Shiv Prasad Sharma

Twenty years' experience of steroids in infantile hemangioma - a developing country's perspective.

BACKGROUND Hemangioma is a common vascular tumor. Though it involutes spontaneously, results are unpredictable. Steroid therapy is an effective mode of its regression. We present our experience of largest series and possible recommendations for treatment. MATERIALS AND METHODS A total of 2398 patients were treated during the study period of 20 years. They were given oral prednisolone, intralesional triamcinolone, or combination of both as per the protocol and followed for the response. Response to the treatment was graded as excellent, good, poor, or no response. RESULTS The male-to-female ratio was 1:2.3. In 81% of patients, hemangioma was noticed within first month of life. The commonest site of involvement was head and neck (57%). The commonest clinical presentation was discoloration and swelling. Mean age and size were 8.43 +/- 7.04 months and 23.64 +/- 20.13 cm(2). Response rate was highest for superficial type using any modality of treatment. Patients younger than 1 year showed better response (90.3%) in comparison with children older than 1 year (80.8%). The specific complications occurring were infections in 249 (12.4%), cushingoid facies and growth delay in 62 (3.1%), and hypertension in 51 (2.5%) patients. CONCLUSION Steroid therapy either oral or intralesional as per the requirement is an easy and safe modality. Results are good to satisfactory in most patients. The complications are minimal. If treatment is needed, it should be used as a first-line therapy, especially when cost is an important concern.

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.

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

IntroductionFetus 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 presentationA 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 babys 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.ConclusionFetus 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.

Conservative management of ulcerated haemangioma – twenty years experience

Ulceration is the most common complication of haemangioma and occurs in 5–15% of cases. The present study was carried out to evaluate the clinical features of ulcerated haemangioma and efficacy of the management protocol adopted by us over a period of 20 years. All patients with ulcerated haemangioma were evaluated on the basis of age at presentation, sex, rural or urban distribution and site of haemangioma. Treatment included application of topical antibiotic and systemic antibiotic and analgesic for pain. The total number of patients was 608. The male to female ratio was 1: 2·28. The rural:urban distribution was 2·43:1. The most common site of involvement was head and neck. Mean age of patients was 5·60 ± 2·44 months. Mean size of haemangioma and ulceration was 47·30 ± 20·67 cm2 and 7·49 ± 4·52 cm2, respectively. The mean time for ulcer healing was 40·06 ± 19·41 days. Ulcer size of more than 10 cm2 took more time to heal. Response to treatment was satisfactory. Ulcerated haemangioma usually occurs before completion of 1 year of age; hence, every patient with haemangioma needs careful attention. Adequate treatment and regular follow up brings satisfactory response in the patients.

Single-stage repair for rectovestibular fistula without opening the fourchette

BACKGROUND Anorectal malformations are one of the most common congenital defects. This study is conducted to demonstrate new technique for treatment of rectovaginal fistula without disturbing the fourchette through posterior sagittal approach. METHOD All the patients of rectovestibular fistula admitted after the neonatal age were treated with posterior sagittal anorectoplasty without opening the fourchette. The results were evaluated for cosmetic appearance and anal continence. RESULT A total of 40 patients were included in our study. All patients were more than 1 month old. Operative time ranges from 70 to 150 minutes. The cosmetic appearance was good. Anal continence was good in 72% cases and fair in 20% cases. Fifteen percent of patients had minimal constipation and 7.5% patients had mucosal prolapse. CONCLUSION Single-stage repair for vestibular anus through posterior sagittal anorectoplasty without opening fourchette has a good cosmetic appearance and good anal continence.

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

BackgroundA 21 day old male child presented with non bilious vomiting and abdominal mass.Case presentationThis 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.ConclusionThe 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.

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.

Double coin in esophagus at same location and same alignment — a rare occurrence: a case report

Coin is the most common foreign body swallowed by pediatric age group. The multiple coin swallowing is extremely rare and very few cases had been reported in English literature. Most of them were present at different site and had different alignment in the esophagus. The location of the coin (trachea vs. esophagus) is commonly determined by the alignment of the coin on radiographic studies. A 4-year-girl was presented to us with history of coin ingestions one day back without any respiratory distress. On radiological study there was suspicion of two coins on same location and alignment. The diagnosis was confirmed after removal. The both coin was removed successfully by esophagoscopy. Unexpected second foreign bodies in pediatric esophageal coin ingestions are rare and it is mandatory to do post operative radiography after removal to exclude duplex coin or tracheal coin. We are presenting this case because of its rarity, difficulty in diagnosis especially when proper history is not available.

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.

Neonatal necrotising fasciitis – Varanasi experience

Neonatal necrotising fasciitis (NNF) is a rare and fatal disorder. Successful outcome depends on timely intervention. This paper presents single‐centre experience of presentation, management and outcome of the condition. Fifteen patients of NNF were managed during the study period of 5 years. Parameters studied were age, sex, site of involvement and treatment. Treatment included intravenous (i.v.) antibiotics and conservative surgery. Age of presentation was 10–28 days. Male to female ratio was 2:1. Neck and scalp were the commonest site (53·3%). Culture reports were unimicrobial in 66% with predominance of Staphylococcus species. Predisposing factors included rural environment (100%), malnourishment (60%), boils (40%) and scratch injury (13%). Forty per cent had idiopathic NNF. Wound healing was by secondary intention in 46·6% and skin grafting in 53·3%. Overall survival was 80%, while the premature had poorer outcome. NNF is a serious disorder. Early treatment with i.v. antibiotics and supportive measures followed by debridement improves survival.