A rare presentation of Y-duplication of the pancreatic tail with enteric duplication cyst as intussusception in a child: A case report.

We report a case of a 2-year-old girl who presented to us with complaints of pain abdomen and non-bilious vomiting. USG was suggestive of intussusception with necrotic bowel and free fluid in the left lumbar region. Operative findings were of enteric duplication cyst of the pancreas which was later confirmed on histopathology. Enteric duplication cysts can occur anywhere in the gastrointestinal tract but duplication cyst within the pancreas is rare. The most common presentation in other reported cases is pain abdomen due to pancreatitis but in our case, we had findings of intussusception. Children usually present with a severe complication like perforation of duplication cyst as in our case. Excision of the cyst and the duplicated pancreatic tail is the mainstay of treatment.

Stuttering Priapism in a Teenage Boy: Lesson to be Learnt.

Untreated priapism can lead to ischemic damage of the penis and impotence. This case report describes a 14-year-old boy who presented with a history of priapism for 2 months, which was undiagnosed, ridiculed, and ignored even by medical practitioners. The underlying etiology was later identified to be chronic myeloid leukemia. Despite the usage of multimodal treatment, it took 7 days for control of priapism. The young boy is now left with an erectile dysfunction. The case highlights that priapism in children is a medical emergency needing aggressive evaluation and treatment.

Vascular malformation of ileum: A possible cause of neonatal intestinal obstruction.

Vascular lesions of gut are rare and they may rarely cause luminal obstruction in neonates. A baby boy born at 36 weeks of gestation presented with small bowel obstruction on day 3 of life. X-ray suggested small bowel obstruction. Contrast enema showed microcolon with multiple filling defects. Exploration revealed thin floppy gut with multiple segments of stenoses and dilatation with surface bluish discoloration but no perforation, two areas were resected. Proximal anastomosis was done and distally a stoma was formed. Histopathology showed vascular malformation in all the layers of gut. The diagnosis was impossible preoperatively and could be established after surgery only.

Role of uroflowmetry before and after hypospadias repair.

AIMS: To study the role of uroflowmetry in the preoperative and early postoperative period in children undergoing hypospadias repair. MATERIALS AND METHODS: Twenty-six cases undergoing hypospadias repair over 1 year (tubularized incised plate [TIP] Snodgrass [17 patients], TIP with Snodgraft [5 patients], Duckett's onlay flap [2 patients], and Duckett's tube [2 patients] urethroplasty) were prospectively evaluated with preoperative ultrasound and uroflowmetry and postoperative uroflowmetry at 3 months after the surgery and at 6 and 9 months interval if these dates fell within the study period on follow-up. The parameters studied were maximum flow rate (Qmax), average flow rate (Qav), total voided volume, voiding time, and type of curve. Preoperative and postoperative uroflow data were compared. RESULTS: Twenty-six cases comprised of anterior hypospadias (n = 8), mid penile (n = 11), and posterior hypospadias (n = 7). Fourteen patients had obstructed flow rates preoperatively. While 69% patients (18/26) had obstructed flow rates at 3 months postoperatively, it dropped to 43% at 9 months. Following TIP (Snodgrass) repair, 88% (15/17) had obstructed flow rates postoperatively. Best results were seen in patients undergoing circumferentially epithelialized urethral reconstruction (TIP with Snodgraft, Duckett's onlay flap, and Duckett's tube). CONCLUSIONS: Abnormal uroflow is an inherent aspect of hypospadias in 50% of the cases. Both preoperative and postoperative uroflow evaluation is necessary for meaningful conclusion. Patients with preoperative normal flow rates but obstructed postoperative flow rates need clinical evaluation. Obstructive flow rates are more common after TIP (Snodgrass) repair. The urinary flow rates improve with time.

Association of Levels of Serum Inhibin B and Follicle-stimulating Hormone with Testicular Vascularity, Volume, and Echotexture in Children with Undescended Testes.

AIMS: The aim of our study was to assess the association between reproductive hormones (inhibin B [inh B], follicle-stimulating hormone [FSH]) with testicular volume, echogenicity, and blood flow (resistive index [RI]) in children with undescended testis (UDT). SETTINGS AND DESIGN: This was a prospective study of 1-year study duration. MATERIALS AND METHODS: A total of 33 patients (16 unilateral and 17 bilateral) UDTs aged 5-12 years with palpable UDT were included in the study. Morning fasting blood samples were taken for estimation of serum inh B and FSH as well as inh B/FSH ratio. Testicular ultrasound was done to compute testicular volume, testicular echogenicity, and testicular vascularity in terms of RI. RESULTS: The mean age of patients enrolled in the study was 8.29 years for unilateral UDT and 7.97 years in bilateral UDT and it was comparable. The study groups were further subdivided into two age-wise subgroups school goers (5-8 years) and prepubertal (9-12 years). The values of inh B, FSH, and inh B/FSH ratios as well as mean testicular volume were comparable between both groups and subgroups. Overall mean testicular volume had a positive correlation with FSH, inh B, and inh B/FSH, but statistical significance was reached only for inh B (P < 0.001) in children with both unilateral and bilateral UDT. Apart from five patients with hypoechogenicity within the testis, all remaining testes were of homogenous echotexture with no instances of irregular echogenicity or tumor. Children with RI >0.6 were separately studied. The incidence of high RI (>0.6) was also comparable in unilateral or bilateral disease. These subjects had unfavorable biochemical parameters in terms of low inh B levels and high FSH levels. CONCLUSIONS: Our findings hint to the fact that palpable UDT forms a homogenous group, whether unilateral or bilateral, whereas impalpable testes may form a separate category and need further studies to substantiate this hypothesis.

Hormonal profile in children with isolated hypospadias associates better with comprehensive score of local anatomical factors as compared to meatal location or degree of chordee.

BACKGROUND: To evaluate if hormonal profile of children with isolated hypospadias (IH) associates better with comprehensive local anatomical factor score (LAFS) than with clinically adjudged urethral meatus location or severity of chordee/k.j. MATERIAL AND METHODS: Ninety-nine children with IH were enrolled, as per inclusion criteria. Meatal location was recorded at first clinical examination in OPD; while LAFS was computed per-operatively using indigenously devised scale, except for neonates. Hypospadiacs were first classified into three standard meatal based groups and subsequently into LAFS based two groups (≤19, >19). For all participants, pre HCG and post HCG (96 hour post- injection) estimation of serum gonadotropins, DHEA-S, estrogen (E), progesterone (P), testosterone (T) and Dihydrotestosterone (DHT) was done. Statistical tests were applied to assess significance of hormonal levels with respect to meatal location, chordee and LAFS. RESULTS: Only FSH levels differed significantly among meatal based groups; while among LAFS groups, multiple hormonal differences were noted; with poor LAFS associated significantly with higher FSH, LH and lower E, T/DHT. Children with severe degree of chordee had poorer T output and a significantly lower LAFS as compared to those with moderate/mild chordee. CONCLUSION: Serotoli cell dysfunction, indirectly indicated by high FSH was found among midpenile hypospadiacs and those with poorer LAFS. Since groups based on LAFS revealed multiple intergroup hormonal differences than what was seen for meatal/chordee based groups; LAFS should be considered a better guide for prognostication and for deciding about hormonal supplementation. Lower androgenic output was particularly noted in children with severe chordee.

Inflammatory myofibroblastic tumor: an unusual mimicker of childhood intrathoracic tuberculosis.

Inflammatory myofibroblastic tumors are rare lesions of uncertain etiology that are often difficult to diagnose because of their myriad clinical presentations. Not uncommon, they mimic persistent pneumonia. We report a 4-year-old girl who presented with prolonged pyrexia, weight loss, severe anemia, hepatosplenomegaly, and nonresolving pneumonia. Initial investigations including flexible bronchoscopy and bronchial washing for usual causes of persistent pneumonia, such as tuberculosis and other infections, were negative. Chest computed tomography revealed a well-defined lesion involving the lingula and left upper lobe with extension into the subpleural space. Pleural tap and biopsy was also noncontributory. Thoracoscopic biopsy was suggestive of an inflammatory myofibroblastic tumor. As the lesion was encasing the major vessels, it was considered inoperable. The patient did not respond to steroid therapy and etoricoxib and later succumbed to the illness. This uncommon tumor should be considered in the differential diagnosis of children who presented with unresolving consolidation with pyrexia.

Colonic gastrointestinal stromal tumour presenting as intussusception.

Gastrointestinal stromal tumours (GIST) are rare in paediatric patients and have a discrete clinicopathological and molecular divergence from that observed in adults. In the present report we present a case of a 2-month-old female in whom colonic gastrointestinal stromal tumour acted as a lead point of colocolic intussusception. Laparoscopically assisted reduction of the intussusception and resection of tumour was done.

Pedicled omental onlay flap for post-traumatic intrahepatic major ductal injury.

We report a 5-year-old girl who presented with post traumatic biliary leakage that failed to respond to conservative management for two weeks. Surgical exploration in the third week revealed a partially healed 5 cm long hepatic laceration in the right lobe of the liver. Bile was found leaking through a rent in the major right intra-hepatic duct at the apex of liver laceration. A pedicled onlay omental flap was used to buttress this rent as direct closure was not possible due to friable tissue. The child recovered uneventfully.

Chylothorax after Primary Repair of Esophageal Atresia with Tracheo-esophageal Fistula: Successful Management by Biological Fibrin Glue.

A neonate, who had undergone primary repair of esophageal atresia with tracheo-esophageal fistula, developed right pleural effusion in the postoperative period. It was initially misdiagnosed as an anastomotic leak, but later confirmed to be chylothorax. Conservative treatment failed. Application of biological fibrin glue (sealant) on the mediastinum through a thoracotomy was curative.

Bilateral congenital diaphragmatic hernia.

Bilateral congenital diaphragmatic hernia (CDH) is a rare birth defect, with a poor prognosis. We describe a case of bilateral CDH discovered while repairing the right sided CDH. Diaphragmatic defect was repaired and a silo was applied on the abdominal wound to avoid abdominal compartment syndrome. The patient however died postoperatively due to severe pulmonary hypertension.

Laparoscopic repair of indirect inguinal hernia in children: does partial resection of the sac make any impact on outcome?

PURPOSE: To test the hypothesis that during laparoscopic hernia repair, partial resection of the distal sac along with suture ligation of the neck is better than simple transection and ligation. METHODS: The following two techniques of laparoscopic hernia repair were compared: Group I, circumferential incision of peritoneum at the deep ring and partial resection of the distal sac and suture ligation at the neck; versus Group II, circumferential incision of the peritoneum at the deep ring and suture ligation at the neck. Twenty-five cases of inguinal hernia were randomly selected in each group between the age group of 6 months to 12 years. The outcome measures were recurrence, intra- or postoperative complications, and time taken for surgery. RESULTS: There were no recurrences in either group. Other parameters for comparison were also not statistically different between the two groups. There was no conversion. CONCLUSIONS: Although partial resection of the sac has been an essential step in open hernia repair over five decades, its value has been questioned by our study, because omitting this step during laparoscopic repair has not adversely affected the outcomes. Partial resection of the sac is not a necessary component of hernia repair. It is a technical necessity of the open approach. Therefore, omitting this step in laparoscopic repair does not adversely affect the outcome.

Rectal involvement in neutropenic enterocolitis.

Neutropenic enterocolitis is a common gastrointestinal complication in children undergoing chemotherapy for a variety of malignancies. It usually involves ileum and caecum, and involvement of rectum has rarely been reported. The authors report neutropenic enterocolitis in a child undergoing chemotherapy for acute lymphoblastic lymphoma which presented with ileus along with a mass like lesion in the rectum.

Retiform pattern of Sertoli-Leydig cell tumor of the ovary in a 4-year-old girl.

Sertoli-Leydig cell tumors are rare sex cord-stromal neoplasms that account for <0.2% of ovarian tumors. These tumors with a retiform pattern pose difficult diagnostic problems, with the majority being misinterpreted as serous papillary cystadenocarcinoma and endodermal sinus tumor. Retiform pattern Sertoli-Leydig cell tumor seen mostly in young patients is clinically malignant and has a worse prognosis, underlining the importance of recognition of this histopathologic entity. In the literature only two such cases have been described in patients under the age of 5 years; reported herein is the third case. Described here is a rare case of ovarian Sertoli-Leydig cell tumor with retiform pattern in a 4-year-old child without any endocrine manifestations.

Malignant hypertension in a child with solitary functioning hydronephrotic kidney.

A child with solitary functioning hydronephrotic kidney and hypertensive encephalopathy is described. Removal of the contralateral dysplastic kidney failed to normalize his blood pressure.

The use of pedicled appendix graft for substitution of urethra in recurrent urethral stricture.

BACKGROUND: Recurrent posterior urethral strictures after failed urethroplasty may need urethral substitution. Skin or mucosal grafts, currently used for this purpose, have a high complication rate. The authors describe the use of pedicled appendix for posterior urethral substitution. METHODS: Two boys with pelvic fracture urethral distraction injuries were treated for recurrent posterior urethral strictures after a failed perineal anastomotic urethroplasty. Through a perineal-transpubic approach the stricture tissue was excised, which resulted in a gap of 5 to 7 cm between the healthy ends. The vermiform appendix was mobilised on its own pedicle and transposed to the perineum; the proximal end of appendix was anastomosed to the prostatic urethra and the distal end (tip discarded) to the bulbar/penile urethra. Omentum was transposed to wrap the anastomosis and fill the dead space. RESULTS: Normal micturition was restored in both patients. No further treatment was required after 1 dilatation in the first case. Both patients are continent. Potency status remains unchanged from the preoperative period with normal erections in 1 case. Follow-up (1 to 3 years) has been satisfactory with no complications. CONCLUSIONS: The appendix is a promising organ for posterior urethral replacement. It can be brought to the perineum on its own vascular pedicle.