Clinical profile and outcome of neonatal pneumothorax in resource-limited neonatal intensive care unit.

OBJECTIVE OF STUDY: To determine the frequency, clinical profile, and outcome of pneumothoraxes in neonates in resource-limited neonatal intensive care unit (NICU) setting. STUDY DESIGN: Prospective cohort. PLACE AND DURATION OF STUDY: The study was carried over a period of 4 years and 6 months at two NICUs of Combined Military Hospital (CMH) Multan (January 2011-August 2013) and CMH Abbottabad (September 2013-July 2015). MATERIALS AND METHODS: All neonates fulfilling inclusion criteria were included in the study. Demographic and clinical parameters were recorded. All stable cases were initially managed on headbox oxygen, nasal oxygen, or improvised bubble continuous positive airway pressure devices (B-CPAP). In suspected cases, pneumothorax was confirmed on a portable chest X-Ray (CXR). Hemodynamically stable neonates were closely observed for spontaneous resolution whereas unstable neonates were managed by needle thoracocentesis or thoracostomy with or without ventilator support. End point of the study was either discharge or death of the neonate. RESULTS: Of a total of 1427 included neonates, 66 (4.6%) developed pneumothorax. Pneumothorax was more common in late pre-term and term infants with normal birth weight (>2.5 kg). Primary diseases leading to pneumothorax were respiratory distress syndrome (RDS) 38 (57.6%), meconium aspiration syndrome (MAS) 10 (15.2%), transient tachypnea of newborn (TTN) 9 (13.6%), birth asphyxia syndrome (BAS) 6 (9.1%), and pneumonia 3 (4.5%). Majority of the patients 58 (87.9%), required thoracostomy, 2 (3%) required only needle thoracocentesis while spontaneous resolution occurred in 6 (9.1%) patients. Out of 66 patients, 48 (72.7%) were discharged and 18 (27.3%) died. Average hospital stay was 10.5 ± 5.85 days. CONCLUSION: Pneumothorax is not an uncommon complication in neonates presenting with respiratory distress in resource-limited NICUs. High index of suspicion is required for early recognition and timely management, as delay is associated with significant mortality.

Branchio-oto-renal syndrome.

The association of branchial arch anomalies (branchial cysts, branchial fistulas), hearing loss and renal anomalies constitutes the branchio-oto-renal (BOR) syndrome also known as Melnick Fraser syndrome. We present a case of this rare disorder in a girl child who presented with profound deafness, preauricular pits, branchial sinuses and renal hypoplasia.

Grebe syndrome: a rare association with congenital heart disease.

Grebe syndrome is a very rare form of short-limbed dwarfism. It is a genetic condition, passed by autosomal recessive inheritance. It is characterized by marked acromesomelic shortening of all the four limbs. There are no other associated anomalies. The affected baby has normal intelligence and normal life span. We present here a case of Grebe syndrome along with congenital heart disease.

Rosai Dorfman disease--a rare entity report of two cases with nodal and extranodal involvement.

Rosai Dorfman Disease (RDD) also known as Sinus Histiocytosis with Massive Lymphadenopathy (SHML)is a very rare variety of reactive histiocytoses. It commonly involves cervical lymph nodes; although involvement of other lymph node regions, skin and other organ involvement can occur. It has a good prognosis so there is a need to differentiate it from other lympho proliferative disorders of poorer prognosis. We present two cases of this rare disorder illustrating its clinical spectrum; a 22 years old woman with involvement of submandibular lymph nodes, nasal septum and sub-glottic region and a 45 years old male with prominent skin involvement.

Brodie's abscess--an uncommon cause of leg pain.

A rare case of Brodie's abscess of distal left tibia is presented in a child which was initially missed on clinical grounds alone. Differentiation from different bone neoplasms was done on radiological grounds. The patient was managed surgically with high dose intravenous antibiotics. Brodie's abscess is very rarely encountered in our reporting of X-rays.

Frequency of Otitis Media with Effusion in recurrent upper respiratory tract infection in children.

OBJECTIVE: To determine the frequency of Otitis Media with Effusion (OME) in children with recurrent upper respiratory tract infections. STUDY DESIGN: A descriptive study. PLACE AND DURATION OF STUDY: Combined Military Hospital, Gujranwala, from September 2003 to September 2005. PATIENTS AND METHODS: Children of either gender suffering from more than 2 episodes of upper respiratory tract infection in the last 12 weeks were studied. The inclusion criteria were mild intermittent ear pain, fullness or "popping" of the ear drum; poor response to normal speech, inattentiveness, behavioural changes; irritability, sleep disturbances; balance problems and poor school performance. Diagnosis of OME was confirmed by pneumatic otoscopy while tuning-fork tests, tympanometry, pure tone audiometry were used as adjunct investigations. Results were described in percentage and 95% confidence interval was determined. RESULTS: Out of the 350 referred cases, pneumatic otoscopy by otolaryngologists diagnosed OME in 147 (42%, 95% CI=37.9-47.1). Out of these, 112 (76%) had retracted tympanic membrane, 25 (17%) had bulging of tympanic membrane and 10/147 (6.08%) cases showed dullness of tympanic membrane with no additional features. Tympanometry confirmed fluid behind the tympanic membrane in 145/147 (98.6%) cases. Tuning-fork tests showed negative Rinne's test in 122 (83%). Pure tone audiometry diagnosed deafness in 138/147 (93.8%) cases. CONCLUSION: OME with hearing loss is common in children having recurrent upper respiratory tract infection and nonspecific ear-related symptoms.

Klippel-Feil syndrome with situs inversus--a rare association.

Klippel-Feil Syndrome (KFS) is a congenital anomaly characterized by a defect in the formation or segmentation of the cervical vertebrae. The clinical triad consists of short neck, low posterior hairline and limited neck movement. Multiple congenital anomalies have been associated with this disease. This is a case of KFS in a young girl along with situs inversus, which is an extremely rare association. Various systemic associations occurring in this multi-system disorder are also discussed.

Klippel Trenauny Syndrome.

Klippel Trenauny Syndrome is a rare congenital syndrome characterized by port wine stain (capillary malformation), limb or hemihypertrophy and other vascular malformations. We present a case of this rare disorder in a young boy at an early stage of this disease.