Situs inversus abdominalis, polysplenia, complex jejunal atresia and malrotation in a neonate: A rare association.

INTRODUCTION: Situs inversus, polysplenia, complex jejuna atresia are rare anomalies in isolation. Their association in a single patient is even rarer with challenges in diagnosis and management. PRESENTATION OF CASE: A 5 day old neonate presented with features of small bowel obstruction. Radiological investigations revealed situs inversus abdominalis with dilated proximal small bowel loops. At laparotomy, abdominal situs inversus, polysplenia, multiple jejunal atresias with apple peel appearance of the ileum with malrotation was seen. CONCLUSIONS: The association of situs inversus, polysplenia and complex jejunal atresia is very rare. Pre-operative diagnosis of situs inversus is important for appropriate incision placement and surgical planning.

Congenital Benign Intrahepatic Cyst with Cystobiliary Communication: An Intraoperative Diagnostic Enigma.

This manuscript reports a 15-month-old male presenting with a congenital solitary benign intrahepatic cyst, wherein the presence of a cyst with frank bile and a large cystobiliary communication came as an intraoperative enigma. The stepwise approach to diagnosis, management, and review of relevant literature carry a useful message.

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.

Symptomatic Mullerian Duct Cyst in a Male Infant.

Symptomatic Mullerian duct cyst is a rare entity in children. A 9-month-old male infant presented with bowel and urinary obstructive symptoms. Imaging investigations revealed a cystic mass in the rectovesical pouch compressing bladder neck and rectum. At laparotomy, a Mullerian duct cyst was found. Most of the cyst was excised and the remaining cyst mucosa was cauterized. The child improved thereafter.

Congenital lobar emphysema: Pitfalls in diagnosis.

Congenital lobar emphysema (CLE) is a rare but life-threatening congenital anomaly leading to respiratory distress in early childhood. Diagnosis requires a strong clinical suspicion. We report a case of a 31/2-month-old infant who was initially diagnosed with pneumonia requiring multiple hospital admissions. After computed tomography of the thorax, a diagnosis on CLE was made. The child was planned for surgery in the next available routine operation theatre. However, suddenly in the evening, she developed respiratory distress and needed emergency surgical intervention. The child improved dramatically after surgery, and the postoperative period was uneventful. Early diagnosis and treatment in such cases can lead to dramatic results.

Isolated hydatid cyst of the pancreas masquerading as pancreatic pseudocyst.

Hydatid cyst of the pancreas is very rare and usually presents with obstructive jaundice. We report a case of a 7-year-old girl with cyst of the pancreas without any obstructive jaundice. The child was treated surgically and hydatid cyst of pancreas was found. Partial pericystectomy was performed. After 1 month on follow-up, the child developed pseudocyst of pancreas, which needed cystogastrostomy. Postoperatively, the child improved and is remaining well after 3 months of follow-up. Hydatid cysts of pancreas are very difficult to diagnose preoperatively and should be kept as a differential diagnosis of cystic intrapancreatic lesions. Serological tests can help in early diagnosis.