Rahşan Özcan

Spontaneous splenic rupture in a patient with congenital afibrinogenemia.

Afibrinogenemia is a rare bleeding disorder which is observed with an incidence of 1:1 000 000. It is an autosomal recessive disease and occurs as a result of mutation in one of the three genes which code the three polypeptide chains of fibrinogen. Basic clinical findings include spontaneous bleeding, bleeding after minor trauma or due to surgery. Splenic rupture in afibrinogenemia has been reported only in 6 cases so far. In this article, we present a 15-year old congenital afibrinogenemia patient with spontaneous splenic rupture.

Rare cause of dysuria: Eosinophilic cystitis

Eosinophilic cystitis is an inflammatory condition characterized by eosinophilic infiltration of whole layers of the bladder wall. The condition occurs more commonly in adults. We report a case of eosinophilic cystitis mimicking a bladder tumor in a 5-year-old boy with symptoms of dysuria and urinary incontinence. The diagnosis was confirmed by histopathology and he underwent clinical treatment with trimethoprim-sulfamethoxazole and antihistamine (cetirizine). The symptoms fully resolved in follow up, which is continuing. Although very rare, eosinophilic cystitis should be considered in cases of dysuria and increased bladder wall thickness but no identified urinary tract infection.

Testis sparing surgery for Leydig cell pathologies in children

OBJECTIVE The aim was to analyze testis-sparing surgical procedures in boys with Leydig cell pathologies. STUDY DESIGN The hospital records of four boys with Leydig cell hyperplasia who underwent testis-sparing surgery for testicular masses between 2000 and 2012 were analyzed retrospectively. Tumor markers were evaluated and all boys underwent scrotal ultrasonography preoperatively. The hormonal profile was also analyzed for symptoms of precocious puberty. The testis was delivered through a high transverse inguinal incision and the tumor was excised by enucleation. After confirming the benign nature of the tumor with frozen-section examination, the testis was reinserted and fixed into the scrotum with absorbable sutures. All cases were followed-up with physical examination, scrotal ultrasonography, and measurement of β-human chorionic gonadotropin (HCG), α-fetoprotein, and hormone levels. RESULTS The mean age of the patients was 9.4 years (1.5-15 years). Testicular mass and scrotal asymmetry were detected in all cases. Ultrasonography was the main initial diagnostic modality for detecting testicular masses (Table). β-HCG and α-fetoprotein levels were normal. Three cases had Leydig cell hyperplasia and one patient was diagnosed to have a Leydig cell tumor. Signs of precocious puberty were detected in the four patients. The mean follow-up period was 4.8 years (2-8 years). Neither recurrence nor testicular atrophy developed in the follow-up. Findings of precocious puberty continued in one patient with Leydig cell hyperplasia, in whom a 2-mm contralateral metachronous lesion was detected and enucleated successfully. DISCUSSION Testis-sparing surgery with its potential long-term psychological, cosmetic, and functional advantages should be used in pediatric patients in whom a benign Leydig cell pathology is confirmed histopathologically. CONCLUSION This intervention with good long-term results can easily be applied through a proper dissection plane in the testicle. Since testicular Leydig cell tumors in childhood have small rates of recurrence, this choice of treatment is efficient in patients with salvageable testicular tissues and normal levels of tumor markers.

Çocuklarda akut karın

Antibiyotiklerin sayisi ve kullanim alanlari her gecen gun artmaktadir Antibiyotikler dogru taniyla dogru zamanda dogru kullanim sekli ile ve dogru bir antibiyotik secimi ile kullanilmalidir Antibiyotikler mumkunse kultur gonderildikten sonra baslanmalidir Ampirik antibiyotik kullanimi ciddi ve acil durumlarda tercih edilmelidir Turk Ped Ars 2010; 45: 80 Yil: 50 2 Anahtar sozcukler: Antibiyotik cocuk enfeksiyonAntibiyotiklerin sayisi ve kullanim alanlari her gecen gun artmaktadir Antibiyotikler dogru taniyla dogru zamanda dogru kullanim sekli ile ve dogru bir antibiyotik secimi ile kullanilmalidir Antibiyotikler mumkunse kultur gonderildikten sonra baslanmalidir Ampirik antibiyotik kullanimi ciddi ve acil durumlarda tercih edilmelidir Turk Ped Ars 2010; 45: 80 Yil: 50 2 Anahtar sozcukler: Antibiyotik cocuk enfeksiyonAntibiyotiklerin sayisi ve kullanim alanlari her gecen gun artmaktadir Antibiyotikler dogru taniyla dogru zamanda dogru kullanim sekli ile ve dogru bir antibiyotik secimi ile kullanilmalidir Antibiyotikler mumkunse kultur gonderildikten sonra baslanmalidir Ampirik antibiyotik kullanimi ciddi ve acil durumlarda tercih edilmelidir Turk Ped Ars 2010; 45: 80 Yil: 50 2 Anahtar sozcukler: Antibiyotik cocuk enfeksiyon

Pudendal Nerve Block Versus Penile Nerve Block in Children Undergoing Circumcision

PURPOSE Penile nerve block is the most popular nerve block for the circumcision in pediatric patients. This study aimed to compare the analgesic efficiency of penile nerve block and the pudendal nerve block on postoperative pain and additional analgesic requirements in children undergoing circumcision. MATERIAL AND METHODS This prospective randomized double-blind study enrolled 85 children, aged 1 to10 years, undergoing circumcision. The patients were randomly divided into two groups either receiving dorsal penile block group (PNB-Group) or pudendal nerve block (PDB-Group). In the PNB-Group, 0.3 ml/kg 0.25 % bupivacaine was used; and, in the PDB-Group, 0.3 ml/kg bupivacaine was applied with nerve stimulator at a concentration of 0.25%. In the postoperative period, the modified CHEOPS pain scale scoring and additional analgesic demand were evaluated at the 5th and 30th minutes and at the 1st and 2nd hours. The subsequent pain evaluations were made by the parents at home, at the postoperative 6th, 12th, 18th and 24th hours. RESULTS Seven patients were excluded from the study, and seventy eight patients were evaluated for analysis. Patients in PDB-Group had significantly lower postoperative pain intensity and lower mCHEOPS scores (3.83 ± 0.98) when compared to the PNB-Group (6.47 ± 0.91) (P < .01) at all measurement times and none of patients in PDB-Group had additional analgesic requirements up to 24 hours. Patients in the PNB-Group had significantly more analgesic requirements at all measurements times except at the 1st, 2nd, 24th hours. 3.8%, 30.8%, 46.2% and 59% of the patients in the PNB group needed additional analgesia respectively at 5th, 6th, 12th and 18th hours. CONCLUSION Pudendal nerve block provided additional analgesic free period and had better analgesic efficiency compared to the penile nerve block lasting until 24 hours after operation.

KOROZİV MADDE İÇİMİNE BAĞLI ÖZOFAGUS DARLIĞI GELİŞİMİ VE HLA İLİŞKİSİNİN İNCELENMESİ

Amac: Koroziv maddeye bagli ozofagus yanigi olusan olgularda ozofagus darligi gelisimi acisindan fark olabilmektedir. Bu calismada, o zofagus darl igi olusumunda Human Lokosit Antijen (HLA) tipine gore bir farklilik olup olmadiginin arastirilmasi amaclandi. Gerec ve Yontem: Koroziv madde alimi sonrasi yapilan ilk endoskopide grade 1 ve uzeri ozofagus yanigi saptanan olgular geriye donuk olarak degerlendirildi. Grup 1 ozofagus darligi gelisen 10, Grup 2 ise o zofagus darl igi gelismeyen 10 olgudan olusturuldu. Tum olgulardan kan ornegi alinarak HLA antijen tiplendirmesi ve istatistiksel analiz yapildi. Bulgular: Yas ortalamasi 4,75 yil (2-10 yil) idi. HLA antijenlerine gore; grup 1’de HLA-DRB1 ’ 11 antijeni, grup 2’de ise HLA-A3 antijeni sayisal olarak daha yuksek bulundu. Ancak istatistiksel olarak anlamli degildi. S onuc: Calismada iki grup arasinda HLA antijenleri acisindan anlamli fark bulunamamistir. On rapor niteligindeki bu calisma, HLA ozofagus darligi arasindaki ileri calismalara yol gosterici olabilir. Olgu sayisinin artirilmasi ile koroziv o zofagus darl iklarini n erken tan i ve tedavisinde HLA antijenlerinin rolu hakkinda daha kesin bilgiler edinilebilecegini dusunmekteyiz.

ÇOCUKLUK ÇAĞI TÜMÖRLERİNİN AKCİĞER METASTAZLARINA CERRAHİ YAKLAŞIM

Amac: Cocukluk cagi solid tumorlerinin akciger metastazlarinda klinigimizin cerrahi yaklasiminin degerlendirilmesidir. Olgular ve Yontem: Akciger metastazi nedeniyle 1978-2016 arasinda basvuran ve cerrahi girisim yapilan olgular geriye donuk olarak incelendi. Yas, cinsiyet, primer tani, akciger metastazinin ortaya cikis zamani, cerrahi tedavi yontemi, patolojik tani ve takip acisindan degerlendirme yapildi. Primer tumoru kontrol altina alinan, baska uzak organ metastazi olmayan ve akciger metastazi icin cerrahi tedavi uygulanan olgular calismaya alindi. Bulgular: Onsekiz olgunun (K/E:11/7) yas ortalamasi 8,1 yil (1,5-14 yas) idi. Primer tumorler; Wilms tumoru (n:9,%50), Ewing sarkomu (n:3,%17), osteosarkom (n:2,%11), hepatoblastom (n:1,%5,5), fibrosarkom (n:1,%5,5), rabdomyosarkom (n:1,%5,5) ve endodermal sinus tumoru (n:1,%5,5) idi. Olgularin 4’unde ilk basvuru aninda akciger metastazi mevcuttu. Onaltisinda metastazin ortaya cikis zamani ortalama 16, 5 ay (7ay-4 yil) idi. Radyolojik degerlendirme tum olgularda preoperatif donemde akciger grafisi ve bilgisayarli tomografi (BT) ile yapildi. Onsekiz olguya toplam 23 torakotomi yapildi. Yirmibir wedge rezeksiyon, 1 lobektomi, 1 lobektomi ve kot rezeksiyonu uygulandi. Patolojik incelemede cikarilan lezyonlarin 3’u inflamatuar reaksiyon olarak degerlendirildi. Toraks dreni alinma suresi ortalama 5,2 gun(3-8), hastanede kalis suresi 7,3 gun(5-10 gun) idi. Cerrahi girisime bagli komplikasyon gorulmedi. Takipte 6 olgu ilerleyici primer hastalik nedeniyle kaybedildi. Sonuc: Cocukluk cagi tumorlerinde akciger metastazlari basvuru sirasinda ve/veya takipte gorulebilir. Cerrahi tedavisinde sinirli rezeksiyon yeterli gorunmektedir. Torakotomi bu olgularda tum lezyonlarin degerlendirilmesini saglamaktadir ve guvenle uygulanmaktadir. Radyolojik incelemeler tumor metastazini desteklese de kesin tani patolojik inceleme ile konulmaktadir.

A rare cause of abdominal pain: ectopic ovary and intestinal malrotation

Altınay-Kırlı E, Özcan R, Öncül M, Özmen E, Eliçevik M, Büyükünal C, Emir H, Topuzlu-Tekant G. A rare cause of abdominal pain: Ectopic ovary and intestinal malrotation. Turk J Pediatr 2017; 59: 699-703. Ectopic ovary is a rare anomaly that can be associated with unicornuate uterus and renal anomalies. Intestinal rotational anomalies are failure of normal rotation and this arrest in development can predispose to develop a malfixated midgut that is a risk factor for volvulus and significant morbidity and mortality especially in early childhood. Cyclic abdominal pain is a common symptom for both of two distinct pathologies in adolescent ages. Here, we report a case of unicornuate uterus together with right ectopic ovary and intestinal malrotation.

Ultrasound-Guided Thoracic Paravertebral Block Experience in a Child

Dear Editor, Postoperative pain treatment is very important in childhood. In addition to classical blocks, paravertebral block is unilaterally or bilaterally used for analgesia during and after thoracal and abdominal surgeries. The paravertebral space contains neural structures, including anterior and posterior branches of the intercostal nerves, the nerves of the sympathetic chain, rami communicantes and Luschka nerves supplying the intervertebral disc. These bare nerve endings are easily affected by local anaesthetics. Paravertebral block is performed with the conventional loss of resistance technique. However, its use in children is limited compared to adults because it is difficult to predict the distance to the pleura and the loss of resistance in children, particularly during thoracic paravertebral block practice. With the use of ultrasonography in peripheral nerve blocks, paravertebral block can now be performed by direct observation through ultrasound. Ultrasound-guided paravertebral block practices have been reported in children (1, 2). We aimed to share our first experience in ultrasound-guided paravertebral block application. A 3.5-year-old girl, who weighed 16 kg and presented with a complaint of cough and who was pre-diagnosed with hereditary right cystic lung disease, was taken to the operating room primarily for bronchoscopy and then, if necessary, for thoracotomy. The patient was premedicated by administering midazolam 1 mg intravenously (iv). Following monitorization, induction was performed with propofol and rocuronium and bronchoscopy was initiated. During the procedure with a rigid bronchoscope, anaesthesia was continued with a sevoflurane/air and remifentanil infusion. Abundant mucopurulent secretion was aspirated because of chronic lung infection. It was decided to perform lobectomy with thoracotomy from the right fourth intercostal space. Bronchoscopy was stopped, and the patient was placed in the left lateral decubitus position. High-frequency linear probe (MyLab5-LA523E, Esaote SpA, Italy) was placed in the paravertebral space in the longitudinal and paramedian position and at the thoracal 7 level, and transverse process, intercostal ligaments, the seashore sign, the pleura and the pleural space were observed (Figure 1). A Stimuplex A 50 mm (B.Braun, Melsungen, Germany) was pushed forward from a lateral to medial direction using the in-plane method and was advanced towards the paravertebral space. While passing through tissues with the needle under the guidance of ultrasound for anatomic coordination, 1 mL 0.9% NaCl was administered twice and the level of the needle tip in the tissues was identified (Figure 2). After entering into the targeted area, aspiration was performed and then the block was performed by administering 0.5 mL kg−1 bupivacaine (0.25%). Meanwhile, the ‘step sign’, which indicates the collapse of the pleura, was observed by expanding the paravertebral space with fluid under ultrasound guidance (Figure 2). During blockade, no blood aspiration was observed and no hypotension occurred. After the beginning of the surgery, remifentanil infusion was discontinued. The anaesthesia of the patient, who was haemodynamically stable for 2.5 h, was maintained with a 2% concentration of sevoflurane (in oxygen/air) and rocuronium. After the operation, the patient was monitored in the recovery room for 1 h following extubation. She experienced pain in her throat due to the rigid bronchoscopy but did not complain of incision pain, and her respiratory depth was adequate. The patient was monitored with the Children’s Hospital of Eastern Ontario Pain Scale (CHEOPS) (4: no pain; 13: very severe) for 24 h. During the 6 h following the moment of blockade, she experienced no pain. It was observed that she was comfortable in the clinic, and she did not have any complications. In the 6th hour, she began to complain of pain, and her pain evaluation score was 7. Therefore, she was administered 15 mg kg−1 paracetamol iv After 1 h, her pain score was evaluated to be 9, and she was administered 20 mg tramadole iv. The same dose was repeated again after 6 h. The CHEOPS pain scores were between 4 and 9 (the score of 9 was observed just once) during 24 h. Figure 1 Before block A: processus transversus, B: pleura, C: intercostal membrane, D: paravertebral space Figure 2 After block A: processus transversus, B:pleura, D: paravertebral space 1,2: 0.9% NaCl was given for control There was no need for additional analgesia during surgery in the space in which many derivations innervated with a single-dose drug, easy extubation and respiration due to adequate analgesia after thoracotomy occur; hence, this plus the maintenance of 6-h analgesia are important gains. If paravertebral block is to be performed, patient comfort can be increased with the routine use of systemic analgesics before the end of analgesia time. In our case, if an opioid had been administered instead of paracetamol at the 6th hour, i.e. when her pain began, and additional paracetamol had been administered every 6 h, better pain scores would have been obtained. In a case report of a 10-year-old child (weight, 40 kg), a lateral spread between the T4–5 and T10–11 levels and parallel to the spine, which covers the longitudinal and intercostal nerves, was observed with 10 mL contrast substance injected into the paravertebral space through a catheter inserted at the level of T10 (3). On the other hand, in a study performed on cadaver babies, a strong relationship was reported between the volume injected as a paravertebral single-dose at the thoracolumbar level and the number of segments with spread. It has been specified that the most appropriate volume to cover the segments between T10 and L1 is 0.2–0.3 mL kg−1 (4). Hence, it was possible to provide analgesia in the thoracal segments with the volume of 0.5 mL kg−1 given from the T7 level in our case. Paravertebral block can be performed through a unilateral or bilateral single dose or catheter. The analgesic action time of single-dose paravertebral block practice is not clearly reported in previous studies. It is reported that the addition of other drugs to a local anaesthetic for prolonging the duration of analgesia obtained by administering a single-dose into the paravertebral space does not provide an advantage (5). It is possible to obtain analgesia for days by providing continuous infusion through the insertion of a catheter. However, because the paravertebral space is too close to the skin in young children (less than 1 cm), the drug administered may leak from the skin and the catheter may easily change place and may be accidentally displaced. Therefore, if it is planned to insert a catheter in young children, these possibilities must be considered. Some complications such as pneumothorax, pleural puncture and vascular injury are possible, but ultrasound guidance reduces these risks. To avoid the side effects of central block in patients who use an anti-coagulant and whose anatomy is not suitable, paravertebral block can be used in the early postoperative period because it provides adequate analgesia. It is an effective analgesia method that can be preferred for providing patient comfort and respiratory rehabilitation provided that a proper combination is established with systematic analgesics. The use of ultrasound makes this block more effective and safer for use in children.

A review of intussusception cases involving failed pneumatic reduction and re-intussusception.

BACKGROUND The aim of the present study was to evaluate cases in which intussusception was unsuccessfully treated with pneumatic reduction (PR), and intussusception recurred following PR. METHODS The medical records of 401 patients who presented with intussusception between 2003 and 2014 were retrospectively analyzed. Included were 61 patients, 20 of whom underwent unsuccessful PR (Group 1), and 41 of whom experienced intussusception recurrence following PR (Group 2). Treatment and outcome were summarized. RESULTS In Group 1 (mean age: 14.2 months; range: 2.5 months-6 years) surgery was indicated due to PR failure in 15 patients, and perforation occurred during PR in 5. In these 5 patients, age was under 1 year (range 6-9 months) and mean time to presentation was 3 days (range 2-4). During laparotomy, pathologies were noted: mesenteric lymphadenopathy (LAP) and/or Peyers patch hyperplasia was observed in 15 cases, Meckels diverticulum in 5 cases. In Group 2 (mean age: 20 months; range: 3 months-6 years), intussusception developed after successful PR in 41 patients, most frequently within the first 24 hours (21.51%). Of the 41 patients, recurrent intussusception (RI) was treated with PR in 36, and laparotomy in 5. Operative findings were mesenteric LAP in 4 and polyp in 1. CONCLUSION PR is effective for the treatment of intussusception and recurrences. Delayed presentation reduces the success rate. In the event of failure, a lead point is usually encountered at laparotomy.