Pleural effusion is a rare complication of ventriculo-peritoneal (VP) cerebrospinal fluid (CSF) shunting and its diagnosis is difficult in patients with neurological and consciousness impairment. Herein we report the case of a child affected by Pfeiffer syndrome and hydrocephalus, shunted at the age of 3 months, who developed acute respiratory failure due to a right-sided pleural effusion 2 years later. Plain chest radiographs and computed tomography (CT) showed the intrathoracic migration of the right VP shunt abdominal tip. Beta-2 transferrin, a marker for CSF, was found in the pleural fluid and the hypothesis of a CSF hydrothorax was confirmed. Effusion was treated with a thoracentesis. Seven days after, the right VP shunt was revised; a ventriculo-atrial (VA) shunt was also placed on the left side to serve as the main CSF shunt and to prevent the recurrence of hydrothorax. We review the pediatric cases of CSF hydrothorax reported in the literature and discuss the mechanisms underlying this complication together with the possible treatments. Pleural effusion due to VP shunt insertion is a rare and potentially life-threatening condition that should be suspected in any patient with a VP shunt and respiratory failure. Signs of hydrothorax may moreover represent the only clinical evidence of a shunt-related complication in case of neurologically severely compromised patients in which neurologic examination cannot help to make a diagnosis.

Medications with methyl-prednisolone sodium succinate containing lactose, which potentially contains traces of cow’s milk proteins (CMP), could cause allergic reactions or compromise treatment of acute allergic reactions in sensitized patients. We describe the unusual case of a one-year-old child affected by short bowel syndrome and history of severe cow’s milk allergy (CMA) and anaphylactic reaction due to intravenous administration of methyl-prednisolone sodium succinate (Solu-Medrol 40 mg, Pfizer). He was admitted to our hospital for severe respiratory failure and was initially treated with methyl-prednisolone (Urbason 40 mg, Sanofi Aventis), then with methyl-prednisolone sodium succinate (Solu-Medrol 40 mg, Pfizer). After the intravenous administration of second steroid, immediate anaphylaxis was recorded and treatment was stopped. Antihistamine and epinephrine were required and symptom resolution occurred. Children who are highly sensitive to milk may have severe allergic reactions also after exposure to CMP through a different administration route than the oral one. Patients who have food allergies need to pay particular attention to the prescription of drugs and their formulation.

Congenital central hypoventilation syndrome (CCHS) is characterized by alveolar hypoventilation increasing during sleep and affected patients are unable to perceive and respond to hypercarbia with increased ventilation and arousal during sleep. PHOX2B gene mutations are considered as responsible for CCHS. Most of patients with CCHS are heterozygous for polyalanine expansion mutations (PARMs) in exon 3, but 10% of patients with classic CCHS are heterozygous for non-polyalanine expansion mutations (NPARMs) of the PHOX2B gene. Data are collected on 3 patients affected by CCHS who referred to the Paediatric Pulmonology Unit of Bambino Gesù Children’s Hospital (Rome, Italy) for a multidisciplinary follow-up program between 2000 and 2017. We describe three cases of patients affected by CCHS for which two novel mutations on exon 3 of PHOX2B gene were detected. The description of these novel mutations and related clinical phenotypes allows to expand the knowledge into NPARM spectrum. Since the presence of Hirschsprung disease is related to NPARMs and the number of alanine repeats, we suggest performing CCHS genetic investigation and periodical assessment also in patients without a clear history of CCHS but affected by Hirschsprung disease. Data are retrospectively collected.

During recent years, interest on Sleep Disordered Breathing (SDB) in pediatric age has increased, due to the impact on quality of life, psycho-physical attitude and other serious morbidities if undiagnosed and untreated. Italian Pediatric Respiratory Diseases Society (SIMRI) SDB-Working Group carried out an exploratory survey in Italy, from January to December 2016, to assess the diagnostic and therapeutic pathways, perception and relevance of SDB in Italian Hospitals. A questionnaire was sent to 180 Pediatric Units (PUs) distributed throughout the Italy; 102 Pediatric Units (PUs; 56.6%) answered and among them 57% dealt with SDB, and 94% recognized SDB as a major problem. Instrumental tests performed by the PUs were saturimetry (66%), nocturnal polygraphy with complete cardio-respiratory monitoring (46%) and full polysomnography (23%). In addition, hospital pediatricians reported that 54% of parents were unaware of the SDB and 84% did not know their complications. In the Northern Italy, the diagnosis was frequently performed with instrumental tools and the treatment was often surgical. In the Southern Italy the diagnosis was clinical, and the treatment was usually with drugs. The results of our study showed a heterogeneity in the diagnosis and treatment of SDB throughout Italy. Parents know little about SDB and their complications. The operator satisfaction was associated with the availability of tools for diagnosing SDB.

In our letter, we comment the paper of Kounis et al., that highlights a poor-known clinical entity determined by systemic use of corticosteroids, the so-called “Kounis syndrome type I”. We appreciated and shared the intent of Authors to treat the important issue of high risk of adverse drug reaction in patients with atopic diathesis and we confirm the need to administer corticosteroids with caution in patients suffering from allergic disease.