Gastric teratoma presented as gastric outlet obstruction and melena; report of rare case.

Gastric teratoma is a very rare tumor, accounting for less than 1 % of all teratomas in infants and children. Melena or upper gastrointestinal tract bleeding in newborns and infants is a rare event and is usually caused by a benign lesion. Gastric teratoma has been reported as a cause of gastrointestinal bleeding on a few occasions. As gastric teratomas generally present as a palpable abdominal mass, more aggressive solid masses of childhood must be excluded. We present intramural extension of gastric teratoma presented as symptom of gastric outlet obstruction and melena.

A functional study on small intestinal smooth muscles in jejunal atresia.

AIM: The present study was aimed to assess the contractile status of neonatal small intestinal smooth muscle of dilated pre-atretic part of intestinal atresia to resolve debatable issues related to mechanisms of persistent dysmotility after surgical repair. MATERIALS AND METHODS: A total of 34 longitudinally sectioned strips were prepared from pre-atretic dilated part of freshly excised 8 jejunal atresia type III a cases. Spontaneous as well as acetylcholine- and histamine-induced contractions were recorded in vitro by using organ bath preparations. Chemically evoked contractions were further evaluated after application of atropine (muscarinic blocker), pheniramine (H1 blocker), and lignocaine (neuronal blocker) to ascertain receptors and neuronal involvement. Histological examinations of strips were made by using Masson trichrome stain to assess the fibrotic changes. RESULTS: All 34 strips, except four showed spontaneous contractions with mean frequency and amplitude of 5.49 ± 0.26/min and 24.41 ± 5.26 g/g wet tissue respectively. The response to ACh was nearly twice as compared to histamine for equimolar concentrations (100 µM). ACh (100 µM) induced contractions were attenuated (by 60%) by atropine. Histamine (100 µM)-induced contractions was blocked by pheniramine (0.32 µM) and lignocaine (4 µM) by 74% and 78%, respectively. Histopathological examination showed varying degree of fibrotic changes in muscle layers. CONCLUSIONS: Pre-atretic dilated part of jejunal atresia retains functional activity but with definitive histopathologic abnormalities. It is suggested that excision of a length of pre-atretic part and early stimulation of peristalsis by locally acting cholinomimetic or H1 agonist may help in reducing postoperative motility problems in atresia patients.

Role of propranolol in ulcerated haemangioma of head and neck: a prospective comparative study.

PURPOSE: Infantile haemangiomas comprise the majority of vascular anomalies and are considered the predominant vascular tumour type 1. We performed this prospective study to evaluate the therapeutic response and propranolol tolerance in infants with ulcerated infantile haemangioma of head and neck region. METHODS: Sixty-four patients with ulcerated infantile haemangiomas (IHs) of head and neck region, without any prior treatment and with age older than 1 month, were included in the study, after informed consent was obtained, and were randomly divided into groups A and B. Group A patients were given oral propranolol at a dose of 2 mg/kg per day in three divided doses as outpatients. Group B patients were given oral ibuprofen at a dose of 10 mg/kg 8-hourly and paracetamol at dose of 16.2 mg/kg 8-hourly. Documentation of gender, age, haemangioma location, duration of ulceration and pain was measured on the second and fifth day after commencement of treatment in both groups using the Children's Hospital of Eastern Ontario Pain Scale. RESULTS: There was no difference in pain score between the two groups (P value 0.074). Mean duration of healing of ulceration in group A was 17.93 ± 2.22 days and in group B was 27.71 ± 2.33 days (P value <0.001). In group A, out of 28 patients, 8 (28.5 %) were complete responders, 16 (57.1 %) were partial responders and 4 (14.2 %) were non-responders. CONCLUSION: Propranolol is a valuable therapeutic alternative for treatment of ulcerated haemangiomas and effectively reduces pain.

Management of anorectal malformation without ligation of fistula: an approach preventing posterior urethral diverticula.

OBJECTIVE: The posterior urethral diverticulum is a common urologic complication requiring reoperations in anorectal malformation cases (ARM). We present a series of 24 cases of male ARM managed without ligation of fistula. MATERIAL AND METHODS: A prospective study was conducted between July 2010 and June 2012 including male neonates with ARM, where rectobladder neck and rectoprostatic fistula were approached by the abdominal route. The fistulous tract was dissected to the distal-most possible length and was excised flush with the urethra without its ligation. A per-urethral catheter was placed in situ. A record was made of any features of urinary leak and a micturating cystourethrogram was performed at the 1-year of follow-up. RESULTS: Twenty-four cases of ARM, 16 with rectobladder neck fistula and eight cases with rectoprostatic fistula were included. Of these, 12 had single-stage primary abdominoperineal pull-through and 10 were managed by primary posterior sagittal anorectoplasty. Two cases with colostomy during the neonatal period were managed by laparoscopic assisted anorectoplasty at 6 months. None of the cases had a urinary leak during the postoperative period. All had a normal micturating cystourethrogram at 1 year. CONCLUSIONS: The approach of dividing fistula without ligation may prevent posterior urethral diverticula, but larger long-term follow-up studies are needed.

Propranolol for infantile haemangiomas: experience from a tertiary center.

AIM: Infantile haemangiomas are the most common tumor of infancy. We report the use of propranolol for treatment of problematic and complicated haemangiomas. PATIENTS AND METHODS: Propranolol was given to 52 children with mean age of 18.2 months at onset of treatment. After clinical and electrocardiographic evaluations, propranolol was administered with a starting dose of 2 mg/kg per day, given in 3 divided doses. Monthly follow up was done, response to oral propranolol therapy and any complications of therapy were recorded. Response to propranolol was classified as Complete Response, Excellent Response, Partial Response and Non Responder. RESULTS: Total 49 patients showed significant improvement after propranolol therapy out of which 4 patients were complete responder, 30 patients (56.7%) were excellent responders; 15 patients (28.8%) were partial responders. 3 patients (5.7%) had growth of haemangiomas despite propranolol therapy and were classified as non-responder. Side effect like hypotension, rashes, gastroesophageal reflux was reported by 3 patients. In our study mean duration of treatment was 6.5 months. At the end of treatment propranolol was stopped by with gradual tapering of dose over a period of 2 weeks. CONCLUSION: Propranolol administered orally at 2 mg/kg per day has rapid effective therapeutic effect in treatment of ulcerated haemangiomas and it appears to be a valuable and effective treatment option for infantile haemangiomas beyond the proliferative phase, and esthetically disfiguring haemangiomas.

Tracheoesophageal fistula with esophageal atresia with absent external ear: a unusual association.

Esophageal atresia with tracheoesophageal fistula (TEF with EA) is a common disease which is associated with many other congenital abnormalities. The most common association is with VACTERL syndrome.. Various types of ear malformations have been reported in CHARGE syndrome associated with EA and TEF. However, absence of external ear has not been described which is being reported in this article. We could not found this association even on extensive search of literature.

Long-term follow up of mesenchymal hamartoma of liver--single center study.

BACKGROUND/AIM: Mesenchymal hamartoma of liver (MHL) is a rare liver tumor of childhood. About 200 cases have been reported till now. Most of the work on MHL is limited to case reports and there are not many long term follow-up studies. We present our 20 years of experience with this uncommon entity. This study aims to highlight clinical features, diagnosis and treatment of MHL. MATERIALS AND METHODS: All patients with a diagnosis of MHL in last 20 years were included in this retrospective study. The patients were evaluated clinically, radiologically and pathologically. RESULTS: The total number of patients with a diagnosis of MHL was nine. Mean age of the patients was 19.89 ± 2.75 months. Right lobe was involved in eight patients. The prominent clinical features were distension of abdomen and anorexia. Surgical options used were hepatic lobectomy, wedge resection and enucleation. Histopathology of the specimens showed cysts of variable size with normal hepatocytes, bile ducts and connective tissue stroma. Overall mortality was one (11.11%). CONCLUSION: MHL is a benign tumor that can present with various clinical features. It should be differentiated carefully from other liver masses especially malignant ones. The diagnosis can be made with the help of radiology and histopathology. Adequate resection is curative in most of the cases and long-term follow up is satisfactory.

High anorectal malformation in a five-month-old boy: a case report.

INTRODUCTION: Anorectal malformation, one of the most common congenital defects, may present with a wide spectrum of defects. Almost all male patients present within first few days of life. CASE PRESENTATION: A five-month-old baby boy of Indian origin and nationality presented with anal atresia and associated rectourethral prostatic fistula. The anatomy of the malformation and our patient's good condition permitted a primary definitive repair of the anomaly. A brief review of the relevant literature is included. CONCLUSION: Delayed presentation of a patient with high anorectal malformation is rare. The appropriate treatment can be rewarding.

Evaluation of topical steroids in the treatment of superficial hemangioma.

Infantile hemangioma is a common disease. Steroids have been used for its treatment; however, intralesional steroids cause pain and other problems. A treatment modality that can avoid these problems is desirable. The authors evaluated the role of topical steroids as an alternative to intralesional steroids in the treatment of superficial hemangioma. Inclusion criteria were <2 superficial type ofhemangiomas <5 cm. The topical steroid mometasone furoate was applied twice daily. Intralesional triamcinolone acetonide was injected at monthly intervals using a 24-gauge needle at doses of 1 to 2 mg/kg. Forty-five (86.5%) patients responded to treatment with the topical steroids, of which 50% had excellent and 36.5% had good response. In the intralesional group, the response rate was 95.7%, of which 63.8% had excellent and 31.9% had good response. Complications in the topical steroid group were mild itching and irritation (19.2%) and hypopigmentation (7.6%). Complications in the intralesional group were pain (100%), bleeding (17%), infection (17%), cutaneous atrophy (8.5%), cushingoid facies (2.1%), and growth retardation (2.1%). Topical steroids are a reasonably good alternative to intralesional steroids as an initial choice for treating superficial hemangioma.

Twin fetus in fetu in a child: a case report and review of the literature.

INTRODUCTION: Fetus in fetu is an extremely rare condition wherein a malformed fetus is found in the abdomen of its twin. This entity is differentiated from teratoma by its embryological origin, its unusual location in the retroperitoneal space, and the presence of vertebral organization with limb buds and well-developed organ systems. The literature cites less than 100 cases worldwide of twin fetus in fetu. CASE PRESENTATION: A two-and-a-half-month-old Asian Indian baby boy had two malformed fetuses in his abdomen. The pre-operative diagnosis was made by performing an ultrasound and a 64-slice computer tomography scan of the baby's abdomen. Two fetoid-like masses were successfully excised from the retroperitoneal area of his abdomen. A macroscopic examination, an X-ray of the specimen after operation, and the histological features observed were suggestive of twin fetus in fetu. CONCLUSION: Fetus in fetu is an extremely rare condition. Before any operation is carried out on a patient, imaging studies should first be conducted to differentiate this condition from teratoma. Surgical excision is a curative procedure, and a macroscopic examination of the sac should be done after twin or multiple fetus in fetu are excised.

Pouch colon associated with anorectal malformations fails to show spontaneous contractions but responds to acetylcholine and histamine in vitro.

PURPOSE: Congenital pouch colon (CPC) associated with anorectal malformation (ARM) is most commonly reported from Northern India. So far, no physiologic study comparing the detailed contractile status of CPC with non-CPC conditions are available. The present article deals with the contractile study and histopathologic observations in CPC, which may be useful for better surgical management. METHODS: Freshly excised 12 neonatal CPC and similar number of non-CPC (control) specimens were transferred to ice-cold (4 degrees C-6 degrees C) Krebs-Ringer solution bubbled with 100% oxygen. Longitudinally prepared 2 to 4 colonic strips were obtained from central part of each specimen and subjected to the contraction recording after exposure to cumulative concentrations of acetylcholine (ACh) and histamine. Acetylcholine-induced contractions were evaluated after application of atropine (muscarinic blocker), and histaminergic contractions were recorded after pheniramine (H(1) blocker), lignocaine (neuronal blocker), and atropine. Histopathologic observations were made by using H&E and Masson trichrome stains. RESULTS: Control specimens showed spontaneous contractions, but CPC strips did not. Both control and CPC responded to ACh and histamine. The response to histamine was greater (P < .05) in CPC as compared to control, whereas the response to ACh was more (P < .05) in control. In CPC, response of histamine (100 micromol/L) was blocked by pheniramine (0.32 mmol/L) and lignocaine (4 mmol/L) by 97% and 80%, respectively, and enhanced by 57% after preapplication of atropine (10 micromol/L). Acetylcholine (100 micromol/L)-induced contractions were attenuated (86%) in presence of atropine. Histopathologic examination showed fewer mature ganglion cells with various changes in muscle layers including fibrosis, disruption, hypertrophy, atrophy, and constriction bands. CONCLUSION: Congenital pouch colon associated with ARM lacks normal spontaneous contractions but retains ACh and histamine-induced contractility. In view of the functional and histologic abnormalities, we propose that CPC associated with ARM is an abnormally functional and developed tissue. Therefore, resection of the pouch should be considered for better functional outcome of the remaining bowel.

A child presented with bilateral congenital constriction ring in lower extremity: a case report.

INTRODUCTION: The congenital constriction ring of lower extremity is very uncommon and rare condition. The actual incidence in general population is not known. In English literature, very few cases are reported time to time as congenital constriction band syndrome associated with musculoskeletal disorder like congenital talipes equino varus. The lesion can involve skin only or goes to deeper structure up to bone, which can lead to gangrene of foot or auto amputation. CASE PRESENTATION: We are presenting a case of bilateral congenital constriction ring in lower limb who presented at age of 4 year without any other associated congenital anomaly, simply managed by Z-plasty, which improves quality of life after physiotherapy. CONCLUSION: Congenital constriction ring of lower limb is extremely rare condition in children. Early diagnosis and management is mandatory, either in single stage or by stage procedure, to prevent auto-amputation of limb and to improve quality of life on feet.

Sutureless skin closure with isoamyl 2-cyanoacrylate in pediatric day-care surgery.

Importance of day-care surgery in pediatric age group is ever increasing and nowadays bulk of surgery is performed as a day-care procedure. Day-care procedure requires short intra-operative period and less postoperative complications. The introduction of tissue adhesive has been received enthusiastically since they may result in equivalent tensile strength-improved cosmetic appearance of the scar and lower infection rate when compared to sutures, staples and adhesive tape. We have used isoamyl 2-cyanoacrylate as a tissue adhesive for skin closure in day-care surgery. Novocryl is ideal for such procedure as it is a sterile, inert, non-toxic, biocompatible and bacteriostatic liquid topical skin adhesive containing isoamyl 2-cyanoacrylate formulation. We applied isoamyl 2-cyanoacrylate glue in 100 patients over skin cut margin after stitching subcutaneous tissue. Skin margin were held together by means of skin hook for 1-2 min. The cosmesis was better as there were no suture marks or lumpiness. There were no major complications in our study. Thus, isoamyl 2-cyanoacrylate can be considered as an alternative to skin suturing in the pediatric day-care surgery.

Noncommunicating isolated enteric duplication cyst in childhood.

Duplications of the alimentary tract are spherical or tubular structures lined by epithelium similar to intestine that are firmly attached to or share the wall of the alimentary tract and have a common blood supply with the adjacent segment of the bowel. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply and do not communicate with the intestine. There are only 4 cases reported in the English literature-3 in childhood and 1 in an adult, and all are male. We report a case of noncommunicating isolated ileal duplication cyst in a 3-year-old female child that presented as a mass in the right iliac fossa, mimicking an intussusception. The duplication had its own blood supply arising from the terminal ileal mesentery. This report describes the first female patient with this condition and reviews the English literature.

Duplication cyst of pyloroduodenal canal: a rare cause of neonatal gastric outlet obstruction: a case report.

BACKGROUND: A 21 day old male child presented with non bilious vomiting and abdominal mass. CASE PRESENTATION: This case is reported because pyloroduodenal duplication cysts are an extremely rare congenital anomaly, whose clinical presentation often mimics those of hypertrophic pyloric stenosis. Ultrasound examination showed cystic mass at pyloric region and barium study was suggestive of extrinsic mass compressing the pyloric region. A laparotomy, a tense cystic mass was present at the pyloroduodenal junction (PDC) which was resected and end to end anastomosis was done. Patients followed an uneventful recovery and doing well. CONCLUSION: The clinical and radiological analysis can reveal configurational changes consistent with a large extrinsic mass rather than muscular hypertrophy and can lead to accurate preoperative diagnosis.

A pilot study on the role of T-tube in typhoid ileal perforation in children.

BACKGROUND: Ileostomy is usually performed for patients of typhoid intestinal perforation with poor general condition, but it is associated with significant morbidity. We have used the T-tube in such patients as an alternative to ileostomy. METHODS: This is a prospective evaluation of a cohort of children with proven typhoid intestinal perforation. Patients with multiple perforations and poor general condition were managed with a T-tube inserted into the bowel lumen after closing all distal perforations (group 3). They were compared with patients who had primary closure of perforation (group 1) or bowel resection (group 2) to determine the efficacy of the use of T-tube. RESULTS: The total number of patients for groups 1, 2, and 3 was 51, 4, and 12 (n = 67). The mean number of perforations for the three groups was 1, 3.5 +/- 0.58, and 4.25 +/- 0.97. The operation time for the three groups was 37.29 +/- 3.24, 59.25 +/- 3.09, and 59.17 +/- 4.17 minutes, respectively. The T-tube was removed after 13.17 days. The mean duration of fistula at T-tube site to heal was 8.58 +/- 2.11 days. The overall follow-up period was 10.94 +/- 1.15 months and none of the patients with T-tube placement had features of intestinal obstruction. CONCLUSIONS: In children with multiple typhoid intestinal perforations and poor general condition, the use of T-tube may be an effective management option.

Use of fibrin glue in preventing urethrocutaneous fistula after hypospadias repair.

UNLABELLED: Urethrocutaneous fistula is one of the most common complications after hypospadias surgery. The incidence of fistula development has varied from 4% to 20% in larger series. We sought to investigate the role of fibrin glue (Tisseel manufactured by Baxter India Pvt Ltd, Chennai, India) to reduce the chances of fistula formation in cases in proximal penile hypospadias. METHOD: A total of 120 patients with proximal penile hypospadias (patients having urethral meatus at posterior third of penile shaft and at penoscrotal junction) were included in the present study. Patients were randomly allocated into 2 groups of 60 each by using Strata 9 software random number table. In group A, fibrin glue was used as a sealant after hypospadias surgery, whereas in group B, no sealant was used. All the operations were performed by single surgeon using transverse preputial tubularized island flap urethroplasty. RESULT: Fistula formation occurred in 6 cases in group A (10%) and 19 cases in group B (32%) (P = .027). The fistulae observed in fibrin glue group A were single and small in size (<1 mm). Multiple (>or=2 fistulae) and larger fistulae (>2 mm) were observed in group B. Overall complication was significantly higher in group B (P = .006). CONCLUSION: Fibrin glue in hypospadias repair does not eliminate fistula formation. However, it seems that it minimizes the incidence of fistula formation.

Evaluation and management of infantile hemangioma: an overview.

Infantile hemangiomas are the most common tumors of infancy and commonly last 7 to 9 years. Although their pathophysiology is not completely understood, recent studies have provided useful information regarding differential diagnosis, assessment approaches, and management options. To enhance understanding of infantile hemangiomas and highlight recent advances in knowledge of pathophysiology and newer diagnostic and therapeutic modalities, a literature search of pertinent articles published between 1985 and 2006 in PubMed was conducted using the term hemangioma with the words types, pathophysiology, treatment, and complications. Despite the generally benign nature of the condition, treatment is required for cosmetic and psychological reasons and requires differential diagnosis, particularly with regard to congenital hemangiomas and vascular and non-vascular tumors, to determine cause and address potential complications such as ulceration. Various emerging treatment modalities are available (interferon, vincristine, cyclophosphamide, bleomycin, imiquimod, becaplermin, and laser therapy) but steroids remain the treatment of choice in cases when hemangiomas require intervention. Overall prognosis in terms of healing hemangiomas using conservative measures is good. Additional research to better understand the pathophysiology and role of genetics is needed. Emerging treatment options should be evaluated through controlled studies to assess effects and side effects.