Aicardi syndrome is a very rare neurodevelopmental disorder, inherited as an X-linked dominant condition with a triad of infantile spasm, partial or complete agenesis of the corpus callosum, and chorio-retinal \"lacunae.\" We report a case of a female infant with the classical triad of Aicardi syndrome. A female infant presented to the Paediatric Neurology Clinic of the Federal Medical Centre Birnin-Kebbi, North-western Nigeria, at the age of two months with complaints of recurrent afebrile convulsions typical for infantile spasms. The patient was delivered at term with normal Apgar scores and anthropometry. Examination revealed an infant with no dysmorphic features and normal systemic examination. Magnetic Resonance Imaging (MRI) of the brain however, showed complete agenesis of the corpus callosum and dilatation of the posterior horn of the lateral and third ventricles. Fundoscopy showed multiple yellowish spots along the vascular arcades in the right eye. The left eye had a one-disc diameter lacuna in the superior nasal quadrant adjacent to the optic disc with multiple yellowish spots. A diagnosis of Aicardi syndrome was made. The child was placed on oral phenobarbital and followed up. At the age of 18 months, the child can only sit without support, hold an object in each hand, smile socially, and babble. The frequency of the seizures had also reduced from >100 episodes per day to 2-3 episodes per day, but the child had developed right-sided spastic hemiparesis. The patient was commenced on physiotherapy and the anti-epileptic drugs were maintained. We recommend clinicians consider Aicardi syndrome in the differential diagnosis of any child presenting with infantile spasms.

A 14-months old female child was diagnosed with bilateral single system ectopic ureters opening into the urethra, with small bladder capacity, horseshoes kidneys, and bilateral hydronephrosis, presenting recurrent febrile UTI accompanied by continuous incontinence and elevated renal function. Early bilateral re-implantation of the ureters (modified Lich-Gregoir) was done in one setting, resulting jn no recurring febrile UTIs and continuous wetting, improving renal function parameter, competent bladder neck, and 10 folds increased in bladder capacity after 1-year follow up. We showed that earlier treatment enables patient to preserve both renal and bladder function without involving complex reconstructive surgery.

OBJECTIVE: To document our experience of initial management of Cloacal malformation in female patients.
METHODS: A descriptive retrospective study was conducted in the Department of Pediatric Surgery of the National Institute of Child Health Karachi from January 2010 to September 2018. Female patients with diagnosis of Cloacal malformation were included in this study. Data regarding the age at presentation, mode of presentation, clinical features, presence of hydrocolpos, and associated anomalies were noted. Surgical procedures performed in these patients and the outcomes were also documented. Data was analyzed on SPSS Version 20.
RESULTS: Sixty females were included in the study. Age ranged from birth to three years with a median of four days. Patients admitted through emergency were 44 (73.33%) while 16 (26.66%) were admitted through outpatient clinic. Hydrocolpos was found in 15 (25.00%) patients. Five (8.33%) patients had massive abdominal distention and were presented with severe respiratory distress. Among them three had massive hydrocolpos, one patient had pneumoperitoneum secondary to Meckel\'s perforation and one patient was having massive colonic dilatation. Hydronephrosis and hydroureter were found in 14 (23.33%) patients, while dilated bladder was found in three (5.00%) patients. After optimization of patients, bowel diversion was done as transverse colostomy in 39 (65.00%) patients, high sigmoid colostomy in 17(28.33%) patients while ileostomy was done in three (5.00%) patients. All patients with hydrocolpos had tube vaginostomy. None of the patients required bladder diversion and urinary tract dilatations were gradually subsided in nine patients in the post-operative period. Associated anomalies were found in 22(36.66%) of cases predominantly of sacral origin. Operative complications were found in 18(30.00%) patients, including stoma site in infection in 12(20.00%) patients, vaginal tube dislodgement in two patients, wrong placement of tube in one patient and vaginostomy stenosis in one case. While bowel stoma retraction occurred in four patients, prolapsed was found in three patients. Renal failure occurred in one patient. In total, 37(61.00%) patients had smooth recovery, 18(30.00%) patients had some complications, while Five (8.33%) patients were expired due to sepsis.
CONCLUSIONS: Majority of cloacal malformations present in neonatal life. Initial management is an important step in dealing with these patients. Care must be taken during the abdominal exploration regarding drainage of hydrocolpos and appropriate placement of bowel stoma.

Eosinophilic panniculitis (EP) is characterized by prominent infiltration of subcutaneous fat with eosinophils. The etiology is diverse. This is not a disease but represents a reaction pattern that may occur in a variety of circumstances. The exact pathogenesis of the disease is still unclear. We present the case of a 6-year-old girl child who was diagnosed with EP.