Laryngomalacia (LM) patients typically present with inspiratory stridor during the first few weeks of life, which usually worsens over the first 6 months of life and peaks in everity at about 6 months of age. The stridor is typically worse with agitation, crying, feeding, and supine positioning1.
Infants with LM may have a difficult time coordinating the suck swallow breath sequence needed for feeding, as a result of their airway obstruction. The increased metabolic demand of coordinating eating and breathing against the obstruction can be so severe that it results in weight loss and failure to thrive.2 Here we present a case of seven-month-old male child with LM suffering from growth faltering.
A seven months old male child presented with upper airway obstruction. Infant faced problem of oral feeding due to airway obstruction.
At admission,
- Weight: 5.130 kg
- Height: 61 cm
- Head circumference: 43 cm
- Mid upper arm circumference (MUAC): 12.5 cm
The child had undergone operation for bilateral inguinal hernia in the neonatal period. Required multiple hospital admissions thereafter, secondary to chest infections after tracheostomy.
CT chest, CT PNS (paranasal sinus cavities), ECHO, Brain MRI was ordered. Bronchoscopy was carried out. Blood pressure (BP) was checked. LFT, RFT, serum electrolytes, chest X - Ray, barium meal, and milk scan were also ordered. Clinical exome sequencing was also carried out to detect any genetic abnormality.
Chest CT, echo, brain MRI, BP, LFT, RFT, serum electrolytes, and chest X ray were all normal. Clinical exome sequencing did not show any genetic abnormality. Barium meal and milk scan were also normal. Bronchoscopy showed signs of LM. CT PNS revealed narrowing and near occlusion (II degree) of oropharynx. Posteriorly placed adenoids were seen. Patients weight was less than that for 3rd percentiles on WHO growth chart.
Patient was diagnosed to be having growth faltering due to severe LM. There was severe upper airway obstruction and narrowing at nasopharyngeal cavity was seen.
Patient was kept in PICU for 3 weeks and post PICU was hospitalized further for next two days, making total of 23 days of hospitalization.
Child was on gastrotomy tube (G-tube) feeding with regular formula with added MCT oil. His weight and height were less than that of 3rd percentile weight and height for his age on growth charts. Child was not able to gain weight because of increased energy requirements due to increased work of breathing and multiple hospital admissions. Though investigations were normal, there was clinical suspicion of GERD. Increasing feed volume therefore was difficult. Patient had to undergo surgery during third week of hospitalization when patient had to be NPO for 3 days.
Aim of nutritional management was to achieve weight and height between 25 to 50 percentiles values for his age. Satisfactory weight gain can further lead to spontaneous resolution of upper airway obstruction. Further, the nutritional management also aimed to improve immunity and normal neurodevelopmental outcomes.
Child was started on energy and nutrient dense formula (ENDF), 100 Kcal/kg/day, 60 to 70 ml every 3 hours. Gradually as the patient tolerated the formula, amount was increased to 160-170 Kcal/kg/day over a period of one week.
The child tolerated the formula well without any episode of diarrhoea. No flatulence or vomiting were noted. Child gained 400 g over two weeks period making his weight 5.53 kg at the end of two weeks. At discharge child showed satisfactory weight gain and was on 160 to 170 Kcal/g/day of ENDF. His weight at discharge was 5.445 kg. Drop in weight at discharge was due to surgery patient had to undergo during third week. He was discharged on G-tube feeding with advice to check weight every two weeks. Since child was continued on G tube ENDF feeding post discharge, parents were informed about signs of intolerance and were advised to report it.
At follow up after two weeks from discharge his weight was 6.00 kg, height was 61 cm, head circumference was 43 cm, and MUAC was 12.5 cm. Patient tolerated formula well and did not show any signs of intolerance. Patient showed acceptable weight gain and was thriving well.
LM is defined as collapse of supraglottic structures during inspiration. Most forms of LM are minor (70—90%), presenting in the form of isolated and intermittent stridor with no alteration of crying or coughing, no dyspnoea, and no swallowing disorders. Only severe forms of LM require therapeutic intervention.3 Severe LM is associated with eight primary symptoms: inspiratory stridor, suprasternal retraction, substernal retraction, feeding diflculty, choking, post-feeding vomit, cyanosis, and failure to thrive.4 Patients with LM can have diflculty coordinating breathing and swallowing, with resultant dysphagia, feeding diflculties, and aspiration. Possible etiologies of dysphagia in patients with LM include airway obstruction leading to diflculty coordinating sucking, swallowing, and breathing, decreased laryngeal sensation secondary to GERD, and altered sensorimotor integrative function of the larynx.1 Children with LM not only have higher caloric demands, they may also have feeding diflculties also. Interventions involving high caloric formula intake may facilitate weight gain.5
In the case presented here we used this approach for improving weight gain. ENDF used here have shown convincing weight gain. Since improved weight gain is a desirable outcome in patients with LM, use of ENDF can be considered as one of the important measures in managing LM.
We can conclude from the case presented here that the nutritional management forms an important aspect of management of growth faltering due to LM. ENDF can be used to tackle LM associated growth faltering.
LFT: Liver Function Test; RFT: Renal Function Test; MRI: Magnetic Resonance Imaging; CT PNS: Computed Tomography Paranasal Sinuses; GERD: Gastroesophageal Reflux Disease; NPO: Nil per os
References:-
- Simons JP, Greenberg LL, et al. Laryngomalacia and swallowing function in children. Laryngoscope. 2016 Feb;126(2):478-84.
- Landry AM, Thompson DM. Laryngomalacia: disease presentation, spectrum, and management. Int JPediatr. 2012;2012:753526.
- Ayari S, Aubertin G, et al. Management of laryngomalacia. Eur Ann Otorhinolaryngol Head Neck Dis. 2013 Feb;130(1):15-21.
- Fattah HA, Gaafar AH, Mandour ZM. Laryngomalacia: Diagnosis and management. Egyptian Journal of Ear, Nose, Throat and Allied Sciences. 2011;12(3):149-53.
- Kusak B, Cichocka-Jarosz E, Jedynak-Wasowicz U, Lis G. Types of laryngomalacia in children: interrelationship between clinical course and comorbid conditions. Eur Arch Otorhinolaryngol. 2017 Mar;274(3):1577-1583.
Disclaimer:
The World Health Organization (WHO)* has recommended that pregnant women and new mothers be informed of the benefits and superiority of breast-feeding, in particular, the fact that it provides the best nutrition and protection from illness for babies. Mothers should be given guidance on the preparation for and maintenance of lactation, with special emphasis on the importance of the well-balanced diet both during pregnancy and after delivery. Unnecessary introduction of partial bottle feeding or other foods and drinks should be discouraged since it will have a negative euect on breast-feeding. Similarly mothers should be warned of the diflculty of reversing a decision not to breastfeed. Before advising a mother to use an infant formula, she should be advised of the social and financial implications of her decision. For example, if a baby is exclusively bottle-fed, more than one can (500g) per week will be needed, so the family circumstances and cost should be kept in mind. Mother should be reminded that breast milk is not only the best but also the most economical food for babies. If a decision to use infant formula is taken, it is important to give instruction on correct preparation methods, emphasizing that unboiled water, unsterilized bottles or incorrect dilution can lead to illness.
*See: International Code of Marketing of Breast Milk Substitutes, adopted by the World Health Assembly in Resolution WHA 34.22, May 1981.