Sandwich therapy in the management of propranolol resistant infantile hemangioma of the lip.

RATIONALE: Propranolol has evolved as a first line management of infantile haemangiomas. Nevertheless, the resistance to propranolol has warranted alternative treatment modalities. We hypothesize that combination of medical and surgical therapy (sandwich therapy) to be beneficial in the management. METHODS: Patients were divided into three groups (total=31), Group A (bleomycin sclerotherapy), Group B (surgical group), Group C (sandwich therapy). A p value <0.05 was taken as significant. RESULTS: In Group A 54.5% of patients had excellent response, 27.3% partial response and 18.2% had no response. Comparing cosmetic outcome, 25% of patients in Group B and 16.7% of patients in Group C had excellent upshot. On the contrary 18.2% of patients in bleomycin had unsatisfactory and 18.2% had poor cosmetic outcome. Sandwich therapy was cosmetically more acceptable than Bleomycin sclerotherapy (p- value = 0.049). In terms of recurrence, sandwich therapy differed significantly from surgical therapy with fewer recurrences (p-value= 0.049). CONCLUSION: Sandwich therapy is superior to bleomycin sclerotherapy in terms of cosmetic outcome and has fewer recurrences compared to surgical group and requires single stage resection. However, further studies with the help of Doppler ultrasound are required in this regard.

Modified Laparoscopic Excision of Choledochal Cyst: Technique and Early Results.

BACKGROUND: Choledochal cyst is a common congenital anomaly requiring surgical treatment. Nowadays, laparoscopic excision is the preferred approach. We studied a modification in the classical laparoscopic approach to facilitate the dissection of a cyst. MATERIALS AND METHODS: A prospective comparative study was done on 42 Type I choledochal cyst children. One group was operated by classical laparoscopic technique, while the other group was operated by modification of classical technique by deliberately opening the cyst wall and dividing the cyst into two hemi-cups, followed by dissection and excision. The intraoperative and postoperative parameters were assessed in both the groups. RESULTS: The age, gender ratio, clinical presentation, and cyst diameter were comparable in both the groups. There was a significantly higher success rate (95.7% vs. 73.7%, P = 0.042) and lesser time for cyst excision (96.43 ± 12.15 vs. 120.91 ± 17.38 min P < 0.001) in the modified technique when compared to the classical technique. Further in three patients, it was possible to convert the classical procedure to a modified technique and complete the cyst excision. The postoperative outcomes were similar in both the groups. CONCLUSION: The modified laparoscopic excision shortens the operative time with higher success rate and comparable short-term morbidity vis-a-vis classical laparoscopic technique.

Endoscopic Third Ventriculostomy in Infants Less than One Year of Age: A Short Series of 14 Cases.

BACKGROUND: Endoscopic third ventriculostomy (ETV) is currently considered as an alternative to cerebrospinal fluid (CSF) shunt systems in the treatment of obstructive hydrocephalus. This procedure allows the CSF to drain in the basal cisterns and reabsorbed by arachnoid granulations, and avoiding implantation of exogenous material. AIMS AND OBJECTIVES: The purpose of this study was to assess the success rate of ETV in infants less than 1 year of age with congenital noncommunicating hydrocephalus. MATERIAL AND METHODS: This study was a 2-year prospective study from August 2017 to July 2019. ETVs were performed in 14 patients younger than 1 year with diagnosis of noncommunicating hydrocephalous. A failure was defined as the need for shunt implantation after ETV. Phase-contrast MRI of the brain was done after 6 months to see patency of ETV fenestration and CSF flow through ventriculostomy. RESULTS: ETV was tried in 18 patients and successfully performed in 14 patients. Out of the 14 patients, shunt implantation after ETV was performed in 3 patients (failed ETV). In the successful cases, etiology was idiopathic aqueductal stenosis in 8, shunt complications in 2, and 1 case was a follow-up case of occipital encephalocele; the mean age was 7.7 months (range 3-12). In the 3 failed cases, etiology was aqueductal stenosis, mean age was 7.6 months (range 3-11). In all ETVs, failed patients MPVP shunting was done. Follow-up of nonshunted patients was done from 6 to 24 months (mean 15 months). There was no mortality or permanent morbidity noted following ETV. CONCLUSION: ETV is a good surgical procedure for less than 1-year-old children.

A Clinicopathological Study to Assess the Role of Intralesional Sclerotherapy Following Propranolol Treatment in Infantile Hemangioma.

Context: As propranolol has emerged as first-line therapy for problematic infantile hemangioma, the number of non-responders and partial responders to propranolol therapy is also increasing. Aims: The study was conducted to evaluate the response of intralesional bleomycin, triamcinolone, and a combination of both as second line of treatment for the residual hemangioma following propranolol therapy. Settings and Design: A prospective comparative study was conducted in patients who were either non-responders or partial responders to previous propranolol treatment. Materials and Methods: The patients randomly received injection bleomycin, injection triamcinolone, and combination of both bleomycin and triamcinolone. The response to treatment was recorded clinically by using photographs. The pathological response was assessed by calculating pre-treatment and post-treatment microvessel density in biopsy of lesion from the non-cosmetic sites using immunohistochemistry. Statistical Analysis Used: χ2 test was used to test the association between the variables. The utility of microvessel diameter (MVD) in terms of clinical response to the therapy was predicted by using receiver operating characteristic (ROC) curve. Results: Out of the 134 patients, 42 received bleomycin and 44 received triamcinolone and were treated with a combination of both. The overall clinical response was better in the combination group compared with the bleomycin group (P = 0.018) and triamcinolone group (P = 0.0005), respectively, after 6 months of follow-up. There was no difference in clinical response between the triamcinolone and bleomycin groups. Change in MVD correlated with the clinical response. Conclusion: The combination of bleomycin and triamcinolone is effective and safe for the treatment of residual hemangioma.

Role of Urinary Transforming Growth Factor Beta-B1 and Monocyte Chemotactic Protein-1 as Prognostic Biomarkers in Posterior Urethral Valve.

BACKGROUND: Posterior Urethral Valves (PUV) are the most common cause of congenital LUT obstruction in males. Biomarkers of glomerular or tubular injury may be of particular value in predicting the need for surgical intervention or in tracing progression of chronic kidney disease. Measurement of biomarker in urine is relatively easy. AIM: To evaluate the changes in values of urinary Transforming Growth Factor Beta 1(TGF-B1) and Monocyte Chemotactic Protein (MCP-1) before and after valve ablation and its prognostic value in Posterior urethral valve. MATERIAL AND METHOD: This prospective study was conducted from September 2016 to August 2018. The study group included 20 consecutive male babies with the diagnosis of PUV treated and followed up versus equal numbers of age matched control without any renal or urinary tract disease. Pre-operative urine samples were collected in Operative room. Cystoscopy and valve ablation was done. Follow up was done clinically by urinary stream and radiologically with VCUG. Follow-up was planned at 1 month, 3 months and 6 months following cystoscopic valve ablation. All collected urine samples were centrifuged at 10,000 rpm for 20 minutes. Supernatant was collected and two divided aliquots were stored at -200c to be thawed on the day of assay. Optical density of each well was recorded at 450 nm and 540 nm A p-value of <0.05 was considered to be statistically significant. RESULT AND DISCUSSION: Out of 20 cases of PUV, 14 (70%) cases were 1st born males of their family. The median age at the time of valve ablation in PUV cases was 2.5 (1.20-3.87) years.. Most common symptoms are fever and UTI. The preoperative median serum creatinine level was 1.65 mg/dl(1.22-2.42) pre-ablation, and fall significantly after ablation. Median eGFR level (calculated) was 25.635 (16.38-35.40) and after 6 months was 71.490 (45.44-96.93). Preoperative median MCP1 in PUV cases was 147.2 (82.8-512.5) and significant difference was also found in 1st, 3rd and 6th months after surgery (p<0.001, p=0.004 and p=0.002).Preoperative median TGF-B1 level was 197.8 pg/ml (79.9-386.4). There was no statistically significant change in TGF-B1 level at preoperative to 1 month and preop to 3 months after surgery but at 6 months after surgery the median TGF-B1 level significantly decreased as compared with preoperative TGF-B1 level. CONCLUSION: TGF ß1 and MCP1 can be considered as prognostic urinary biomarkers in patients of PUV and can be used to specify and counsel patient's attendant regarding possibility of ESRD and need for further intervention.

Three-Layered Closure of Persistent Oroantral Fistula Using Chin Graft, Buccal Fat Pad, and Buccal Advancement Flap: A Case Report with Review of Literature.

Various techniques have been used for the repair of oroantral fistula (OAF) but majority of them have focused on the soft tissue closure alone, and most of the time, the osseous floor of the sinus was ignored. Existing literature supports that bone grafts supported by Buccal Fat Pad (BFP) heal well without undergoing significant resorption and necrosis. Through this case report, we wish to elaborate on the clinical success of using BFP and autogenous chin graft for simultaneous reconstruction of a large long-standing oroantral fistula with underlying osseous defect. The combination technique can prove beneficial for osseous regeneration of sinus floor and improve chances for future implant prosthetic rehabilitation.

Modified Cervicofacial Flap for Large Cheek Defect Reconstruction under Local Anesthesia.

The cervicofacial flap was first described in 1969. For the past several years, it has been the flap of choice for the reconstruction of facial defects especially cheek defects. In recent years, with the advent of microvascular free flap tissue transfer, the use of a cervicofacial flap has been sparse. This article highlights the importance and application of locoregional flaps such as a cervicofacial flap in the reconstruction of soft tissue defects on a face. This case was unique from the reconstruction point of view. An 81-year-old patient presented to us with a long standing ulcerated growth in his left cheek which was neglected due to lack of symptoms and his poor financial condition. Clinically, the extent was defined from the suborbital skin region till the mid cheek region and medially from the lateral nasal crease up to the cheek prominence area. An initial biopsy suggested basal cell carcinoma. The lesion was excised, and a 4.5 × 5.5 cm defect was reconstructed using a modified "cervicofacial" flap under local anesthesia. Our experience shows that this technique is a reliable, easy to harvest flap with good outcomes. Moreover, it can be utilized in those candidates not suitable for a free flap procedure under general anesthesia.

Homocysteine and vitamin B12: Other causes of neural tube defects in Eastern Uttar Pradesh and Western Bihar population.

BACKGROUND: Neural tube defects (NTDs) are congenital malformations with an incidence of 1-10/1000 live births. Homocysteine and vitamin B12 metabolism have been shown to be associated with NTDs. AIM: To investigate the status of maternal and neonate's folic acid, homocysteine, and vitamin B12 levels and their association with the risk of development of NTDs in the population of Eastern Uttar Pradeshand Western Bihar, India. MATERIALS AND METHODS: This study is a cross-sectional, retrospective study where 96 mothers who either had a first NTD child or had a history of NTD child in the family and 126 neonates with spina bifida were recruited during the period 2012-2015. Eighty-four control mothers whose previous and current pregnancies were normal, and 87 control neonates who had no defects and were within the same age range as the NTD affected neonates, recruited from the department of pediatric surgery, were enrolled in the study. Plasma concentrations of folic acid, vitamin B12, and homocysteine were compared between cases and controls. RESULTS: The folic acid level in the mothers and neonates was within the normal limit. A significant increase in the level of homocysteine in mothers with affected pregnancy and in neonate cases in comparison to control mothers was obseved. Further, a significant decrease in the level of vitamin B12 in mothers with NTD neonates and in the affected neonates was noted. A negative correlation was found between homocysteine and vitamin B12 levels in case and control mothers. CONCLUSION: A correlation of an increase in serum homocysteine with a decrease in vitamin B12 was seen in mothers of neonates with NTD. A similar observation as made in the neonates with NTDs. It may be suggested that maternal decrease in vitamin B12, in mothers who have normal folic acid may be associated with NTD in their children.

Intradural teratoma in a neonate with meningomyelocele.

Meningomyelocele is a common congenital problem. The teratoma is a neoplasm composed of tissues foreign to the part in which they arise. An intradural teratoma within a meningomyelocele is a very rare association. We report a case of intradural teratoma with a brief review of the relevant literature.

Primary Eosinophilic Gastritis in a Child with Gastric Outlet Obstruction.

A 3-year-old girl presented with multiple episodes of vomiting, fever, and hematemesis for the past 2 months. Except for hemoglobin, her rests of the laboratory tests were unremarkable. Her barium X-ray showed absence of the duodenal bulb and the C-loop. Her endoscopy showed deformed stomach with multiple ulcers and diverticuli. The gastric outlet was not visualized. Distal gastrectomy with gastro-duodenal anastomosis was performed. Histopathological findings revealed transmural dense infiltrates of eosinophils, consistent with eosinophilic gastritis.

Single stage management of a unique variant of congenital pouch colon with triplet fistula and normal anus.

Congenital pouch colon (CPC) in the female patient presents with highly variable and anomalous anatomy. We herein report the first case of CPC with uterus didelphys having normal anal opening, H-type vestibular fistula, two other fistulous communications between pouch colon and two vagina managed in a single stage with excellent postoperative outcome.

Novel technique of repair of large tracheo-esophageal fistula following battery ingestion in children: review of two cases.

Surgical repair of acquired tracheo-esophageal fistula may result in tracheal stenosis or esophageal stricture. We used fistula with esophageal cuff as flap to repair the tracheal defect. Esophageal repair was performed by rotating ends through 90° in opposite direction. This technique offers excellent repair in a single stage.

Clinico-immunological response to intratumoral versus intravenous neoadjuvant chemotherapy in advanced pediatric solid malignancies.

BACKGROUND: There is minimal literature on the use of intralesional chemotherapy in the pediatric age group. We undertook this present study to evaluate the two modalities (intratumoral and intravenous) of giving chemotherapy in terms of toxicity of chemotherapy, hematological parameters, efficacy of chemotherapy in reduction in volume of the tumor as well as resectability of tumor with special emphasis on immunological parameters. MATERIALS AND METHODS: Advanced cases of Wilms' tumor and Neuroblastoma were included in the study. Intratumoral chemotherapy was given through 25 G spinal needle under aseptic precautions and ultrasound guidance in the same dose as in systemic chemotherapy. Intravenous group was given chemotherapy in the usual way. Reassessment was carried out after every course of chemotherapy. RESULTS: Group A included 16 cases of Wilms' tumor and 6 cases of neuroblastoma. In group B, there were 14 cases of Wilms' tumor and 8 of neuroblastoma. Vomiting, diarrhea, mucositis, and thrombophlebitis were more common in the intravenous group (P<0.05). The fall in Immunoglobulin A, Immunogloblulin G, Immunoglobulin M, and T-cell rosetting was more common in the intravenous group (P<0.05). Seventy percent of patients had completely resectable tumor at the end of 6 doses of intratumoral chemotherapy as compared to 50% resectability in the intravenous group (P<0.05). CONCLUSION: Intratumoral chemotherapy, besides causing less of the adverse effects and increasing the resecability rate, also causes less suppression of the immune system. This may be offered as an alternative safe and effective modality of treatment for advanced solid tumors.

Hemangioma: review of literature.

Hemangiomas are tumors identifed by rapid endothelial cell proliferation in early infancy, followed by involution over time. All other abnormalities are malformations resulting from anomalous development of vascular plexuses. The malformations have a normal endothelial cell growth cycle that affects the veins, the capillaries or the lymphatics and they do not involute. Hemangiomas are the most common tumors of infancy and are characterized by a proliferating and involuting phase. They are seen more commonly in whites than in blacks, more in females than in males in a ratio of 3:1.

Clinical spectrum of neural tube defects with special reference to karyotyping study.

BACKGROUND: Neural tube defects are common congenital malformations of the central nervous system. Despite years of intensive epidemiological, clinical, and experimental research, the exact etiology of NTD remains rather complex and poorly understood. The present study attempted to look into the association of occurrence of NTD with reference to folic acid levels, along with karyotyping status. MATERIALS AND METHODS: Detailed history was taken with emphasis on age of the baby and mother, parity, antenatal folic acid intake. Five milliliters of blood was drawn from all the babies and their mothers and divided equally in preheparinized vials (for karyotyping) and plain vials (for folic acid estimation). The total duration was 2 years. RESULTS: The total number (n) in the study group was 75. The folic acid level was less in affected babies and their mother when compared to matched controls. Chromosomal defect was observed in nine of the 75 patients. Karyotyping defects were higher in children born to mothers of the age group 31-40 years and when their birth order was second. CONCLUSION: Folic acid supplementation needs to be continued to prevent the occurrence of NTD, and the perinatal identification of NTD should alert one to the possibility of chromosomal abnormalities and prompt a thorough cytogenetic investigation and genetic counseling.

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.

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 cm(2) and 7.49 +/- 4.52 cm(2), respectively. The mean time for ulcer healing was 40.06 +/- 19.41 days. Ulcer size of more than 10 cm(2) 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.

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.