Tongue flap as salvage procedure for recurrent and large palatal fistula after cleft palate repair.

BACKGROUND: Recurrent palatal fistula is a common complication of cleft palate repair. The main causes are poor surgical technique or vascular accidents and infection. Local flaps are not adequate for larger and recurrent fistula. The aim of this study is to analyze the utility of tongue flap in recurrent and large palatal fistula repair. MATERIALS AND METHODS: From January 2008 to July 2016, 18 patients with recurrent palatal fistula were included in the study. All the patients had undergone repair of cleft palate and fistula previously. Tongue flap repair of the recurrent palatal fistula was performed in all 18 patients. The flaps were divided after 3 weeks and final inset was done. Flap viability, fistula closure, residual tongue function, esthetics, and speech impediment were assessed. RESULTS: In all the patients, fistula could be closed primarily by tongue flap. None of the patients developed flap necrosis while flap dehiscence and bleeding were observed in one patient each. No functional deformity of the tongue and donor-site morbidity was seen. Speech was improved in 80% cases. CONCLUSION: The central position, mobility, excellent vascularity, and versatility of tongue flap make particularly suitable choice for the repair of large fistula in palates scarred by previous surgery. It is very well tolerated by children. We, therefore, recommend tongue flap for large and recurrent palatal fistula in children.

Intussusception in a premature neonate: A rare and often misdiagnosed clinical entity.

Intussusception is a very rare cause of intestinal obstruction in neonates. It is of extremely rare occurrence among premature neonates. We present a case of 11-day-old premature neonate who presented with abdominal distension, intolerance to feeds, vomiting, significant bilious aspirate and bleeding per rectum. The initial diagnosis of necrotizing enterocolitis (NEC) led to a delay in the diagnosis. On exploratory laparotomy, it turned out to be a case of ileo-colic intussusception with Meckel's diverticulum as a lead point. This site of intussusception (ileo-colic) and presence of a lead point among premature neonate is of exceedingly rare occurrence and very few such cases have been reported. In this article, the published work about clinical features and management on intussusceptions in premature neonates has been reviewed. The authors intend to highlight the difficulty in distinguishing the NEC and intussusception. Subtle clinical and radiological features which can help in differentiating the two conditions have been emphasized. This can avoid the delay in diagnosis and management which can prove critical. High index of suspicion with timely intervention is the key for optimizing outcome. A diagnosis of intussusception should always be considered in any preterm infant with suspected NEC.

Neonatal rhabdomyosarcoma: an unusual presentation.

Though rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in the paediatric age group, its occurrence in new born is rare. Often it presents as a large mass arising from anywhere in the body, most common site being head and neck region. Here we are describing a case of neonatal embyonal RMS with multiple subcutaneous nodules.

Minimal access surgery in newborns and small infants; five years experience.

AIMS AND OBJECTIVES: The aim of this study was to assess and present the outcome (initial experience and lessons learnt) of minimally invasive surgery for various indications in neonates and small infants (< 5 kg) at a single medical centre. MATERIALS AND METHODS: A retrospective analysis was performed on 65 patients (age day 2 to 10 months) managed with minimal access surgery (MAS) for various indications, between 2005 and 2010. We analyzed demographic information, procedures, complications, outcomes, and follow-up and overall feasibility of the procedure. RESULTS: No serious complications except one death in congenital diaphragmatic hernia (CDH) (due to other comorbidities) occurred. Intra operative hypercarbia and hypoxia were observed more frequently in thoracoscopic procedures. Intra operative hypothermia was not common and was well tolerated. Conversion to open procedure (n = 5), post operative ileus (n = 3), port site infection (n = 5) were other complications. CONCLUSION: MAS in neonates and small infants is a technically demanding but a feasible choice available. Some prior experience in older children is required for safe and effective outcome. Good quality optics, video equipments and instruments are required for safe and effective procedure. Intra operative measurement of oxygen saturation and temperature, and diligent post operative ICU care are mandatory for safe and successful outcome.

Role of hyperbaric oxygen therapy in severe head injury in children.

AIM: A brain injury results in a temporary or permanent impairment of cognitive, emotional, and/or physical function. Predicting the outcome of pediatric brain injury is difficult. Prognostic instruments are not precise enough to reliably predict individual patient's mortality and long-term functional status. The purpose of this article is to provide a guide to the strengths and limitations of the use of hyperbaric oxygen therapy (HBOT) in treating pediatric patients with severe brain injury. MATERIALS AND METHODS: We studied total 56 patients of head injury. Out of them 28 received HBOT. Only cases with severe head injury [Glasgow Coma Scale (GCS) < 8] with no other associated injury were included in the study group. After an initial period of resuscitation and conservative management (10-12 days), all were subjected to three sessions of HBOT at 1-week interval. This study group was compared with a control group of similar severity of head injury (GCS < 8). RESULTS: The study and control groups were compared in terms of duration of hospitalization, GCS, disability reduction,and social behavior. Patients who received HBOT were significantly better than the control group on all the parameters with decreased hospital stay, better GCS, and drastic reduction in disability. CONCLUSION: In children with traumatic brain injury, the addition of HBOT significantly improved outcome and quality of life and reduced the risk of complications.

Giant epignathus with midline mandibular cleft: Insights in embryology and management.

A congenital teratoid tumor arising and protruding through the mouth is classified as epignathus or fetus in fetu. On review of literature, we found various reports of midline mandibular and lower lip cleft associated with flexion contracture of neck, midline cervical cord, but there is only one report of association with midline dermoid. We present an unusual case of midline cleft of mandible with an epignathus. A 2.3-kg male child, delivered transvaginally in the 38(th) gestational week, was referred to us for management of a large irregular growth hanging outside the mouth. On examination, he had a wide median cleft of the mandible with tongue adherent to the "V"-shaped defect in the area of lower lip. A midline irregular mass of size 12 × 8 × 5 cm with variegated consistency was arising in the midline from the floor of the mouth between the tongue and lower lip. X-ray and computed tomography scan showed a rounded soft tissue mass arising from the alveolus with multiple calcifications within it along with a large triangular calcification and absence of hyoid bone. The mass was excised by mobilizing the tip of tongue. Staged repair was planned for the defect in the mandible. Unfortunately, the baby succumbed postoperatively to complex congenital heart disease. Histopathology was suggestive of epignathus. We discuss hereby the embryology and current management strategies of the problem.

Laparoscopic repair of pediatric inguinal hernia--is vascularity of the testis at risk? A study of 125 testes.

AIM: The aim of this study was to study the effects of laparoscopic inguinal hernia repair on testicular perfusion and size. MATERIALS AND METHODS: A prospective study concerning laparoscopic inguinal hernia repair was performed for an 18-month period to evaluate testicular perfusion and size in the preoperative, early postoperative (within 48 hours of surgery), and late postoperative periods (6 months after surgery) using Doppler ultrasound (DUS) (both duplex and power Doppler mode). Laparoscopic closure of the deep inguinal ring was accomplished with a purse string suture (Nylon 3-0) using standard 3-port technique. The testis units were divided in 2 groups: group 1 comprising testis units in which a resistive index (RI) could be calculated and group 2 with instances in which an RI could not be calculated but showed blood flow consistently on DUS. RESULTS: A total of 112 boys underwent laparoscopic inguinal hernia repair with 100 available for complete follow-up and data analysis. One hundred twenty-five inguinal (25 bilateral) hernia repairs were performed. Group 1 had 80 testis units. There was no significant difference in values of RI between preoperative, early postoperative, and late postoperative periods. Group 2 had 45 testis units. Resistive index could not be calculated. Seventy-five percent showed only systolic blood flow on spectral analysis; hence, RI, 1; and the rest showed the presence of blood flow on power Doppler scan. All testis units consistently showed blood flow in the early and late postoperative period. No testicular atrophy was found at 6-month follow-up examination on DUS. CONCLUSION: Laparoscopic repair of inguinal hernia in children does not affect testicular perfusion or growth.

Neonatal lingual and gastrointestinal mucormycosis in a case of low anorectal malformation-a rare presentation.

We report a 1.7 kg male infant with a low anorectal malformation treated at an outside facility and referred to us on postoperative day 11. At presentation, his upper abdomen was distended, and he had perianal mucoid discharge. The tongue had a blackish discoloration. An erect abdominal radiograph showed a few fluid-filled bowel loops in the upper abdomen with a gasless lower abdomen and pelvis, suggestive of upper small bowel obstruction. There were no specific radiological features of necrotizing enterocolitis. He underwent laparotomy and bowel resection for perforated jejunum. Histopathology of the tissue specimen was suggestive of mucormycosis. Postoperatively, he received intravenous amphotericin B (liposomal) and was started on liquid enteral nutrition after 2 weeks. However, the anterior two thirds of his tongue gradually sloughed off. He is awaiting reconstruction of the tongue. The purpose of this report is to emphasize that physicians should have a high index of suspicion for oral and gastrointestinal tract mucormycosis in neonates with metabolic disturbances who present with a discolored oral mucosa and an abdominal mass with intestinal obstruction. Early diagnosis and an aggressive approach of combined medical and surgical treatment may improve the outcome of patients with this potentially lethal invasive disease.

Early experience with thoracoscopic repair of congenital diaphragmatic hernias in pediatric age group: results and lessons learned.

PURPOSE: To describe the surgical technique, initial results, and overview indications of thoracoscopic repair of congenital diaphragmatic hernia. PATIENTS AND METHODS: A retrospective review was undertaken of patients with CDH who underwent thoracoscopic repair from January 2006 to July 2010, in department of pediatric surgery in a tertiary care institute in India. Patients underwent surgery under general anesthesia. Reduction of the hernia contents was carried out using one trocar for telescope and two operating trocars. Pleural insufflation with carbon dioxide was maintained at a pressure of 6-10 mmHg. The hernia defect was repaired using nonabsorbable interrupted sutures. RESULTS: There were 17 patients, including 12 boys and 5 girls. Among 12 infants, there were 6 patients younger than 30 days. The other 5 patients were older than 1 year. The hernia was located in the left side in 14 patients and in the right side in 3 patients. The mean operative time was 110 min. Conversion was required in three patients. There were one recurrence and one postoperative death. CONCLUSIONS: Thoracoscopic repair is feasible and safe for children with CDH, including selective newborn. The technique causes minimal trauma, results in good respiratory function, and promotes early recovery.

Early management of mesenteric cyst prevents catastrophes: a single centre analysis of 17 cases.

BACKGROUND: Mesenteric cysts are rare intra-abdominal masses in the paediatric age group with varied presentation, ranging from an asymptomatic mass to acute abdomen. This study reviews our experience in the diagnosis and treatment of 17 mesenteric cysts in our centre, with especial reference to acute abdominal symptoms. PATIENTS AND METHODS: Seventeen patients (age less than 10 years) with mesenteric cysts were managed in our hospital. The age ranged from 15 days to 10 years. Patients were admitted with acute or chronic symptoms. They were evaluated with complete history, clinical examination, blood investigations and radiological investigations (x-ray abdomen erect, ultrasound abdomen (USG) and computed tomography (CT) scan in selected cases) to reach a provisional diagnosis. The diagnosis was proven on laparotomy and histologically confirmed. RESULTS: The main presenting symptoms were abdominal pain or lump. The most common mode of presentation was acute small intestinal obstruction. USG was not conclusive in all. Abdominal CT scan with intravenous contrast was diagnostic in nine patients. Five patients had volvulus on exploration. Cysts were located in small intestinal mesentery in 14 cases and three were in the sigmoid mesentery. Seven patients had complete excision, intestinal resection was required in four and marsupialisation with cauterisation of margins was done in six patients. Histologically, all were lymphangiomatous mesenteric cysts. CONCLUSION: The diagnosis of mesenteric cysts should be kept in mind in any patient presenting with acute abdominal symptoms. Small bowel volvulus with mesenteric cyst constituted a significant number in children with acute abdominal symptoms. Early diagnosis and treatment yields excellent outcome.

Ruptured rectal duplication cyst with classical bladder exstrophy.

A newborn boy was brought to us, 2 hours after birth, with a mucosal-lined left hemiperineal lesion associated with classical bladder exstrophy and an anterolaterally displaced anus. Perineal anatomy was restored by excising the mucosa lined lesion. The bladder closure for classical bladder exstrophy was done at the same time. Histologically, gastric, respiratory, and small intestinal epithelia were present in the mucosa. A rectal duplication cyst that had ruptured in utero through the hemiperineum could explain the anomaly. The association of classical bladder exstrophy with ruptured rectal duplication cyst has never previously been described in the literature.

Laparoscopic inguinal hernia repair in the pediatric age group--experience with 437 children.

BACKGROUND/PURPOSE: A retrospective analysis of prospectively collected data of pediatric patients that underwent laparoscopic inguinal hernia repair. MATERIAL AND METHODS: A retrospective review was performed of the prospectively collected data of 576 laparoscopic internal ring closures in 437 children (age, 30 days-11 years; median, 1.9 years) from June 1999 to February 2009. The internal ring was closed with a 3-0 nonabsorbable suture. Both extracorporeal and intracorporeal methods of knotting were used. All patients were asked to return at 1 week and 6 weeks postoperatively for routine follow-up. RESULTS: A contralateral patent processus vaginalis was present in 13% (45/352) of boys and 15% (12/83) of girls on the right side, and 7% (25/352) of boys and 6% (5/83) of girls on the left side. Follow-up range was from 1 week postoperatively to 108 months. There were 14 recurrences (2.4 % [14/576], 11 in boys and on the right side and 3 in girls) and 2 hydroceles 0.35% (2/576). Mean operating time was 23 minutes for unilateral and 29 minutes for bilateral inguinal hernia. There was neither metachronus hernia nor testicular atrophy observed during follow-up. CONCLUSION: Laparoscopic inguinal hernia repair is technically easier, as there is no need to dissect the vas deferens and vessels. The risk of metachronous hernia is reduced, and we believe the cosmetic result is better. Although recurrences were more common early in the series, currently they are much less frequent. Laparoscopic inguinal hernia repair appears to have less morbidity than open herniotomy and can be used as routine procedure in the pediatric age group.

Urethral ratio on voiding cystourethrogram: a comparative method to assess success of posterior urethral valve ablation.

OBJECTIVE: To develop a simple, objective and reproducible quantitative measurement to assess success of posterior urethral valve ablation. METHOD: In 30 patients with posterior urethral valves the diagnosis was confirmed by voiding cystourethrogram (VCUG). Our protocol was to perform valve ablation, and repeat VCUG at 12 weeks postoperatively. Urethral ratio was calculated by dividing the posterior urethral diameter by the anterior urethral diameter. Thirty males undergoing VCUG for urinary tract infections were evaluated as normative controls. RESULTS: Median age of controls was 12 months (2 days-6 years) and of study group was 13 months (1 day-11 years). Mean urethral ratio in pre-fulguration group was 4.94 (+/-2.97) and in post-fulguration group was 2.134 (+/-1.19) (P<0.001). The mean urethral ratio in the control group of 1.73 (+/-0.577) was significantly different from the pre-fulguration group result (P<0.001), but not significantly different in comparison to the post-fulguration group (P=0.104). CONCLUSION: Calculation of urethral ratio on VCUG as a method of assessment of outcome of fulguration is objective, reproducible, and allows preoperative and postoperative VCUG from different facilities to be compared. A post-fulguration urethral ratio of 2.5-3 represents an acceptable result postoperatively.

Laparoscopic management of persistent mullerian duct syndrome.

Persistent mullerian duct syndrome (PMDS), characterized by the presence of mullerian structures in a virilized male, frequently presents as undescended testis, either intraabdominal or within a hernial sac. We describe a 10-month-old infant with PMDS successfully managed by the laparoscopic approach. At the age of 1.5 months, the patient presented with a left inguinal hernia and bilateral nonpalpable gonads in another center and underwent left inguinal exploration. The uterus and a gonadlike structure along with the hernia sac were found in the inguinal canal. Left inguinal herniotomy was performed after reduction of the uterus and gonadlike structure. No gonadal biopsy was obtained. The patient was further investigated in the same center. His karyotype was 46,XY. Magnetic resonance imaging of the abdomen and pelvis revealed a uterinelike structure posterior to the urinary bladder, but neither testis nor ovaries were visualized. At 10 months of age, he was referred to our department for further management. A laparoscopic single-stage orchiopexy was performed. Both testes were identified and brought to the scrotum by splitting the uterus in the midline and then bringing the testes with the vas and attached uterine tissue into the scrotum. The aim of placement of well-vascularized testes in the scrotum was achieved as confirmed on follow-up color Doppler ultrasound study 6 months postoperatively, which showed normal vascularity. Laparoscopic surgical techniques for this condition are also discussed.

Effect of Candida infection on outcome in patients with perforation peritonitis.

Perforation peritonitis is treated with surgery and antibiotics. This study was conducted to identify bacterial and fungal microorganisms responsible for peritonitis in patients with hollow viscus perforation and to examine the influence of these microorganisms on the outcome. A prospective study was conducted from May 2005 to September 2006 involving 84 consecutive patients with spontaneous gastrointestinal perforation peritonitis, who were referred for surgery. Peritoneal fluid was analyzed by microbial culture and biochemical tests for bacteria and fungi. The Jabalpur Prognostic Score was calculated. Forty-two of the 84 patients had positive peritoneal fluid cultures. Escherichia coli was the most common bacterium (n=26) and Candida (n=13) the most common fungus isolated. Bacterial isolates were largely sensitive to amikacin while all the Candida isolates were sensitive to fluconazole. Mortality was significantly higher in patients with positive peritoneal cultures (15/42) compared with those with negative peritoneal cultures (0/42, p<0.001), and in patients with mixed bacterial and fungal-positive cultures (10/13) compared with those with isolated bacterial cultures (5/29, p<0.001). Using the Jabalpur Prognostic Score, positive fungal cultures were found to be associated with a significantly higher than expected mortality. Patients with gastrointestinal perforations and positive peritoneal cultures have a poor prognosis, which is significantly worsened by the association of positive fungal cultures. Early recognition and treatment of fungal infection is advisable.