DiGeorge Syndrome (DGS) is a congenital disorder that affects the thymus, parathyroid glands, and... more DiGeorge Syndrome (DGS) is a congenital disorder that affects the thymus, parathyroid glands, and heart and brain. Thymus involvement in DGS may vary between absence/hypoplasia of thymus to various forms of reduced T cell function. TBX1 deficiency causes a number of distinct vascular and heart defects, suggesting multiple roles in cardiovascular development, specifically, formation and growth of the pharyngeal arch
A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric openi... more A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric opening, a foreshortened colon, imperforate anus, and meningocele is presented. The surgical management of this disorder is discussed and available literature is reviewed. The patient was successfully treated with a combined, single-stage surgical correction.
Turk Pediatri Arsivi-turkish Archives of Pediatrics, Jun 1, 1999
Although various methods of treatment are described for complicated hemangiomas the treatment mod... more Although various methods of treatment are described for complicated hemangiomas the treatment modalities of these are still controversial Between 1993 and 1997 fifteen infants with complicated cutaneous hemangiomas have prospectively been treated by intralesional corticosteroid injections triamcinolone dexamethasone and short term oral prednisolon therapy The localizations of the hemangiomas were as follows: eyelid 3 upperlimb 2 retroauricular 2 labium major 3 forehead 1 lower limb 1 submandible 1 neck 1 and forearm 1 The indications for treatment were; ulceration 2 bleeding 2 infection 3 ambliopy 3 unesthetic apperance 2 and both infection and bleeding 3 The moan age of the patients was 7 4 3 2 months 2 5 12 months and all of them were female No visceral hemangioma and platelet trapping were determined in any case An average of 2 9±1 2 intralesional injections were performed Mean regression obtained was 75 8±19 4 13 patients The remaining two hemangiomas were unchanged The patients did not present with early or late complications in their follow up period with an avarage duration of 27 months As a result intralesional corticosteroid injections with short term oral prednisolone treatment are effective modes of therapy for complicated cutaneous hemangiomas in infants Key words: Cutaneous Hemangioma Corticosteroid Infant
IntroductionTransanal endorectal pull-through (TEPT) is the latest advancement in the treatment o... more IntroductionTransanal endorectal pull-through (TEPT) is the latest advancement in the treatment of Hirschsprung’s disease (HD). The aim of this study was to evaluate the safety and efficacy of TEPT as a definitive treatment for patients with HD.Patients and methodsA retrospective study of 50 patients who underwent TEPT at Uludağ University, Bursa, Turkey, between June 2001 and April 2012 was conducted. These patients were evaluated with regard to their age, sex, preoperative findings, and length of the aganglionic segment, intraoperative and postoperative complications, and results of the follow-up.ResultsForty-three patients were boys and seven were girls. The median age was 3 months (range 0–96 months). The most common symptoms were abdominal distention and vomiting. HD was diagnosed in 38 patients using barium enema and anorectal manometry, in 11 patients using rectal biopsy, and in 1 patient using laparotomy with biopsy. The mean time from the beginning of enteral feeding was 2.2 ± 1.1 days. The mean follow-up period was 26.7 ± 20.8 months. The postoperative complications included transient perianal excoriation in 12 patients, enterocolitis in 10, anastomotic stricture in 3, soiling in 3, recurrent constipation in 2, prolapse of the pulled through colon in 1, anastomotic leak in 1, and rectovestibular fistula in 1 patient.ConclusionsTEPT is a feasible and safe procedure in children with rectosigmoid HD.
European Journal of Pediatric Surgery, Oct 1, 1998
Ileal duplication cysts within a giant omphalocele are very rare. Only a few cases have been repo... more Ileal duplication cysts within a giant omphalocele are very rare. Only a few cases have been reported in the English literature (4). We report one case of giant omphalocele, which included a huge ileal duplication cyst, detected by prenatal US, and diagnosed at surgery after birth. This case illustrates the diagnostic and therapeutic problems occurring during pregnancy and the neonatal period.
The solitary rectal ulcer syndrome (SRUS) is an unusual disorder in childhood. Although well reco... more The solitary rectal ulcer syndrome (SRUS) is an unusual disorder in childhood. Although well recognized in adult literature, the pediatric experience with this condition is limited, so SRUS often goes unrecognized or misdiagnosed. There are very few pediatric case reports in the English literature. This report describes four patients who presented with rectal bleeding, constipation, mucous discharge, and lower abdominal pain, with a diagnosis of SRUS. The diagnosis was made by rectoscopy, defecogram, anorectal manometry and histopathological evaluation. In two patients, defecogram showed a rectocele with both, the sphincter failed to relax to voluntary squeeze pressure on anorectal manometric examination. The histopathological finding in all patients was fibrous obliteration of the lamina propria with disorientation of muscle fibers. All of the patients responded well to conservative therapy, which included defecation training, laxatives, sulfasalazine, and application of rectal sucralfate enema, and remained asymptomatic on the follow-up. Although rare in the pediatric population, SRUS should be relatively easy to recognize in the child with rectal bleeding, after elimination of other causes. If suspected, the diagnosis of SRUS may be made at endoscopy and confirmed by rectal biopsy.
The frequencyof additional congenitalanomaliesin associationwith oesophageal atresia(OA) is about... more The frequencyof additional congenitalanomaliesin associationwith oesophageal atresia(OA) is about50% (1–4). The most commonly associatedmalformations are other atresiasof the gastrointestinaltract, imperforateanusandVATER (vertebraldefects,anal atresia, tracheoesophageal fistula with esophagealatresia,radial and renaldysplasia)complex(4,5). Hypertrophic pyloric stenosis(HPS) complicatingthe postoperative period of OA and tracheoesophageal fistula (TEF) is lesswell known(6). In this paperwe presenttwo cases of OA and TEF, whose postoperativecourseswere complicatedwith HPS.
European Journal of Pediatric Surgery, Dec 1, 1999
Three experimental groups have been constructed to evaluate the long-term outcome of fetal adrena... more Three experimental groups have been constructed to evaluate the long-term outcome of fetal adrenal transplantation in rats. In Group 1 (n = 10), rats underwent bilateral adrenalectomy. In Group 2 (n = 10), rats underwent a sham procedure which included flank incision and arterial canulization. In Group 3 (n = 20), a left adrenalectomy was performed followed immediately by transplantation of a fetal adrenal graft into the greater omentum and marked with a prolene suture (Stage 1). One year later, the right adrenal gland of the recipient was removed followed by a determination of the fetal adrenal graft function (Stage 2). Graft function was evaluated by measuring ACTH and corticosterone levels; and a histologic examination of the transplanted fetal adrenal gland was obtained at autopsy. In Group 1, all the rats died within first 8 hours, following bilateral adrenalectomy. In Group 2, and Group 3, all rats survived after Stage 1 operation. During the Stage 2 operation, it was observed that three rats (15%) had neither fetal adrenal transplant nor prolene suture, seven rats (35%) had a well vascularized and developed fetal adrenal graft and a prolene suture. There was no visible fetal adrenal graft but the prolene suture was present in the remaining rats (50%) in Group 3. After removal of the right adrenal gland (6 and 12 hours later), the mean plasma level of ACTH increased with a decline in mean serum corticosterone level in Group 3 compared to the sham-operated animals (p < 0.001). In spite of visible, and viable transplants, all rats died within 48 hours following Stage 2 operation. The mean weight of the fetal adrenal transplant showed a sixteen-fold increase compared to the initial weight (p < 0.001) and histologic examination showed all 3 zones of adrenal cortex, but there were no medullary cells noted. Although the transplanted fetal adrenal grafts survived, their hormonal function was not enough to maintain host viability. Based on these results it is concluded that, insufficiency of the transplanted fetal adrenal gland may be secondary to either graft rejection or suppression of the transplanted tissue by the functional recipient adrenal despite the fetal adrenal transplant survival.
Journal of International Medical Research, Jul 1, 1997
A total of 24 neonates and infants, aged from 2 days to 10 months, received treatment with intrav... more A total of 24 neonates and infants, aged from 2 days to 10 months, received treatment with intravenous fluconazole for microbiologically documented or presumed fungal infection. The mean fluconazole dosage was 6 mg/kg/day (range 2-16 mgJkg/day) and the mean duration of therapy was 25 days (range 5-72 days). Efficacy was evaluated in neonates with proven fungal infections, as documented by the presence of pathogen at baseline. A positive clinical response was achieved in 23 of the 24 clinically evaluable patients (96%); eradication ofthe fungal organism was also achieved in 23 of the 24 evaluable patients (96%). Adverse events occurred in two patients (8%) but therapy was not discontinued in either patient. The present results confirm the efficacy and safety of fluconazole in the treatment of neonates and infants with severe fungal infections.
Journal of International Medical Research, Sep 1, 1997
Growth and development were monitored for up to 42 months in nine neonates to whom ciprofloxacin,... more Growth and development were monitored for up to 42 months in nine neonates to whom ciprofloxacin, a fluoroquinolone, was given in the neonatal period at a dosage of 20 mg/kg/day. Ciprofloxacin was used only as a 'life-saving' therapy in cases of sepsis produced by bacterial agents resistant to other antibiotics. Two other groups of nine neonates, matched by birth weight and gestational age, were studied as controls: one group with sepsis, which was effectively treated with cefotaxime and a group of healthy neonates. No statistically significant differences in growth and development between the groups were found during follow-up for 42 months. No osteoarticular problems or joint deformities were observed in the ciprofloxacin group. Ciprofloxacin appears to provide a therapeutic option as a 'life-saving' therapy for newborns with sepsis produced by multiply resistant organisms.
Turkish journal of trauma & emergency surgery, Apr 1, 1998
... Çocuklarda kotlar eris/kine oranla daha az mineralize ve daha esnek oidugu için toraks travma... more ... Çocuklarda kotlar eris/kine oranla daha az mineralize ve daha esnek oidugu için toraks travmasi esnasinda kotlar genellikle esner, kirilmaz. ... Pediatric Surgery. Chicago: Yearbook Medical Publishers, 198Ъ. Pp 143 Smyth ВТ. ...
The oromandibular limb hypogenesis syndromes (OLHS), which are congenital malformations involving... more The oromandibular limb hypogenesis syndromes (OLHS), which are congenital malformations involving the tongue, mandible, and the limbs are extremely rare with a few sporadic cases reported in the literature. In this report, the authors report a case of OLHS with gastroschisis, that has not been described previously according to the medline search.
European Journal of Pediatric Surgery, Jun 1, 1999
ABSTRACT A patient, 5 years of age, presented with a swelling on the right side of the neck. Ultr... more ABSTRACT A patient, 5 years of age, presented with a swelling on the right side of the neck. Ultrasonography and computed tomography confirmed a diagnosis of phlebectasia of the right internal jugular vein (IJV). The patient underwent operation and the dilated IJV was excised. Dilatation of the IJV with a Valsalva maneuver suggested a mechanical obstruction in the neck or mediastinum. However, the exact cause is still unknown. Finally, we found the patient to have a thinning of the wall but no other associated abnormality.
European Journal of Pediatric Surgery, Oct 1, 1999
An l-day-old female newborn who had typical elinical features of Aicardi syndrome, such as agenes... more An l-day-old female newborn who had typical elinical features of Aicardi syndrome, such as agenesis of the corpus callosum, ocular abnormalities and infantile spasm associated with a palatal hemangioma is reported. The intraoral mass, which oceluded incompletely the oropharynx and right side of the nasopharynx, was partially excised under Resume Les auteurs rapportent l'observation d'une fillette de 1 jour atteinte du syndrome typique d'Aicardi: agenesie du corps calleux, anomalies oculaires, spasticite infantile et Mmangiome du palais. La masse intrabuccale entrainant une occlusion incomplete de l'oro-pharynx et de la partie a droite du nasa-pharynx fut partiellement excisee sous anesthesie generale. D'apres la consultation du medline, il s'agit du premier cas de syndrome d'Aicardi associe aun Mmangiome palatin.
DiGeorge Syndrome (DGS) is a congenital disorder that affects the thymus, parathyroid glands, and... more DiGeorge Syndrome (DGS) is a congenital disorder that affects the thymus, parathyroid glands, and heart and brain. Thymus involvement in DGS may vary between absence/hypoplasia of thymus to various forms of reduced T cell function. TBX1 deficiency causes a number of distinct vascular and heart defects, suggesting multiple roles in cardiovascular development, specifically, formation and growth of the pharyngeal arch
A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric openi... more A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric opening, a foreshortened colon, imperforate anus, and meningocele is presented. The surgical management of this disorder is discussed and available literature is reviewed. The patient was successfully treated with a combined, single-stage surgical correction.
Turk Pediatri Arsivi-turkish Archives of Pediatrics, Jun 1, 1999
Although various methods of treatment are described for complicated hemangiomas the treatment mod... more Although various methods of treatment are described for complicated hemangiomas the treatment modalities of these are still controversial Between 1993 and 1997 fifteen infants with complicated cutaneous hemangiomas have prospectively been treated by intralesional corticosteroid injections triamcinolone dexamethasone and short term oral prednisolon therapy The localizations of the hemangiomas were as follows: eyelid 3 upperlimb 2 retroauricular 2 labium major 3 forehead 1 lower limb 1 submandible 1 neck 1 and forearm 1 The indications for treatment were; ulceration 2 bleeding 2 infection 3 ambliopy 3 unesthetic apperance 2 and both infection and bleeding 3 The moan age of the patients was 7 4 3 2 months 2 5 12 months and all of them were female No visceral hemangioma and platelet trapping were determined in any case An average of 2 9±1 2 intralesional injections were performed Mean regression obtained was 75 8±19 4 13 patients The remaining two hemangiomas were unchanged The patients did not present with early or late complications in their follow up period with an avarage duration of 27 months As a result intralesional corticosteroid injections with short term oral prednisolone treatment are effective modes of therapy for complicated cutaneous hemangiomas in infants Key words: Cutaneous Hemangioma Corticosteroid Infant
IntroductionTransanal endorectal pull-through (TEPT) is the latest advancement in the treatment o... more IntroductionTransanal endorectal pull-through (TEPT) is the latest advancement in the treatment of Hirschsprung’s disease (HD). The aim of this study was to evaluate the safety and efficacy of TEPT as a definitive treatment for patients with HD.Patients and methodsA retrospective study of 50 patients who underwent TEPT at Uludağ University, Bursa, Turkey, between June 2001 and April 2012 was conducted. These patients were evaluated with regard to their age, sex, preoperative findings, and length of the aganglionic segment, intraoperative and postoperative complications, and results of the follow-up.ResultsForty-three patients were boys and seven were girls. The median age was 3 months (range 0–96 months). The most common symptoms were abdominal distention and vomiting. HD was diagnosed in 38 patients using barium enema and anorectal manometry, in 11 patients using rectal biopsy, and in 1 patient using laparotomy with biopsy. The mean time from the beginning of enteral feeding was 2.2 ± 1.1 days. The mean follow-up period was 26.7 ± 20.8 months. The postoperative complications included transient perianal excoriation in 12 patients, enterocolitis in 10, anastomotic stricture in 3, soiling in 3, recurrent constipation in 2, prolapse of the pulled through colon in 1, anastomotic leak in 1, and rectovestibular fistula in 1 patient.ConclusionsTEPT is a feasible and safe procedure in children with rectosigmoid HD.
European Journal of Pediatric Surgery, Oct 1, 1998
Ileal duplication cysts within a giant omphalocele are very rare. Only a few cases have been repo... more Ileal duplication cysts within a giant omphalocele are very rare. Only a few cases have been reported in the English literature (4). We report one case of giant omphalocele, which included a huge ileal duplication cyst, detected by prenatal US, and diagnosed at surgery after birth. This case illustrates the diagnostic and therapeutic problems occurring during pregnancy and the neonatal period.
The solitary rectal ulcer syndrome (SRUS) is an unusual disorder in childhood. Although well reco... more The solitary rectal ulcer syndrome (SRUS) is an unusual disorder in childhood. Although well recognized in adult literature, the pediatric experience with this condition is limited, so SRUS often goes unrecognized or misdiagnosed. There are very few pediatric case reports in the English literature. This report describes four patients who presented with rectal bleeding, constipation, mucous discharge, and lower abdominal pain, with a diagnosis of SRUS. The diagnosis was made by rectoscopy, defecogram, anorectal manometry and histopathological evaluation. In two patients, defecogram showed a rectocele with both, the sphincter failed to relax to voluntary squeeze pressure on anorectal manometric examination. The histopathological finding in all patients was fibrous obliteration of the lamina propria with disorientation of muscle fibers. All of the patients responded well to conservative therapy, which included defecation training, laxatives, sulfasalazine, and application of rectal sucralfate enema, and remained asymptomatic on the follow-up. Although rare in the pediatric population, SRUS should be relatively easy to recognize in the child with rectal bleeding, after elimination of other causes. If suspected, the diagnosis of SRUS may be made at endoscopy and confirmed by rectal biopsy.
The frequencyof additional congenitalanomaliesin associationwith oesophageal atresia(OA) is about... more The frequencyof additional congenitalanomaliesin associationwith oesophageal atresia(OA) is about50% (1–4). The most commonly associatedmalformations are other atresiasof the gastrointestinaltract, imperforateanusandVATER (vertebraldefects,anal atresia, tracheoesophageal fistula with esophagealatresia,radial and renaldysplasia)complex(4,5). Hypertrophic pyloric stenosis(HPS) complicatingthe postoperative period of OA and tracheoesophageal fistula (TEF) is lesswell known(6). In this paperwe presenttwo cases of OA and TEF, whose postoperativecourseswere complicatedwith HPS.
European Journal of Pediatric Surgery, Dec 1, 1999
Three experimental groups have been constructed to evaluate the long-term outcome of fetal adrena... more Three experimental groups have been constructed to evaluate the long-term outcome of fetal adrenal transplantation in rats. In Group 1 (n = 10), rats underwent bilateral adrenalectomy. In Group 2 (n = 10), rats underwent a sham procedure which included flank incision and arterial canulization. In Group 3 (n = 20), a left adrenalectomy was performed followed immediately by transplantation of a fetal adrenal graft into the greater omentum and marked with a prolene suture (Stage 1). One year later, the right adrenal gland of the recipient was removed followed by a determination of the fetal adrenal graft function (Stage 2). Graft function was evaluated by measuring ACTH and corticosterone levels; and a histologic examination of the transplanted fetal adrenal gland was obtained at autopsy. In Group 1, all the rats died within first 8 hours, following bilateral adrenalectomy. In Group 2, and Group 3, all rats survived after Stage 1 operation. During the Stage 2 operation, it was observed that three rats (15%) had neither fetal adrenal transplant nor prolene suture, seven rats (35%) had a well vascularized and developed fetal adrenal graft and a prolene suture. There was no visible fetal adrenal graft but the prolene suture was present in the remaining rats (50%) in Group 3. After removal of the right adrenal gland (6 and 12 hours later), the mean plasma level of ACTH increased with a decline in mean serum corticosterone level in Group 3 compared to the sham-operated animals (p < 0.001). In spite of visible, and viable transplants, all rats died within 48 hours following Stage 2 operation. The mean weight of the fetal adrenal transplant showed a sixteen-fold increase compared to the initial weight (p < 0.001) and histologic examination showed all 3 zones of adrenal cortex, but there were no medullary cells noted. Although the transplanted fetal adrenal grafts survived, their hormonal function was not enough to maintain host viability. Based on these results it is concluded that, insufficiency of the transplanted fetal adrenal gland may be secondary to either graft rejection or suppression of the transplanted tissue by the functional recipient adrenal despite the fetal adrenal transplant survival.
Journal of International Medical Research, Jul 1, 1997
A total of 24 neonates and infants, aged from 2 days to 10 months, received treatment with intrav... more A total of 24 neonates and infants, aged from 2 days to 10 months, received treatment with intravenous fluconazole for microbiologically documented or presumed fungal infection. The mean fluconazole dosage was 6 mg/kg/day (range 2-16 mgJkg/day) and the mean duration of therapy was 25 days (range 5-72 days). Efficacy was evaluated in neonates with proven fungal infections, as documented by the presence of pathogen at baseline. A positive clinical response was achieved in 23 of the 24 clinically evaluable patients (96%); eradication ofthe fungal organism was also achieved in 23 of the 24 evaluable patients (96%). Adverse events occurred in two patients (8%) but therapy was not discontinued in either patient. The present results confirm the efficacy and safety of fluconazole in the treatment of neonates and infants with severe fungal infections.
Journal of International Medical Research, Sep 1, 1997
Growth and development were monitored for up to 42 months in nine neonates to whom ciprofloxacin,... more Growth and development were monitored for up to 42 months in nine neonates to whom ciprofloxacin, a fluoroquinolone, was given in the neonatal period at a dosage of 20 mg/kg/day. Ciprofloxacin was used only as a 'life-saving' therapy in cases of sepsis produced by bacterial agents resistant to other antibiotics. Two other groups of nine neonates, matched by birth weight and gestational age, were studied as controls: one group with sepsis, which was effectively treated with cefotaxime and a group of healthy neonates. No statistically significant differences in growth and development between the groups were found during follow-up for 42 months. No osteoarticular problems or joint deformities were observed in the ciprofloxacin group. Ciprofloxacin appears to provide a therapeutic option as a 'life-saving' therapy for newborns with sepsis produced by multiply resistant organisms.
Turkish journal of trauma & emergency surgery, Apr 1, 1998
... Çocuklarda kotlar eris/kine oranla daha az mineralize ve daha esnek oidugu için toraks travma... more ... Çocuklarda kotlar eris/kine oranla daha az mineralize ve daha esnek oidugu için toraks travmasi esnasinda kotlar genellikle esner, kirilmaz. ... Pediatric Surgery. Chicago: Yearbook Medical Publishers, 198Ъ. Pp 143 Smyth ВТ. ...
The oromandibular limb hypogenesis syndromes (OLHS), which are congenital malformations involving... more The oromandibular limb hypogenesis syndromes (OLHS), which are congenital malformations involving the tongue, mandible, and the limbs are extremely rare with a few sporadic cases reported in the literature. In this report, the authors report a case of OLHS with gastroschisis, that has not been described previously according to the medline search.
European Journal of Pediatric Surgery, Jun 1, 1999
ABSTRACT A patient, 5 years of age, presented with a swelling on the right side of the neck. Ultr... more ABSTRACT A patient, 5 years of age, presented with a swelling on the right side of the neck. Ultrasonography and computed tomography confirmed a diagnosis of phlebectasia of the right internal jugular vein (IJV). The patient underwent operation and the dilated IJV was excised. Dilatation of the IJV with a Valsalva maneuver suggested a mechanical obstruction in the neck or mediastinum. However, the exact cause is still unknown. Finally, we found the patient to have a thinning of the wall but no other associated abnormality.
European Journal of Pediatric Surgery, Oct 1, 1999
An l-day-old female newborn who had typical elinical features of Aicardi syndrome, such as agenes... more An l-day-old female newborn who had typical elinical features of Aicardi syndrome, such as agenesis of the corpus callosum, ocular abnormalities and infantile spasm associated with a palatal hemangioma is reported. The intraoral mass, which oceluded incompletely the oropharynx and right side of the nasopharynx, was partially excised under Resume Les auteurs rapportent l'observation d'une fillette de 1 jour atteinte du syndrome typique d'Aicardi: agenesie du corps calleux, anomalies oculaires, spasticite infantile et Mmangiome du palais. La masse intrabuccale entrainant une occlusion incomplete de l'oro-pharynx et de la partie a droite du nasa-pharynx fut partiellement excisee sous anesthesie generale. D'apres la consultation du medline, il s'agit du premier cas de syndrome d'Aicardi associe aun Mmangiome palatin.
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