wide range of defects concerning the outer ear. Prominent ear, lop ear and microtia are most frequently encountered congenital ear problems in pediatric plastic surgery. The aim of this study is to present our experience in the correction of congenital ear deformities, with a special emphasis on the correction of prominent ears.
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Proteus Syndrome – Rare Genetic Disorder in Children (Case Raport)
(Faculty of Medical Sciences, 2018-11)
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Lazo JOVCHESKI
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Njomza LUMANI
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TREATMENT OF UNSTABLE FOREARM FRACTURES WITH ELASTIC INTRAMEDULLARY OSTEOSYNTHESIS IN CHILDREN
(Association of Albanian Medical Doctors in Macedonia, 2018)
Todorovich L.
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Kamilovski M.
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Lumani Nj.
Introduction: Over the past years there has been a marked increase in the use of intramedullary fixation in the management of displaced forearm fractures in children. This reflects high incidents of redisplacement, malunion and functional limitations. Aim: This retrospective study was performed to analyze the functional results of the elastic intramedullary titanium nail fixation used in the treatment of unstable, diaphyseal forearm fractures in children. Material and methods: From March 2013 to March 2012, 235 patients with forearm fractures were treated at our institution. Percutaneous intramedullary f ixation with titanium nail was performed in 132 patients, 96 with unstable reposition and 36 with redislocation 48 hours after the injury. The mean age of the patients was 9.6 (5-13) years. In 34 patients (31%) limited open approach to one or both was necessary for reduction. Result: In 12 patients we encountered minor complications. The average union time was 7.5 (5-13) weeks. We rated the functional results using the Price grading scheme. Thirty-two patients obtained excellent results, four very good, four good and two scored poorly. Conclusion: Despite the minor complications, percutaneous intramedullary fixation with titanium nails is an appropriate and safe treatment method for unstable fractures of the forearm in children which cannot be treated by closed manipulation.
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CASE REPORT of 2 year old female child with Proteus Syndrome (PS)
(Association of Albanian Medical Doctors in Macedonia, 2018)
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Lazo Jovcheski
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Njomza Lumani
Proteus syndrome is a rare genetic disorder, characterised by overgrowth of various tissues. Organs and tissues affected by the disease grow out of proportion to the rest of the body. The overgrowth is usually asymmetric. Proteus syndrome is a result of mutation in the AKT1 gene. The overgrowth becomes apparent between the age od 6 and 18 months. Progressive skeletal malformations, bullous pulmonary disease, tumors, vascular malformations and skin diseases are reported. Our patient is 2 years old female, hospitalized in our clinic with suspicion for PS. The child had pain and clinically noted localized tumor in the right axillary and partial pectoral region, with painful and restricted ROM in the right shoulder. We also noticed changes on the left forearm, and on the left small fingers, without local od systematic sings of inflammation. We made surgical excision of the soft overgrowth tissue od pectoral-axilary region. The patohistological result we got was fatty tissue. We find this case interesting to show for it is very rare genetic condion with an incidence less then 1 in 1 milion people world wide.

Sokolova, Rozeta

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